Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace

Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace

Jennifer Niskala Apps Laura Weiss Roberts

l

Robert F. Newby

Editors

Pediatric Neuropsychology Case Studies From the Exceptional to the Commonplace

Jennifer Niskala Apps Medical College of Wisconsin Department of Psychiatry and Behavioral Medicine 9000 W. Wisconsin Ave. Milwaukee WI 53226 USA

Robert F. Newby Medical College of Wisconsin Departments of Neurology and Pediatrics 9200 W. Wisconsin Ave. Milwaukee WI 53226 USA

Laura Weiss Roberts Medical College of Wisconsin Department of Psychiatry and Behavioral Medicine 8701 Watertown Plank Road Milwaukee WI 53226 USA

ISBN 978-0-387-78964-4

e-ISBN 978-0-387-78965-1

Library of Congress Control Number: 2008930758 # 2008 Springer Science+Business Media, LLC All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, Inc., 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now know or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights.

Printed on acid-free paper. 987654321 springer.com

To those who have always believed and encouraged, beginning with my unconditionally loving mom and continuing with my inspirational husband. —JNA For David, Michael, Kate, and Anna: Parents learn from their children. —RFN For my beautiful, resilient, compelling children, Madeline, Helen, Willa, and Thomas. —LWR

Preface

For each one of us, life is a story. Our own story. It begins before we are born and is written slowly, painstakingly, a day at a time – or at some moments in a sudden flurry. Each person who touches our lives writes a bit of that story, and in turn we touch others and write bits of their stories. Our story is a collection of our experiences, evidence of our impact in the world, and the knowledge we have of ourselves as individuals. When a child comes into the life of parents, several stories unite. We imagine this union, growing up on the expectation that parenthood, and childhood, should somehow be uniquely perfect. So what then happens when an unexpected chapter of that story is introduced? We have all heard that children are not born with a “how to” manual. No parent raising a child doubts the truth of this statement. However, for some parents, the immensity of this truth can seem difficult to bear. Some parents face the task of writing entire chapters of their child’s life while dealing with trauma, illness, and disabilities, which were never supposed to happen. A pediatric neuropsychologist is both fascinated and frustrated by these chapters in children’s lives. For most of us, we venture into this career because of our love for children and our intense desire to help those children in need. A neuropsychologist’s career begins with training in psychology, focusing on the “normal” and aberrant development of people, behaviorally and emotionally. We then turn our focus to the cognitive functions, studying the relationship of the brain to behaviors. In pediatric neuropsychology, these diligently applied skills are used to listen to each child’s story, interpret each parent’s experience, evaluate the brain’s functional abilities, and try to find ways to guide the story’s path. The unconditional love of a parent resonates beauty. Each pediatric professional who intervenes in a child’s life helps teach a family how to understand and grow with their child. Those of us who work in these professions know, on a personal level, how moving and amazing these stories of love can be. It is with respect to the journey children take that we collected some of those stories here. Our intention in creating this book was to represent the incredible diversity of challenges that many children and their families work so hard to understand and overcome, while acknowledging the professionals witnessing and mediating this process. By bringing such a wide collection of stories to the reader, we offer insight into the field of pediatric neuropsychology but also into the lives of the children and vii

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Preface

families with whom we work every day. This book is intended to inform our colleagues not only in pediatric neuropsychology but in all fields of intervention for children who are struggling, and we have attempted to include many of their voices as authors. In addition, we hope to educate students across disciplines and bring a sense of recognition to families with similar battles. This book is divided into three sections, with individual chapters telling the personal story of a child and family seeking help. The first part presents cases of children experiencing unexpected neurological insults resulting in interruptions in their development. The second part, by contrast, acquaints the reader with children experiencing unique patterns of development. Finally, the third part presents cases that raise questions, particularly with different interpretations from various points of view and by various areas of expertise. Each chapter, or story, is intended to both stand alone and contribute to the whole of the book. Please feel free to read selectively the chapters relevant to your interests, or start at the beginning and follow our retelling as we lead you along. An appendix is provided at the end of the book compiling all the different tests, or assessment measures, used throughout with brief explanations of how to interpret various scores. Key terms presented in chapters are defined at the end of each chapter. Some of these terms are unique to a case, while others may reoccur many times. Similarly, each chapter provides recommendations for intervention, and at times these recommendations may overlap across chapters. Much like the unique needs of the children represented here, we believe each chapter has unique needs for the presentation of information. Therefore, it was decided on a case-by-case basis, with the guidance of the authors who knew these children best, what terms, tests, recommendations, and information needed to be presented. We have endeavored for symmetry across the chapters, although we also hope the individual character of each child shines through. This book has been an exciting and challenging project. The initial invitation from Springer Publishing to consider a book such as this left us literally giddy with anticipation. The chance to contribute something to our field representing the hard work we commit to children and families every day was both daunting and awe inspiring. All the contributing authors responded with enthusiasm as they completed their chapters, seeming to find joy in letting go of their clinical role for a brief moment, instead connecting individually with and sharing these children’s stories. For us as editors and authors, we are immensely grateful to each one of you who worked with us. Your passion for each child’s story is what gives this book life. “Thank you” also to the families and children whose lives will help write the chapters of other families’ stories now; to our own families, for the support and love that make our personal stories so rich; to our faithful and tireless editorial assistant, Ann Tennier, without whom this book would never have taken form: Your support and organization made reality out of creativity. Most of all, thanks to all the students, professionals, parents, and families who work every day to create a better life for a child. Jennifer Niskala Apps Robert F. Newby Laura Weiss Roberts

Contents

Part I

Dangers of Childhood: Neurological Disorders 3

1

Beating the Odds: Prematurity and Posthemorrhagic Hydrocephalus Jennifer I. Koop

2

Never, Ever Shake a Baby: Pass It On . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 Amy K. Heffelfinger

3

Grand Larceny in the First Grade: Traumatic Brain Injury in the School-Aged Years . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 Jonathan E. Romain

4

A Slippery Descent: Adolescent Traumatic Brain Injury . . . . . . . . . . . . . . 33 Peter L. Stavinoha

5

Steamrolled: Sports-Related Concussions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43 Michael Sharland and Thomas A. Hammeke

6

Bad Signs: Sickle-Cell Disease and Stroke . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51 Kimberly Rennie Weissgerber and Amanda Epping

7

Life Interrupted: Medulloblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 Peter L. Stavinoha

8

The Mystery of the Falling Grades: Seizure Disorder . . . . . . . . . . . . . . . . . . 69 Lynn Bennett Blackburn

9

When Half a Brain is Better than One: Recurrent Seizures . . . . . . . . . . . 77 Lynn Bennett Blackburn

10

Pathological Left-Handedness: Stroke and Seizures . . . . . . . . . . . . . . . . . . . . 87 Elizabeth N. Adams

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Contents

11

Moving Forward: Psychotherapy with a Youth After Brain Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 Mary C. Kaven

12

Family Matters: Psychosocial Factors on Neuropsychological Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109 Grace W. Fong

13

Dog Attack: Physical Trauma with Associated Brain Injury . . . . . . . . 121 Jennifer Niskala Apps

14

Back to Life: Anoxic Brain Damage in a Near Drowning . . . . . . . . . . . . 129 John M. Oestreicher

Part II

How It Can All Go Wrong: Developmental Disorders

15

The Energizer Bunny Meets Shirley Temple: Attention Deficit Hyperactivity Disorder, Combined Type . . . . . . . . . . . . . . . . . . . . . . 141 Michael J. Zaccariello

16

‘‘He is not Working up to Potential’’: Atypical Attention Deficit Hyperactivity Disorder with Executive Weaknesses . . . . . . . . . . 151 Jennifer Niskala Apps and Dawn Pflugradt

17

Sorting Sounds: Reading Disability with Phonological Awareness Deficit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159 Robert F. Newby

18

Well Compensated But Never Quite Solved: Lingering Dyslexia . . . . 167 Robert F. Newby

19

Emily Confronts Her Fiercest Bear: Word Reading Disorder with Naming Speed and Phonological Deficits . . . . . . . . . . . . . . . . . . . . . . . . 175 Molly Drake Shiffler

20

A Tale of Two Assessments: Reading Fluency . . . . . . . . . . . . . . . . . . . . . . . . 191 Kara Lindstedt and Michael J. Zaccariello

21

Lost in Space: Nonverbal Learning Disability . . . . . . . . . . . . . . . . . . . . . . . . 201 Richard J. Clark

22

Beyond Diagnosis: Applied Behavior Analysis Treatment of Moderate Autism Spectrum Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 217 Christopher P. Wiebusch

Contents

xi

23

When Quirks and Quick Learning Create a Quandary: Mild Autism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231 Elizabeth N. Adams

24

On Eggshells: Pediatric Bipolar Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245 Jennifer Niskala Apps and Dawn Pflugradt

25

It Helps to Know Genetic Basis: Williams Syndrome as an Example of Cognitive Disability . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 255 Bonita P. Klein-Tasman, Frank J. Gallo, Kristin D. Phillips, and Kathi M. Fine

26

Mixed Bag: Tics, Compulsions, and More . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267 Kenneth L. Grizzle

Part III

Things that Go Bump in the Night: Interesting Questions and Controversies for Our Field

27

A ‘‘Sensational’’ Way to Understand and Serve Children: Illustration of a Sensory Processing Model . . . . . . . . . . . . . . . . . . . . . . . . . . . . 281 Winnie Dunn

28

Sense and Sensibility: Relating Behavior Control Issues with Self-Regulation of Sensory Input . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295 Robert F. Newby and Winnie Dunn

29

Elusive, Inclusive, or Conclusive? (Central) Auditory Processing Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 307 Susan Oliff Carneol

30

A Little of This, A Little of That: (Central) Auditory Processing Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325 Lisa R. Cox

31

A Hunt for the Elusive Neuropsychological Impairment: Conversion Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335 Michael J. Zaccariello

32

A Mystery of Perplexing Symptoms: Neuropsychological Assessment in a Case of Dysautonomia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 343 Robert F. Newby and Safwan S. Jaradeh

Appendix Description of Tests and Reported Scores . . . . . . . . . . . . . . . . . . . . . 351 Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 357

Contributors

Elizabeth N. Adams Minnesota Epilepsy Group, Saint Paul, MN Jennifer Niskala Apps Department of Psychiatry and Behavioral Medicine, Medical College of Wisconsin, Milwaukee, WI Children’s Hospital of Wisconsin, Milwaukee, WI Lynn Bennett Blackburn Departments of Neurology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI Susan Oliff Carneol Children’s Hospital of Wisconsin, Milwaukee, WI Richard J. Clark St. Francis Children’s Center, Glendale, WI Wisconsin School of Professional Psychology, Milwaukee, WI Lisa R. Cox Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Winnie Dunn Department of Occupational Therapy Education, University of Kansas Medical Center, Kansas City, KS Amanda Epping Medical College of Wisconsin, Milwaukee, WI Kathi M. Fine Department of Psychology, University of Wisconsin, Milwaukee, WI Grace W. Fong Children’s Healthcare of Atlanta, Atlanta, GA

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Contributors

Frank J. Gallo Department of Psychology, University of Wisconsin, Milwaukee, WI Kenneth L. Grizzle Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI Thomas A. Hammeke Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Amy K. Heffelfinger Departments of Neurology, Neurosurgery, and Pediatrics, Medical College of Wisconsin, Milwaukee, WI Safwan S. Jaradeh Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Mary C. Kaven Department of Psychiatry, Child and Adolescent Division, University of New Mexico School of Medicine, Albuquerque, NM Bonita P. Klein-Tasman Department of Psychology, University of Wisconsin, Milwaukee, WI Jennifer I. Koop Departments of Neurology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI Kara Lindstedt Department of Clinical Psychology, Marquette University, Milwaukee, WI Robert F. Newby Departments of Neurology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI John M. Oestreicher Affinity Medical Group, Appleton, WI Dawn Pflugradt Milwaukee, WI Kristin D. Phillips Department of Psychology, University of Wisconsin, Milwaukee, WI Laura Weiss Roberts Department of Psychiatry and Behavioral Medicine, Department of Population Health, Center for the Study of Bioethics, Medical College of Wisconsin, Milwaukee, WI

Contributors

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Jonathan E. Romain Children’s Hospital of Wisconsin, Milwaukee, WI, Department of Psychiatry and Behavioral Medicine, Medical College of Wisconsin, Milwaukee, WI Michael Sharland Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Molly Drake Shiffler Department of Literacy and Language Development, Cardinal Stritch University, Milwaukee, WI Peter L. Stavinoha Children’s Medical Center, University of Texas Southwestern Medical Center, Dallas, TX Kimberly Rennie Weissgerber NeuroBehavioral Health Clinic, Glendale, WI, St. Luke’s Medical Center, Milwaukee, WI Christopher P. Wiebusch Wiebusch and Nicholson Center for Autism, Inc., Pediatric Neuropsychology and Treatment Associates, S.C., Pewaukee, WI Michael J. Zaccariello Division of Neuropsychology, Department of Neurology, Medical College of Wisconsin and Froedtert Hospital, Milwaukee, WI

Part I

Dangers of Childhood: Neurological Disorders

Children are perfect in their parents’ eyes. Although parents may know their children have flaws and weaknesses, their children complete them in a way that often blinds parents to those qualities. Parents’ love for their children is so strong and absolute that they forget all else. They defend their children fiercely and protect them passionately, and when their child is injured or hurt by something outside of their control, parents can be overcome. This section presents a series of cases in which children experience unexpected deviations in their otherwise normal development. Their parents are often beset by the severity and enormity of what has happened. Each parent’s question to the pediatric neuropsychologist is slightly different. Some are not ready to hear what their child now needs, while others cannot get the information quickly enough. In every case, managing the child’s and the family’s needs is a delicate balance. This section opens with a chapter about premature birth. According to the March of Dimes Web site, http://www.marchofdimes.com/, premature birth is the number one obstetrical complication in the United States. For parents first meeting their new child, prematurity adds a stressful and frightening chapter to their story. The next four chapters describe children experiencing head injuries from different circumstances. The developing brain can be injured in various ways, with a range of short and long-term sequelae. According to the Centers for Disease Control Web site, http://www.cdc.gov/ncipc/tbi/TBI.htm, brain injuries are the leading cause of death and disability in children and adolescents. Further, one-third of all pediatric injuries involve some form of brain injury. The causes of these injuries vary greatly by age and ethnicity. Under age 5, falls are the most frequent cause. Motor vehicle crashes are another common cause of these injuries. Mild traumatic brain injuries (TBIs) can occur with concussion, either because of injuries or sportrelated activities. Of children under age 14, an estimated 475,000 TBIs occur each year, and children under age 4 are at highest risk. In addition, brain injuries in infants are a particular concern when they result from Shaken Baby Syndrome. Although it is difficult to estimate the prevalence of this trauma, the National Center on Shaken Baby Syndrome approximates that 20% of infants presenting for medical care with these injuries will die, with the majority of the survivors having permanent brain injuries (http://dontshake.com/).

2

Dangers of Childhood: Neurological Disorders

The following seven chapters present children with medical illnesses disrupting their development and resulting in neuropsychological sequelae. Many acute and systemic medical illnesses can have long-standing impact on the developing brain, resulting in neurological trauma. The National Cancer Institute reports that just over 3 of every 100,000 children will be diagnosed with some form of brain cancer (http://seer.cancer.gov/publications/childhood/). Not only can the experience of cancer itself result in neurological insults, but the treatments used for cancer, including radiation and chemotherapy, have documented long-term effects on a child’s mental capacities. Seizure disorders also cause neurological insults for children. According to the Epilepsy Foundation (http://www.epilepsyfoundation.org), each year 120,000 children under age 18 years will experience a seizure and 45,000 children will develop epilepsy. At any given moment 326,000 children in elementary school and junior high school have epilepsy. The neurological impact of this illness can range from mild to severe, depending on the location and type of seizure activity, seizure frequency and severity, and the medical interventions used to treat the seizures. The last two of these seven chapters highlight the impact such difficulties can have on families. When a child experiences an illness or injury, particularly a lifethreatening one, the dynamics of the family change. Parents work to protect and conserve their family’s identity while focusing on making things ‘‘better.’’ As neuropsychologists, we often must address the family’s needs in addition to the child’s cognitive, social, and emotional needs. Chapter 11 particularly highlights the interaction between neuropsychology and therapy. Chapter 12 also touches on the complications of the family’s vs. the children’s needs, while presenting a different etiology of developmental disruption. Chapters 13 and 14 relay stories of children experiencing more unusual accidents. Although many childhood accidents result in direct brain injuries, some result in indirect neurological insult. In these two chapters, injuries to the child’s body, through physical injury in one case and anoxia, or lack of oxygen, in the other, resulted in long-term neuropsychological sequelae. For some families, making sense of why such apparently random accidents happened to their child is perhaps the hardest part. As you read each chapter, you will find several tools to assist in your understanding of the information provided. Key terms are in bold face at their first use and defined at the end of the chapter, where references for both clinicians and families are also provided for those wanting more information about the condition(s) presented. Test scores from neuropsychological evaluations are provided in tables. The appendix at the end of the book lists the tests and describes normative test data and standardized scores.

Reference Langlois, JA, Rutland-Brown, W, Thomas, KE. (2005). The incidence of traumatic brain injury among children in the United States: Differences by race. Journal of Head Trauma Rehabilitation. 20(3), 229–238.

Chapter 1

Beating the Odds: Prematurity and Posthemorrhagic Hydrocephalus Jennifer I. Koop

Lucy was a miracle child. She overcame extreme odds and significant medical complications to become a spunky, chatty, child beauty pageant contestant. When she was referred for an evaluation at age 5 years by a neurosurgeon, she was doing well academically in a regular-education kindergarten classroom. However, both her parents and teachers acknowledged that she talked ‘‘too much’’ and had ‘‘selective hearing’’ problems. Directions at home and at school often needed to be repeated several times before Lucy would comply. Lucy appeared easily distracted, especially by her own thoughts or stories, which she enjoyed creating and telling to others. Lucy was also a social butterfly who was indiscriminant in her socialization and would approach anyone, even strangers, to begin a conversation. She was described as a very physically active but clumsy young girl who didn’t enjoy down time. Overall, though, Lucy was simply a happy and delightful little girl who was immediately liked by all who met her. Lucy was born 13 weeks early, after 27 weeks of gestation. Weighing less than 2 pounds at birth, her whole body practically fit in the palm of her father’s hand. Unfortunately, Lucy’s father wasn’t able to hold her as much as he would have liked during the first 3 months of her life. Lucy stayed in the neonatal intensive care unit for 11 weeks. She experienced medical complications typically associated with premature birth, including immature lungs, necessitating use of a ventilator for several weeks; patent ductus arteriosus, a heart malformation requiring ligation surgery; retinopathy of prematurity, requiring bilateral eye surgery; and jaundice, requiring light treatment. Most significantly, on day 3 of life, Lucy sustained a Grade III intraventricular hemorrhage affecting both cerebral hemispheres (Table 1.1). She subsequently developed hydrocephalus and underwent 11 spinal taps to control her intracranial pressure, but placement of a ventriculoperitoneal shunt was not deemed necessary at the time (Table 1.2). Once Lucy was discharged home, she was a mellow infant who slept and ate well. She seemed to develop normally. She walked and talked on time. She did not demonstrate any unilateral muscle weakness, and her motor skills appeared to be developing evenly. In fact, Lucy was described as an active toddler who was

J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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J.I. Koop

Table 1.1 Papile (1978) grading of intraventricular hemorrhages Degree of Description Symptoms on imaging studies severity Grade I Subependymal hemorrhage Blood observed in the area surrounding the vessels of germinal matrix only Blood products reabsorbed before dispersion into the ventricles or parenchyma Grade II Intraventricular hemorrhage Minimal dispersion of blood products into without ventricular dilatation lateral ventricles No distension of ventricles Grade III Intraventricular hemorrhage with Blood products evident in ventricular system ventricular dilatation Distension of ventricles evident on ultrasound Greater risk of development of hydrocephalus Grade IV Intraventricular hemorrhage with Blood products evident in ventricles and parenchymal involvement parenchyma Parenchymal involvement most often in frontal regions Distension of ventricles evident on ultrasound Greater risk of development of hydrocephalus

Table 1.2 Common complications of premature birth Respiratory distress syndrome Bronchopulmonary dysplasia (chronic lung disease) Necrotizing enterocolitis (gastrointestinal infection) Patent ductus arteriosus (cardiac malformation) Infection Hyperbilirubinemia (significant jaundice) Apnea (episodes of breathing arrest) Retinopathy of prematurity (visual abnormalities that can cause blindness) Intraventricular hemorrhage

constantly on the go. She was referred for early intervention therapies but was discharged shortly after starting because she demonstrated adequate developmental progress. Notably, Lucy demonstrated left-hand dominance for manual-motor tasks, despite the absence of any family history of left-handedness. Because of her prematurity, Lucy was evaluated by several neurologists and neurosurgeons over the years. At age 1 year, magnetic resonance imaging (MRI) of Lucy’s brain revealed abnormalities consistent with hydrocephalus, including grossly enlarged lateral ventricles with associated atrophy of the surrounding cortical tissue. However, given the lack of evidence of increased intracranial pressure and her apparent ‘‘normal’’ development and functioning, surgical intervention was not recommended. Lucy remained healthy. She was diagnosed with asthma, which is common in children who are born prematurely, but her condition was well controlled with medications. Repeated imaging studies over the first 4 years of her life suggested stability of the structural neurological abnormalities.

1 Beating the Odds: Prematurity and Posthemorrhagic Hydrocephalus

5

In fact, Lucy was only reevaluated by the neurosurgeon that referred her for neuropsychological testing after she was hospitalized for a tonsillectomy and adenoidectomy. The neurosurgeon’s question at the time of the evaluation was whether Lucy demonstrated any cognitive dysfunction that would suggest a need for surgical intervention to alleviate her persistently enlarged ventricles but presumed compensated hydrocephalus. Compensated hydrocephalus is thought to develop when equilibrium of intracranial pressure is reestablished despite a persistently greater than normal volume of cerebral spinal fluid within the ventricular system, possibly as a result of enlarged ventricular size.

Test Results Lucy underwent her first neuropsychological evaluation at age 5 years, 4 months. She was reevaluated on two other occasions, at age 6 years, 0 months, and age 6 years, 7 months, to monitor the stability of her cognitive profile. The measures administered on each occasion assessed general intellectual capabilities (Table 1.3), fine motor skills, attention and executive functioning, and language skills, and the third evaluation assessed memory functions (Table 1.4). Ideally, specific measures of visual-spatial skills also should have been included across all evaluations. However, Lucy’s age at the time of the initial evaluation greatly restricted the number of available standardized tests with appropriate normative data, resulting in the somewhat limited battery. Across all evaluations, Lucy presented as a very sociable and happy child who smiled frequently. She often initiated conversation with the examiner and talked almost incessantly unless redirected to the task at hand. Lucy was very imaginative and went into great detail when sharing stories. Her speech was significant for some mild articulation difficulties, particularly when pronouncing R, which had resolved by the time of the third evaluation. Overall, Lucy was cooperative and appeared to be putting forth her best effort on all tasks. She responded well to praise and Table 1.3 Intellectual Differential Abilities Scale – Upper Preschool Version

5 y, 4 m Scorea Percentile General conceptual ability score 89 23rd Verbal cluster 108 70th Verbal comprehension [51] 53rd Naming vocabulary [59] 83rd Nonverbal cluster 74 4th Picture similarities [33] 5th Pattern construction [40] 16th Copying [38] 12th Early number concepts [48] 45th a Standard score, (scaled score), [T-score], {z-score}.

6 y, 0 m Score Percentile 99 47th 117 87th [53] 61st [67] 96th 87 19th [50] 50th [43] 25th [39] 14th [46] 37th

6 y, 7 m Score Percentile 94 34th 116 86th [55] 69th [64] 92nd 80 9th [28] 1st [46] 34th [47] 38th [43] 24th

6 Table 1.4 Other cognitive functions Tests 5 y, 4 m Score NEPSY Sensorimotor domain 80 Imitating hand positions (11) Preferred hand Nonpreferred hand Visual-motor precision (1) Car Time Errors Motorcycle Time Errors Fingertip tapping (8) Repetitions Sequences Preferred hand Nonpreferred Attention/executive 74 domain Tower (8) Visual attention (5) Cats Time Omissions Commissions Faces Time Omissions Commissions Auditory attention/ (8) response set Attention task (9) Omissions Commissions Response task (6) Omissions Commissions Clinical Evaluation of Language Fundamentals – Preschool Receptive language 116 Sentence structure (13) Linguistic concepts (11) Basic concepts (14) Expressive language 108 Word structure (10)

J.I. Koop

Percentile 9th 63rd 26th–75th 26th–75th <1st

6 y, 0 m Score Percentile

6 y, 7 m Score Percentile

87 (8)

89 (11)

(5)

19th 25th 11th–25th 26th–75th 5th

(4)

23rd 63rd 26th–75th 26th–75th 2nd

11th–25th 11th–25th

26th–75th <2nd

>75th <2nd

>75th 3rd–10th 25th 26th–75th 26th–75th 26th–75th 26th–75th 4th

88

26th–75th <2nd 63rd 26th–75th 26th–75th 26th–75th >75th 21st

110

>75th <2nd 63rd 26th–75th 26th–75th 26th–75th 26th–75th 75th

(11) (9)

63rd 25th

(9) (13)

37th 84th

25th 5th

(12)

(11)

>75th 26th–75th 26th–75th

>75th >75th 3rd–10th

>75th >75th 26th–75th

26th–75th >75th 3rd–10th 25th

11th–25th 26th–75th 11th–25th 5th

>75th >75th 11th–25th 75th

(5)

37th 11th–25th 26th–75th 9th 11th–25th 26th–75th

(5)

86th 84th 63rd 91st 8th 50th

112 (13) (11) (12) 114 (16)

(6)

(12)

5th 3rd–10th 26th–75th 9th >75th 3rd–10th

(12)

79th 84th 63rd 75th 82nd 82nd

Not Administered

(11)

75th 26th–75th >75th 63rd 26th–75th >75th

(continued)

1 Beating the Odds: Prematurity and Posthemorrhagic Hydrocephalus Table 1.4 (continued) Tests Formulating labels Recalling sentences Total language Wide Range Assessment of Memory and Learning 2 Story memory Recall Recognition Verbal learning Recall Recognition Design memory Recognition Visual learning Delayed recall

5 y, 4 m Score (14) (10) 112

Percentile 91st 50th 79th

Not administered

6 y, 0 m Score (13) (8) 113

Percentile 84th 25th 81st

Not administered

7

6 y, 7 m Score Percentile

(11) (11) (13) (11) (10) (13) (9) (11) (7) 89

63rd 63rd 75th 63rd 50th 75th 37th 63rd 16th 23rd

verbally acknowledged that she was doing well on certain tasks. Thus, the results obtained during the evaluation were thought to provide a valid estimate of her cognitive functioning. Review of Lucy’s evaluation results begins with the data from the initial evaluation at age 5 years, 4 months. At that time, Lucy’s overall intellectual capabilities fell within the low average range. However, the overall score reflected a significant discrepancy between solidly average verbal abilities and below average or borderline visual-spatial or nonverbal abilities. Thus, the overall score is likely not an accurate estimate of Lucy’s true ability level. Within the nonverbal domain, Lucy had particular difficulty on two tasks, one that required her to recognize similarities between simple line drawings and one that required her to copy increasingly complex geometric figures. Lucy’s difficulty on the latter task was consistent with her performance on fine motor tasks. Her fine motor speed in simple finger tapping and motor planning in copying hand positions were intact. However, her visual-motor integration and fine motor coordination in completing mazes fell in the impaired range, reflecting poor motor control. Her attention skills also were mildly to moderately impaired. On a visual attention task (NEPSY Cats) she demonstrated a rapid and careless response pattern (as suggested by her speed of completion) and made a significant number of commission errors, suggesting poor impulse control. Her receptive and expressive language abilities were average, with little variability between tasks. Results of the second and third evaluations were generally consistent with those obtained during the first evaluation. Lucy continued to demonstrate a significant discrepancy between average to high average verbal skills and borderline to low average visual-spatial skills. She also continued to demonstrate fine motor difficulties, with evidence of increasing motor planning difficulties with her dominant

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(left) hand. Her attention skills also remained mildly to moderately impaired. Interestingly, and commensurate with her profile of verbal and nonverbal abilities, the addition of memory testing in the third evaluation revealed solidly average memory for verbal material but mildly impaired memory for visual-spatial material.

Formulation and Recommendations Lucy’s pattern of performance on measures administered during the neuropsychological evaluations revealed relative weaknesses and mildly to moderately impaired visualspatial abilities, fine motor skills, attention skills, and visual memory. This pattern of performance, the information gathered through clinical interview with Lucy’s parents, and Lucy’s medical history are consistent with two neurobehavioral syndromes. Lucy clearly demonstrated behavioral symptoms and impaired performance on attention measures that were consistent with Attention Deficit Hyperactivity Disorder, Combined Type (ADHD-C). As defined in the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR) of the American Psychiatric Association (2000), ADHD is characterized by a pattern of at least six of nine symptoms of inattention and impulsivity or hyperactivity that have persisted for at least 6 months and significantly impact functioning in at least two domains. As outlined in the history section, Lucy demonstrated many symptoms of inattention, hyperactivity, and impulsivity since her early toddler years that continue to impact her functioning at home and school and also were observed behaviorally in the testing environment. Additionally, and perhaps more importantly, Lucy’s pattern of strengths and weaknesses was consistent with many aspects of a nonverbal learning disability (NLD). Although NLD is not a specific learning disability as defined by federal and most state departments of education, NLD is a syndrome defined by neuropsychological deficits that significantly impact learning. In general, children with NLD demonstrate weaknesses and difficulties in visual-spatial processing, visual-motor coordination, tactile perception, attention, abstract reasoning, problem-solving, perception of emotions, and social communication. Academic problems in math, reading comprehension, and the mechanics of written language are common. In contrast, basic language processing skills, rote verbal memory, reading word recognition, and spelling are usually relatively strong. The cognitive profile of NLD is presumed to reflect impairment in nondominant hemisphere functions, which theoretically are most dependent upon white matter tract integrity. The neuropsychological profile described earlier and demonstrated by Lucy is commonly observed in children with a history of prematurity and/or hydrocephalus. Prematurity is presumed to be associated with attention and motor impairment because the normal development of these functional systems is disrupted by the trauma of preterm birth. This disrupted development is thought to be further exacerbated by the numerous medical interventions that premature infants typically experience (e.g., ventilation, repeated exposure to anesthesia for surgeries, related potential hypoxic episodes). The ventricular enlargement and increased intracranial

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pressure of hydrocephalus repeatedly have been associated with deterioration of white matter tract integrity and even delays or decreases in myelination. Perhaps the most important aspect of Lucy’s repeated neuropsychological testing is the suggestion of changes in motor, attention, and adaptive behavioral functioning. There is some evidence that neuropsychological testing is more sensitive to subtle changes in neurological status for children with unshunted, compensated hydrocephalus than other neurological techniques (e.g., computerized tomography (CT) imaging of changes in ventricular size or external monitoring of intracranial pressure). Thus correlation of the neuropsychological findings with other clinical indicators of neurological status (i.e., imaging studies) was recommended to best appreciate the significance of the observed variability and potentially clinically significant changes in cognitive performance over an 18-month period. The data from the repeated evaluations were shared with the neurosurgeon who had referred Lucy for the initial evaluation. After he had considered the neuropsychological results, especially those obtained during the third evaluation, and correlated them with neuroimaging and physiological data, the neurosurgeon recommended a surgical procedure called endoscopic third ventriculostomy, in which intracranial pressure is reduced by draining some cerebral spinal fluid from the third ventricle. Given Lucy’s cognitive profile and reported academic difficulties, her parents were encouraged to request a meeting with school personnel to develop and implement an appropriate Individualized Education Program (IEP). Once the school personnel determined that Lucy was eligible under federal criteria to receive an IEP, specific modifications and accommodations within the regular education classroom could be made. Lucy’s fine motor and visual-spatial weaknesses suggested that she would benefit from occupational therapy to strengthen her skills in these areas. Fine motor and visual-motor integration skills are particularly important during the early elementary school years because students are taught to print and to write in cursive, and an increasing number of assignments are dependent upon a student’s ability to provide written responses. Lucy’s visual-spatial difficulties impacted her ability to perform many demands of school. Although specific examples of modifications are provided, the overarching recommendation was for school personnel to utilize and emphasize Lucy’s relatively stronger verbal skills to compensate for her visual-spatial difficulties whenever possible. Copying problems or other assignments from a book or chalkboard or writing at length will be very effortful. It was suggested that the requirements for writing be reduced and that some of Lucy’s assignments and testing be oral. As she progresses in school, Lucy should begin to receive training in the use of a computer and word processor to help her prepare her assignments. Explicit directions simply given orally will be difficult for Lucy if she does not know how to proceed perceptually. She may need modeling and/or direct teacher intervention. Reading graphs, maps, and charts may be difficult. Lucy may need individual help in interpreting these educational materials, primarily though verbal explanations accompanying the visual material.

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Mathematics instruction should include practical and hands-on tasks. Lucy will need help with measuring devices and estimating time, size, and distance. Her visual-spatial impairment suggests that when she learns computational procedures, she may need to divide her sheet into columns to facilitate placement of the numbers. Lucy’s attention deficits will require additional instructional modifications to maximize her participation in and benefit from academic instruction. She will require more frequent attentional cueing and support for her relative lack of organizational skills. Redirection should be provided in a subtle positive manner, such as a tap on the shoulder or hand on the desk. Preferential seating close to the teacher or near the front of the classroom will likely help reduce distractions and position Lucy in a location that facilitates frequent redirection from or checking in with the teacher. Complex tasks should be broken down into smaller sequential steps. Instructions should be given one at a time, and longer sets of instructions should be broken down, repeated, rehearsed, or written down for Lucy. Additional instruction in organizational skills such as outlining, diagramming, or planning the sequence of steps to complete a task would be helpful. It is always difficult to predict cognitive outcomes for young children, especially in the context of a potentially progressive neurological condition. However, if Lucy’s hydrocephalus remains stable and does not progress and result in increased intracranial pressure, it would be presumed that her cognitive profile also would remain stable. That is, she will likely continue to require interventions for ADHD and NLD. Use of a stimulant or other attention-enhancing medication may be warranted at some time to improve her attention capabilities. The underlying neurological cause of Lucy’s attention difficulties, however, may make pharmacological intervention more difficult. With regard to the NLD symptoms, Lucy will likely continue to demonstrate a pattern of verbal strengths and visual-spatial weaknesses that will impact her academic performance.

Additional Resources Key Concepts and Terms Hydrocephalus Accumulation of cerebral spinal fluid within the ventricular system causing dilatation of ventricles and potentially increased intracranial pressure, which can compress brain tissue. In early childhood, hydrocephalus also often results in enlargement of the head circumference. Otherwise known as ‘‘water on the brain.’’ Intraventricular hemorrhage (IVH) A common complication of premature birth prior to 32-weeks gestation. IVH involves bleeding within the germinal matrix surrounding the lateral ventricles. Hemorrhages range in severity and are graded from I to IV, with IV being the most serious. Hydrocephalus develops following

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IVHs in 55% of cases with Grade III and up to 80% of cases with Grade IV hemorrhages. Ventriculoperitoneal shunt The most common treatment for hydrocephalus is placement of a shunting device into the ventricle. The ventriculoperitoneal shunt is attached to a catheter positioned to end into the abdomen, diverting excess cerebral spinal fluid into the abdominal cavity.

References Resources for Clinicians American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders: DSM-IV-TR. Washington, DC: American Psychiatric Association. Dykes, F. D., Dunbar, B., Lazarra, A., & Ahmann, P. A. (1989). Posthemorrhagic hydrocephalus in high-risk preterm infants: Natural history, management, and long-term outcome. The Journal of Pediatrics, 114(4 Pt 1), 611–618. Fletcher, J. M., Brookshire, B. L., Landry, S. H., & Bohan, T. P. (1996). Attentional skills and executive functions in children with early hydrocephalus. Developmental Neuropsychology, 12 (1), 53–76. Fletcher, J. M., Francis, D. J., Thompson, N. M., Davidson, K. C., & Miner, M. E. (1992). Verbal and nonverbal skill discrepancies in hydrocephalic children. Journal of Clinical and Experimental Neuropsychology : Official Journal of the International Neuropsychological Society, 14 (4), 593–609. Fletcher, J. M., Landry, S. H., Bohan, T. P., Davidson, K. C., Brookshire, B. L., Lachar, D., et al. (1997). Effects of intraventricular hemorrhage and hydrocephalus on the long-term neurobehavioral development of preterm very-low-birthweight infants. Developmental Medicine and Child Neurology, 39(9), 596–606. Papile, L. A., Burstein, J., Burstein, R., & Koffler, H. (1978). Incidence and evolution of subependymal and intraventricular hemorrhage: A study of infants with birth weights less than 1,500 gm. The Journal of Pediatrics, 92(4), 529–534. van de Bor, M., & den Ouden, L. (2004). School performance in adolescents with and without periventricular–intraventricular hemorrhage in the neonatal period. Seminars in Perinatology, 28(4), 295–303.

Resources for Families Hydrocephalus Association. http://www.hydroassoc.org/ Linden, D. W., Paroli, E. T., & Doron, M. W. (2000). Preemies: The essential guide for parents of premature babies. New York: Pocket Books. March of Dimes: Pregnancy, babies, prematurity. http://www.marchofdimes.com/ March of Dimes: Prematurity. http://www.modimes.org/prematurity/prematurity.asp

Chapter 2

Never, Ever Shake a Baby: Pass It On Amy K. Heffelfinger

At that moment, the sun stopped shining and our happy, carefree world came crumbling down. We were told our healthy 4½-month-old baby boy was in critical condition and could die. Our son, Jeremy, had just learned to smile about a week before, and now his smile was gone. I remember two doctors addressing me and my family in a small room. Their faces were without expression. They announced that my son was the victim of shaken baby syndrome (SBS) and might die. I immediately looked at my mother, a retired nurse, for some explanation. My mom began to sob; the pain in her face is still etched in my mind. I now knew how bad it was, but I still had never heard of SBS. Now Jeremy was in a drug induced coma, and we waited for the swelling in Jeremy’s brain to stop. We were told that it would stop swelling in 3 days. Four days after Jeremy was injured, his brain continued to swell, permanently destroying healthy brain cells. He was so swollen that the doctors could not open his eyes to check his pupils. Two huge vials of fluid were removed from the top of Jeremy’s head to reduce the pressure. He had two blood transfusions. A respirator kept him alive. After a week, Jeremy began to seize and screamed for 14 hours straight until he had to be sedated. This went on for several days. He was inconsolable. Jeremy was put on phenytoin, an antiseizure medicine. To measure the levels of this medicine, a daily blood culture needed to be done. Every morning he was awakened at dawn to have blood drawn from his tiny arm, a terrifying routine. On the 21st day, we went home. We were told how severe his injury was and that he was blind in both eyes due to retinal hemorrhages. We did not know what was to come, only that the road ahead would be long and hard. We only thanked God that we still had our precious baby. Jeremy escaped death by a heartbeat, thanks to the skill of professionals, modern technology, and the fact that heaven was flooded with prayers, and continues to be, on his behalf. During the first year after his injury, Jeremy had three brain surgeries and to this day endures daily physical, occupational, and speech therapies. His future, and what he will be capable of, is unknown. Our son has had to fight and work extremely hard each and every day of his short life. Time has helped our family heal and accept what happened to our precious child. His hair now covers the 7-in. scar on his head. Although he is very sensitive to light, we know that he can see. He doesn’t use his hands like he should; they hang by his side for convenience. We have to continue to tell him to walk because his brain and his feet do not have the complete system down yet. Someday. We have extremely high expectations for Jeremy because he has continued to prove his doctors wrong. We are blessed with a close and loving family; all are involved in his care and devoted to his well-being.

J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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A.K. Heffelfinger We travel this profound journey together as a family with Jeremy leading the way. He is carving a path for us to meander along, different from traditional roads. Still, it echoes with the sounds of joy and laughter bursting forth from our precious little boy. He is a delightful, amazing child with much to say, and he sure says it. He is Auntie’s Sweet Pea. He is Grandma’s Darling Boy. He is Dad’s Little Buddy. He is Mom’s Angel. He is our miracle.

History and Reason for Referral Jeremy, a 6-year-old boy, was referred for his third neuropsychological evaluation due to nonadherence to rules and poor attention. He was initially referred at age 4 years by his neurologist due to a history of traumatic brain injury (TBI) due to SBS. Jeremy was found unresponsive at the babysitter’s home when he was 4½months old. Initial stabilization included intubation and ventilatory support. For 3 days, he presented comatose, with upper extremities rigid and fists clenched, but was exhibiting reflex of lower extremity movements to pain. He had increased intracranial pressure, and he received a subdural peritoneal shunt, which drains from subdural space into the abdominal cavity. He was in the pediatric intensive care unit for 1 week and in the hospital for 3 weeks. Discharge diagnoses included SBS, right frontal parietal subdural hemorrhage, retinal hemorrhages, and posttraumatic seizures. As in many cases with SBS, a previous injury was evident on brain scans following the events precipitating hospitalization. Phenytoin was prescribed and discontinued 1 year after the injury. Jeremy’s retinas recovered, but cortical damage in visual areas was suspected. He received sensory integration therapy, occupational therapy, physical therapy, and speech and language therapy through the Birth to Three Program until his third birthday. He attended a 4-year-old kindergarten program for 2 years and received occupational and physical therapy services. Jeremy’s development of course had been interrupted due to the severity of the abuse. Jeremy’s prenatal development, delivery, and early development were unremarkable. He was smiling, rolling, and holding his head up before being shaken, but he lost these skills. He never crawled but sat at 13 months and walked at 14 months. His gait was abnormal, and he did not use his hands when walking. His language development was reportedly a strength, and he was speaking several words by age 1 year. Emotionally, he was often inconsolable following the injury. He was highly sensitive to textures and touch, not liking high levels of physical affection. He also was hypersensitive to sights and sounds, although his hearing, taste, and smell were believed to be acute and nondisrupted. Jeremy was afraid of animals and sleeping alone. He was able to verbalize his fears by commanding what to do in situations. In fact, Jeremy often required control in situations. He was easily disrupted by change and required routines to be followed. He was very attentive to detail and could be

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emotionally frustrated if the doors were not closed or if his clothing was not as he would like. Jeremy’s parents prepared him for transitions, using timers to indicate when change would occur. They also adapted to his routines. Socially, Jeremy had been disinterested in peers until he began school. Jeremy lived with his mother, a part-time product manager; his father, a teacher; and his 3-year-old sister. His family history was significant for some obsessive– compulsive tendencies in his father. Neuropsychological testing at ages 4 and 5 years indicated several residual neuropsychological impairments, particularly with visual perception, ataxic and apraxic motor difficulties, attention, and executive functioning. Jeremy also had trouble in areas that would require integration of different skills, such as picture naming and visuospatial construction. He had close to age-appropriate strengths in verbal knowledge. There were ongoing concerns regarding his oppositional and controlling behaviors. Jeremy had been working with a psychologist, focusing on social skills training. His parents reported that his therapy had been very successful in helping them to play with him and also with his social development. Jeremy was to attend full-day kindergarten with the help of an aide, shared among three students, as well as pullout occupational therapy services three times a week to work on fine motor skills. Physical therapy services would be available as needed. He had been attending the ‘‘Handwriting Without Tears’’ program to work on early writing skills. Jeremy’s parents’ current concerns were regarding how his behavior was hindering his advancement in many areas. Both his teachers and his parents did not know what he was capable of doing. He was constantly verbally negotiating tasks he did not want to participate in or he would procrastinate. For example, in therapy he would make deals such as ‘‘I will do it if you will do it.’’ If he were threatened with something such as ‘‘we are going to call your mom,’’ he would then immediately complete the tasks. This type of behavior occurred at home also. Jeremy’s parents’ felt that his attention had improved and that he was now able to focus while he was playing. However, although he was able to focus on things that he was interested in, he continued to be unable to focus on tasks that he did not want to do. Emotionally he was less fearful than at previous evaluations, but he continued to have a more fearful personality compared to same-age peers. His parents were very concerned with his emotional functioning. Daily he would cry for 30 to 60 minutes if he did not get his way or if someone took his toy. They had not found ways to soothe him other than to give him his way. They noted that even though his sister was only 3, she was able to verbally express how she felt, but he was unable to do this. Socially, he still had difficulties with same-age peers, but he had increased social interests and interactions with them, which was a significant improvement. Cognitively, Jeremy’s parents recognized that he continued to be delayed but reported that he was making gains. For example, he knew his alphabet and could recognize some letters, and he could count to 30 but could not recognize numbers. He continued to have a significant tendency to perseverate on things such as motors or things that have wheels.

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Test Results The threefold purpose of this assessment dictated the assessment battery. First, because Jeremy had a longstanding history of problems with emotional and behavioral regulation, it was essential to focus on whether there were actual neuropsychological impairments in attention and executive functioning to help explain his difficulties. Second, it was important to determine the magnitude and rate that he was gaining neuropsychological skills, using data from the previous evaluations for comparison. Third, because he was now 6-years old, comprehensive assessments of cognition and memory were conducted to understand more fully his neuropsychological functioning. Because of his given difficulties with basic sensory processing and attentional focus, qualitative observations were carefully made. Jeremy presented for two evaluations, and his behavior was consistent. He was unable to separate from his parents, becoming tearful when attempted. However, at one time during the testing, his father was able to leave the room, and Jeremy functioned adequately. Each week Jeremy was hesitant at first but quickly warmed up to the examiners. His behavior was notable for requiring numerous prompts and reminders to look at visual stimuli to make sure that he had seen them before responding. He also needed frequent redirection to remain on task, but he was not hyperactive, nor did he leave his seat. It was clear that he wanted to do well the majority of the time, but there were occasions where he appeared to answer incorrectly to avoid the testing situation. He was very socially engaging and enjoyable to work with. He continued to have difficulties manipulating his hands, especially on command. On the basis of these observations, the results were believed to be an accurate representation of his current level of functioning (Table 2.1). Specific tests of processing speed (e.g., coding, symbol search) were not given because of Jeremy’s significant difficulties with visual processing and motor control; therefore, a Full Scale IQ was not calculated. Overall, Jeremy’s verbal abilities were in the low average range and represented a significant strength. In contrast, his overall nonverbal/visuospatial skills were in the mildly mentally deficient range. His working memory, the ability to hold and mentally manipulate information, was in the range of borderline impairment (Table 2.2). These findings were consistent with those from his previous general cognitive testing when he was 4 years old and continued to document significantly stronger verbal abilities in comparison to weaker nonverbal/visuospatial skills. Jeremy continued to have a significantly difficult time completing the motor tasks that were administered to him. On a measure where he was to tap his fingers rapidly together and then tap his fingers in a sequence, his performance was severely affected by his attention in that he was unable to tap consistently for the time allotted. He also was unable to complete the motor program to tap his fingers in a sequence. On another task, he was to demonstrate motor planning by copying finger position movements. This task was discontinued because Jeremy was unable to do most of the hand positions. These findings suggested that Jeremy continued to exhibit constructional apraxia, that is, he had difficulty using his hands, copying

2 Never, Ever Shake a Baby: Pass It On Table 2.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal Comprehension Index Perceptual Reasoning Index Working Memory Index Similarities Vocabulary Comprehension Block Design Picture Concepts Matrix Reasoning Digit Span Letter-Number Sequence Coding Symbol Search a Standard score, (scaled score), [T score], {z score}.

Table 2.2 Other cognitive functions Tests NEPSY Cats Time Omissions Commissions Attention Omissions Commissions Tower Arrows Memory for faces Kaufman Assessment Battery for Children Gestalt closure Wide Range Assessment of Memory and Learning – Second Edition Verbal learning Delayed recall Recognition Story memory Delayed recall Recognition Design memory Recognition Sentence memory

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Scorea 81 59 74 (6) (7) (7) (4) (2) (4) (5) (6) (8) (10)

Score

Percentile 10th 1st 4th

Percentile

2nd–10th <2nd <2nd

(2) (3) 75

2nd–10th 26th–75th <1st 5th

(1) 85 90 90 80 70 90 55 90 85

16th 25th 25th 9th 2nd 25th 1st 25th 16th

motor movements, and manual motor sequencing that extended beyond his simple manual motor problems. He completed a measure of visuospatial integration where he was to trace within a mazelike racetrack, and he made numerous errors and had

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difficulty pacing himself, impairing his performance. Although he had these difficulties, it was important to note that he had been unable to attempt this task at previous testing. Measures of visual and auditory attention were very difficult for Jeremy. He had numerous errors of omission and commission, indicating significant inattention and impulsivity. Observationally, he had difficulty sustaining attention for any significant period of time because he was highly distracted by any thoughts or sounds. Also, it was very hard for him to learn the rules needed to complete these tasks, such as ‘‘when you hear the word red, put the red piece in the box.’’ He consistently forgot the rules or was unable to apply them. For example, Jeremy completed a task that measures planning ability, sequencing, and strategy generation by requiring him to rearrange colored balls on pegs of various heights to match a presented goal configuration. He worked hard but tended to have perseverative responses and was unable to learn the basic rules. Jeremy’s ability to understand visual-spatial information was well below that expected for his age and a primary deficit resulting from his early brain injury. He had much difficulty on a measure that required him to merge an array of disjointed pieces of a familiar picture to identify the picture accurately. For example, he perceived pieces of a face as a leaf, a chair as a dog, and a hammer as a cross. He exhibited a similar level of difficulty when presented with an array of arrows and asked which two pointed to the center of a large target. On a task of spatial and tactile awareness in which Jeremy was blindfolded and asked to place various shapes in a form-board, he had some difficulty with problem-solving and becoming organized to complete the task successfully. However, he did not appear to struggle with the spatial aspects of the task, that is, he seemed to understand spatial relationships. This is consistent with his parents’ report that he has learned the location of keys on the phone to enter phone numbers. Jeremy’s performance on a verbal list-learning task was mildly impaired for immediate recall yet average after a delayed interval and on the recognition trial. This pattern suggests that his variable attention impacted his ability to learn the information; however, once the material was learned, he retained it quite successfully. Jeremy’s memory for verbal information presented in a context (e.g., stories) was mildly impaired for both immediate and delayed recall. However, he scored within the average range on the recognition task. His memory for sentences was mildly impaired. On tasks of nonverbal memory, his performance was mildly impaired for recall of faces and severely impaired for recall of geometric designs. Again, however, his performance was average when he was asked to recognize the geometric shapes instead of recall them freely from memory. Overall, Jeremy benefited greatly from cues provided during each recognition trial and scored well within the average range for both verbal and nonverbal information; this indicated that he was quite capable of forming new memories and was effectively retaining information over time.

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Formulation and Recommendations Results from this testing and observation of Jeremy indicated that activities often involving right hemispheric activities, such as engaging, disengaging, and sustaining attention, and executive functions that involve learning and applying rules to his emotional and behavior regulation continued to be severely impaired for Jeremy. Jeremy’s behavioral difficulties appeared to result primarily from the behavioral outcome of his impairments. Jeremy was delightfully verbal, with good verbal knowledge and memory. He was able to talk about many things in a very mature and age-appropriate way. The content of his verbalizations would often include words, excuses, and manipulations that he used to justify or distract him from his severe areas of impairment. Because his verbalizations were accurate rationalizations, he appeared to be very manipulative and disagreeable when it came to task completion. However, observation of his skills indicated that Jeremy continued to have extreme difficulty in learning and applying rules to behavior unless the reward was high enough to maintain his attention and motivation. Even when complying, he would easily lose attention and the ability to follow rules. This was an excellent example of how executive functioning and behavior are related and complimentary, yet not equivalent. With stricter behavioral management strategies, the parents, therapists, and teachers were able to decrease noncompliance while not alleviating the difficulties with executive functioning. Jeremy’s difficulties appeared similar to the behavioral regulation problems of a child with a developmental attention deficit hyperactivity disorder (ADHD). However, Jeremy’s attention difficulties and impulsivity were a primary result of his early brain injury and, therefore, differed from ADHD in several ways. They appeared more ‘‘severe’’ to those working with him. Also, he was not hyperactive. Jeremy’s presentation would fall under the diagnosis of acquired or secondary ADHD. Medication may help alleviate some of his difficulties. Children with neurological injuries resulting in primary attention/concentration and impulsecontrol problems often do not respond to pharmacologic treatments in the same way as children who have primary ADHD. Thus, management often requires trials of several different medications and dosages. Jeremy responded well to stimulant medication when he was 7 years old and learned to read shortly after treatment began. There was no decline in Jeremy’s overall functioning when his present performance was compared to that during previous evaluations. (Previous data were not presented here for the sake of brevity, but comparisons of raw score increases and steadiness of standard scores over time allowed these conclusions to be made.) To the contrary, he had shown a steady increase in skill development in all areas. In areas of strength, verbal knowledge, and reasoning, he had gained skills rapidly and maintained his standard scores. In areas of weakness, motor,

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attention, executive functioning, and visual processing, he made gains in raw scores, but his standard scores declined because they were less than expected for his age. Jeremy presented with an expected difference between verbal and nonverbal processing. His verbal knowledge and reasoning were low average whereas his nonverbal abilities were significantly below age expectation. He exhibited very rudimentary impairments in sensory (especially visual) and motor, attention, and executive functioning. Because nonverbal measures rely primarily on these underlying skills, they are responsible for these impairments. Jeremy’s spatial abilities, when the motor and visual components were removed, were less disrupted, based on parent report and testing. The course of Jeremy’s injuries and the long-standing residual neuropsychological, emotional, and functional outcomes are common in SBS (Barlow, Thomson, Johnson, & Minns, 2005; Duhaime, Alario, & Lewander, 1992; Ewing-Cobbs, Prasad, Kramer, & Landry, 1999). TBI is one of the leading causes of death and disability in childhood (Duhaime et al.), and inflicted head trauma, or SBS, is the leading cause of TBI in children under the age of 2 years. This type of injury cuts across all demographic lines and is almost always caused by someone the family knows. Some of the most common presenting symptoms are retinal hemorrhage, subdural hemorrhage, increased intercranial pressure, and seizures. If the child survives, the risks include persistent vegetative state, visual disturbances to blindness, mild motor impairments to paraplegia, and learning disability to all levels of intellectual disabilities. As can be imagined, the toll on families is huge, and of course, ongoing family factors such as functioning, mental health, and stress can in turn negatively influence the child’s recovery. The neuropsychological outcome relates to the severity of the injury. Although research is limited, children with SBS had impairment in cognitive, language, motor, and emotional and behavioral regulation (Barlow et al.; Ewing-Cobbs et al.). Several recommendations were made for the family. First, it was essential that Jeremy’s parents work with a therapist to improve their behavioral management of him to provide maximal rewards and emotional motivations to complete daily living skills and appropriate behaviors. It also was important that his new teachers review the current and previous evaluations to recognize that the discrepancy between Jeremy’s verbal skills and other areas of functioning are extreme and are responsible for his compliance issues in many situations. It was highly recommended that Jeremy be evaluated by his neurologist for possible pharmacologic treatment of his inattention and impulsivity. Although, as stated previously, some children do not respond similarly to children with developmental ADHD, others are quite successful. To address Jeremy’s weaknesses with visual-perceptual processing, the use of a systematically, verbally-based step-by-step approach to tasks was encouraged. Instruction, particularly for subjects that make relatively less use of language

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such as mathematics, should be broken into a list of verbal steps. It may be helpful to test Jeremy’s knowledge of academic material orally, asking him questions and having someone record his answers. Jeremy’s ability to recognize previously learned information was much stronger than his ability to recall that information freely, without structure. Therefore, it was suggested that he be provided cues or be offered information and asked to choose the best answer when being evaluated for his knowledge of academic material whenever possible. Because it appears Jeremy has relatively well-preserved spatial processing versus visual perceptual processing, he likely would be successful in learning to use a keyboard or other similar devices. Also, having him close his eyes might enable him to better focus, listen, and inhibit visual distractions.

Update Jeremy is now 9 years old. When he was age 7 years, he began having seizures. His parents reported that his sensory issues and attention problems returned to the level of difficulty they had been when he was initially seen at age 4 years. He underwent a right hemispherectomy and has been seizure-free for 6 months. He has not completed a postsurgical evaluation at this time.

Additional Resources Key Concepts and Terms Peritoneal shunt A mechanical device that diverts cerebrospinal fluid flow from the ventricles into the peritoneum, which is the serous membrane over the viscera and lining of the abdominal cavity. Retinal hemorrhages Bleeding in the retina. Shaken baby syndrome A syndrome seen in abused infants and children. The patient has been subjected to violent, whiplash-type shaking injuries inflicted by the abusing individual. This may cause coma, concussions, and increased intracranial pressure due to tearing of the cerebral veins with consequent bleeding into the subdural space. Retinal hemorrhages are usually present. Subdural hemorrhage Bleeding into the space between the arachnoid and dura mater membranes covering the brain.

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References Resources for Clinicians Barlow, K. R., Thomson, E., Johnson, D., & Minns, R.A. (2005). Late neurologic and cognitive sequelae of inflicted traumatic brain injury in infancy. Pediatrics, 116(2), 2004–2739. Duhaime, A. C., Alario, A. J., & Lewander, W. J. (1992). Head injury in very young children: mechanisms, injury types, and ophthalmologic findings in 100 hospitalized patients younger than 2 years of age. Pediatrics, 90, 179–185. Ewing-Cobbs, L., Prasad, M., Kramer, L., & Landry, S. (1999). Inflicted traumatic brain injury: relationship of developmental outcome to severity of injury. Pediatric Neurosurgery, 31, 251–258.

Resources for Families National Institute of Neurological Disorders Shaken Baby Syndrome. www.ninds.nih.gov/disorders/shakenbaby/shakenbaby.htm National Center on Shaken Baby Syndrome, www.dontshake.com Shaken Baby Association, Inc.,www.shakenbaby.net

Chapter 3

Grand Larceny in the First Grade: Traumatic Brain Injury in the School-Aged Years Jonathan E. Romain

Cody was the well-behaved, endearing, mild mannered, normally developing, 6-year-old girl any family would be blessed to have. The weather held the first sign of spring, and Cody’s mother was returning home from running errands with her only daughter, who just came along for the ride and to get out of the house. Cody was seat belted and well secured in the vehicle, as she is every time she goes on a family outing. As the car rounded the corner just before the family’s house, it slid and deviated into the shoulder of the road, rolling over. After the car stopped, Cody’s mother, who did not experience any loss of consciousness, exited the car and took stock of the situation. Somehow during the event, Cody had slipped from the grasp of her seat belt and gone through the side window, and she was ultimately found unresponsive several feet from the car. Cody’s mother, in a well-appreciated panic, scooped up her child and rushed to the closest neighbor’s house. After a quick call to 911, a helicopter arrived at the scene, and Cody was off to the local regional medical center. The initial Glasgow coma score en route to the hospital was 6, suggesting a severe brain injury (Table 3.1). Cody was initially spontaneously breathing on the scene, although at some point during the initial moments of hospitalization, her status declined and she required intubation. A computerized tomography (CT) scan of the head revealed active bleeding within aspects of the right frontal lobe, as well as the right middle portion of the base of the skull, extending to the right side of the skull. A small amount of subarachnoid blood and a moderate amount of subarachnoid air also were present, as a hematoma within the brain tissue. An increase in cerebrospinal fluid (CSF) necessitated a temporary catheter to relieve the pressure. Cody was ultimately stabilized and eventually extubated. A compounding injury during the crash was an extensive skull fracture of the facial bones, with the force of impact centering just above her right eye. Even though the long healing process was just beginning, there was already some mention that Cody might lose vision in the right eye. Cody’s mother recalls that when she first heard these details, she did not care about broken bones or the possible vision loss, only that her daughter awaken from her coma. Once she returned from this unintended sleep, Cody’s mom was sure she would have her daughter back.

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Table 3.1 Glasgow coma scale Eye opening Best motor response Best verbal response E M V Spontaneous Obeys commands Oriented and converses 4 6 5 To speech Localizes pain Disoriented and converses 3 5 4 To pain Flexion-withdrawal Inappropriate words 2 4 3 No response Flexion-abnormal Incomprehensible sounds 1 3 2 Extension No response 2 1 No response 1 E + M + V = 3–15 GCS of 3–8 is suggestive of a severe brain injury; GCS of 9–12 is suggestive of a moderate brain injury; GCS of 13–15 is suggestive of a mild brain injury.

Cody was transferred to a larger children’s hospital, where she underwent intensive inpatient rehabilitation care. To the surprise of her mother and practitioners on the unit, Cody made tremendous gains and quickly progressed from coma to active engagement in cognitive and physical activities within a 2-week period. Extensive repair and likely plastic surgery to reconstruct some of the facial features loomed, but this was still in the distant future. Cody’s right eye remained nonfunctional, and she had significant ptosis, although that was of little consequence to Cody’s mother, who was thrilled with the return of her child. She did raise some concern over Cody’s impatience and poor safety awareness on the unit, but as it is explained to all parents of children with closed head injury, some disinhibition early in the recovery was to be expected. When Cody was back home with her mother, the process of community and school reintegration began. The timing of the crash was relatively fortuitous (if there ever can be such a thing as perfect timing in an accident), because Cody had an entire summer to recover before the new school year. There was a problem, however. As Cody continued to recover, the poor impulse control persisted and actually appeared to worsen. Within the first few weeks of school, Cody was almost constantly reprimanded for verbal outbursts, ‘‘threatening’’ her peers, and even occasionally making obscene finger gestures to the teacher. She also engaged in seemingly bizarre behavior, including cutting a portion of her own hair with the teacher’s scissors and then becoming inconsolable when she realized what she had done. She also began taking things that were not hers with little thought of the consequences. Clearly, this transition back was anything but smooth. Things were not going very well on the home front either. Cody’s mother had prided herself on being a successful single mother and now she had her parents on speed dial to help her with the trials of raising a dysregulated child. Cody continued to retain much of her charm and good-natured personality; however, her persona

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was masked by impulsive actions that the well-behaved child would possibly consider but never actually do. Prior to considering a neuropsychological evaluation, a crude Individualized Education Program (IEP) was constructed. This document portrayed Cody as an oppositional and defiant child, who should be in the most restricted classroom environment. To be fair, Cody was new to the school, and the teachers and administration had no history for comparison. The mistake that was being made, however, was the failure to conceptualize Cody as a child with an acquired injury rather than as an inherently emotionally and behaviorally disturbed young girl. The problems continued to escalate as efforts were focused on almost constantly admonishing Cody for negative attention-seeking behavior. One day, just prior to the winter holidays, Cody was ‘‘apprehended’’ taking another child’s pencil. This was apparently the last straw, and at the request of the classroom teacher, a police officer was brought into the school to give Cody a stern lecture on the consequences of stealing. One can only imagine the scene of this young girl gazing up at someone in a uniform waxing on the merits of good citizenship. Upon hearing of this action, Cody’s mother decided it was time to reach for outside support, and she sought neuropsychological intervention.

Test Results Cody’s mother was referred to neuropsychology from the department of physical medicine and rehabilitation approximately 6 months following the crash. Her initial concerns included finding her daughter’s strong points and weaknesses and addressing her behavior. More specifically Cody’s mother wanted to know ‘‘what actions can be used for punishment.’’ By this question, it was clear that Cody’s mother’s limits were reached and that she had begun to adopt some of the same views as the school, which included immediate consequences for negative behaviors. No positive reinforcement schedule had been adopted at this point. In reality, it is quite possible that Cody was only rarely engaging in positive, on-task, and appropriate behaviors, making rewards and praise difficult to come by, which is often the case with children who have oppositional and defiant behaviors. The family arrived at the appointment with school records. A review of these documents revealed a litany of behavioral consequences and supportive data to suggest the need for Cody’s placement in the special education classroom. It appeared that formal psychoeducational testing was initiated but ultimately discontinued due to variable compliance. This was actually a positive finding in that the examiner was subsequently free to administer measures without concern for recent exposure or practice effects. The approach to this evaluation was to implement a flexible battery of neurocognitive tests, with emphasis on those areas commonly impacted by closed head injury with maximization of frontal lobe involvement (e.g., attention, language functions, memory and learning, and aspects of higher order reasoning or executive functions).

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Cody presented as a sweet, gregarious, bubbly, yet decidedly active and disinhibited young girl. She had a charming smile and a glint in her eyes that was visible beneath the facial disfigurement. A flurry of questions and statements streamed from her mouth: ‘‘Who are you?’’ ‘‘My name is Cody.’’ ‘‘We stayed in a hotel with a pool last night.’’ ‘‘That’s a nice shirt.’’ ‘‘Is that new?’’ This latter inquiry, in fact, turned out to be her most recent perseveration, and she proceeded to ask this question countless times about anything and everything throughout the morning. Despite her increased activity level and affinity for distraction and all things unrelated to testing, rapport was fairly easily established, and focus to task was maintained with the provision of strong external structure in a one-to-one, relatively barren testing environment (Table 3.2). The evaluation found Cody to be functioning within the borderline range of global cognitive abilities, with somewhat better developed verbal than nonverbal reasoning. Modest relative weakness was observed in speed of mental processing, and significant deficit was seen in verbal attention and working memory. The discrepancy between subtest scores was significant, rendering Cody’s Full Scale IQ as representative of ‘‘statistical averaging,’’ and although her overall IQ placed her within the borderline range, it was necessary to view her as having areas of significant relative strength and weakness, rather than as a child with globally depressed cognitive abilities. It is further essential in all evaluations of acquired brain dysfunction, particularly during the acute phase (approximately the first 6 months post‐injury) and post-acute stage (approximately 6–12 months post‐injury) of recovery that the scores be viewed as a level of current functioning and a baseline level of ability to track improvement in cognition over time. Due to time constraints and fatigue, only a screening of academic skills was completed (Table 3.3). Results revealed broadly average range single-word decoding, spelling, and mathematics skills. Although there was no assessment of fluency Table 3.2 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Scorea Verbal Comprehension Index 98 Perceptual Reasoning Index 84 Working Memory Index 65 Processing Speed Index 78 Full Scale IQ 78 Similarities (11) Vocabulary (9) Comprehension (9) Block Design (7) Picture Concepts (8) Matrix Reasoning (7) Digit Span (5) Letter-Number Sequence (3) Coding (5) Symbol Search (7) a Standard score, (scaled score), [T score], {z score}.

Percentile 45th 14th 1st 7th 7th

3 Grand Larceny in the First Grade: Traumatic Brain Injury in the School-Aged Years Table 3.3 Academic achievement Wide Range Achievement Test – Third Revision, Tan Form Reading Spelling Arithmetic

Score 101 95 94

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Percentile 53rd 37th 34th

or more intricate aspects of comprehension and applied reasoning, these results substantiated the fact that Cody was likely a broadly average range child before the injury. Notably, reading decoding is a commonly used measure to gauge a level of premorbid global functioning. Clinicians often find screening of basic math calculation skills also to be of some utility as children with acquired brain injury frequently demonstrate gaps for certain computations (e.g., forgetting portions of the multiplication table or how to solve for fractions). This of course was not operative in Cody’s case, because she only just had begun to learn the fundamentals of math, but it was encouraging that she had retained broadly average math skills relative to her grade. Performance on ancillary neuropsychological measures revealed impaired sustained attention and impulse control, as well as variable executive functions (Table 3.4). While sequencing and predicting cause-and-effect relationships on standardized neuropsychological testing were intact, abstract reasoning and reactive flexibility were impaired. Although somewhat variable, these findings are reflective of frontal lobe pathology consistent with a traumatic brain injury. Low average to impaired performance was also appreciated on measures assessing auditory comprehension and verbal memory functions; however, additional analysis and comparison between tasks suggest that these deficits were more a function of attention and working memory impairment, rather than language and memory deficits per se. Memory deficits further appeared to be a consequence of poor retrieval rather than an inability to encode information, as Cody’s remote recall of verbal material was solidly within the average range when she was given multiple-choice questioning.

Formulation and Recommendations In summary, Cody presented with deficits in sustained attention and impulse control, as well as aspects of executive functions, which is fully consistent with a brain injury and the identified frontal lobe focus. The results from the present evaluation provide a snapshot of her current condition. On the basis of the recency of her injury, Cody was still technically in the post-acute stage of recovery. Only during the long-term stage of recovery (beginning approximately 1 year after the injury) can a clearer picture of the potential for persistent deficits be fully appreciated. In this particular case, the neuropsychological data merely quantify, illustrate, and confirm Cody’s impaired self-regulatory behaviors impacting classroom and interpersonal functioning. This information was helpful in facilitating the school’s reconceptualization of Cody’s behaviors as a consequence of her acquired injury,

28 Table 3.4 Other cognitive functions Tests NEPSY Visual attention Conners’ Continuous Performance Test – Second Edition Omissions Commissions Hit RT standard error Variability Hit RT ISI change Hit SE ISI change Tower of London Wisconsin Card Sorting Test The Token Test for Children Part I Part II Part III Part IV Part V Overall score Grooved Pegboard Right hand trial 1 Left hand trial 1 Right hand trial 2 Left hand trial 2 Beery–Buktenica Developmental Test of Visual-Motor Integration Wide Range Assessment of Memory and Learning – 2 Story memory Immediate recall Delayed recall Recognition Verbal learning Immediate recall Delayed recall Recognition

J.E. Romain

Score 80

Percentile 9th

[119] 99th [36] 10th [87] 99th [77] 99th [96] 99th [66] 96th 97 42nd Discontinued 101 76 82 76 86 85

53rd 5th 12th 5th 18th 16th

91 81 104 94 85

27th 10th 61st 34th 16th

85 75 75

16th 5th 5th

80 9th 80 9th Affirmative response bias

rather than a function of bad parenting or merely a child behaving poorly. Several additional recommendations proved helpful and are presented in three categories: school placement, behavior, and adjunctive support.

School Placement It was strongly encouraged that Cody receive special education services under the traumatic brain injury (TBI) categorization. The general rule for children recovering from TBI is to provide maximum services initially and to taper off as

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necessary. This may appear counterintuitive, because the convention in school often is to provide the ‘‘least restrictive environment.’’ For Cody, a one-to-one aide would be reasonable, unless she demonstrates that she can work with a degree of independence and refrain from distracting others or engaging in negative interactions with children in the classroom. Students with TBI differ from others with disabilities in that they do not experience well-defined progress and they tend to show significant variability in their learning profiles. Instead of yearly progress reports and triennial reevaluation, students with TBI require more frequent evaluation and planning. Initially, the IEP might be revised every semester, and assessment might be reasonable as frequently as at 6-month intervals.

Behavior Cody clearly presented with limited tolerance for frustration, increased irritability, and an increased level of impulsivity, which, when combined, make it difficult for her to weigh consequences. Rather, she reacts impulsively, which is fully consistent with a brain injury. Therefore, it will be important for caregivers and school personnel to work with Cody to assist her in coping with conflicts to minimize her frustration. In addition, Cody will likely benefit from a behavior modification plan at home and in school. Such a program may be organized as a token economy and may also include patient and family counseling and social skills training. The primary goal of this type of program is to replace maladaptive behaviors with positive behaviors that generalize to everyday life and to limit aggression and negative emotional states. Any acting-out behavior should be confronted in a nonjudgmental, matter-of-fact way, pointing out what is appropriate or inappropriate and suggesting an alternative behavior. Avoid using unanswerable questions, such as ‘‘What were you thinking?’’ Additionally, any behavior program should focus on reducing the antecedent (preceding occurrence, cause, or event) that elicits the inappropriate behavior and reducing the probability that an inappropriate behavior (e.g., hitting) will be reinforced. Examples of proactive intervention strategies include the public posting of classroom rules and strategically placing the child near the teacher and away from other students who tend to act out. Further, it will be important to recognize and reward appropriate behavior immediately after it is demonstrated, which will promote Cody’s motivation and improve compliance.

Adjunctive Support During this evaluation, a lengthy discussion ensued about the potential consideration for a psychopharmacological consultation to evaluate the use of medication to improve attentional skills, decrease perseverative thoughts, and minimize

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disinhibited verbalizations and behaviors. Although the etiology of Cody’s inattentive and disinhibited behaviors is trauma related, symptoms of this type often effectively can be addressed medically in children with a history of closed head injury. Cody’s family was encouraged to work with physical medicine and rehabilitation, her primary care doctor, or perhaps a psychiatrist, who could provide medication management, such as a neurostimulant. It was strongly encouraged that counseling services be initiated through an outpatient setting to assist Cody in learning self-restraining strategies and in gaining insight into her impaired functioning, while preserving her self-esteem.

Update Approximately 1 year from her initial evaluation and after at least two reconstructive facial surgeries, Cody again came bounding into the office with the same wry smile and twinkle in her eyes (not to mention the incessant chattering) that has endeared her to people. Her right eye is permanently nonfunctional, but she remains medically stable, without evidence of recurring hydrocephalus or seizure. The perseverations persist, as does the distractibility, although her performance on formal testing did reveal modest improvements in most areas, with remarkably average to above average academic skills, suggesting that she is continuing to make age-appropriate maturational gains. Most importantly, the school appears to have made modifications to foster classroom growth and limit negative interactions, and Cody’s self-esteem and sense of self-efficacy appear to remain intact due to the parent and teacher shift from viewing her as an inherently bad child to one with an acquired injury. Toward the end of a long day of testing, with Cody still in active and chatty mode, she spontaneously exclaimed that she was doing a great job with her newest household chore – feeding, bathing, and generally exercising the family’s Labrador Retrievers and their first litter of puppies. Perfect.

Additional Resources Key Concepts and Terms Individualized education program (IEP) A written educational plan developed by school personnel, with input from parents and other professionals, for the provision of formal special education services for those who qualify. Neurostimulant Traditional stimulant medications such as those used for the management of ADHD, which have been gaining increasing favor in their use for improving attention and focus in traumatic brain injury.

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Perseveration A failure to inhibit a repeated response or thought. A common consequence of acquired brain injury due to the frequent involvement of the frontal lobes, but also seen with diffuse injury. Ptosis Drooping of a body part, in this case the eyelids; often the result of cranial nerve injury. Practice effects The recent prior exposure to a particular test resulting in an artifactual increase in performance during the subsequent administration. Seizure A disturbance of electrical activity within the brain, in this case secondary to TBI, resulting in a variety of presentations, some of which include altered consciousness and generalized motor manifestations, while others might manifest as staring, specific motor involvement, or merely strange sensory symptoms. Prevalence in the closed head injured population has been estimated to be about 5%. Token economy A reinforcement plan where a child earns ‘‘tokens’’ for good behavior to exchange as currency for a material reward or activity. These tokens also can be withheld or removed for noncompliance.

References Resources for Clinicians Bigler, E. D., Clark, E., & Farmer, J. E. (1997). Childhood traumatic brain injury: Diagnosis, assessment, and intervention. Austin, TX: Pro-Ed. Carney, J. M., Gerring, J. P., & Rosen, C. M. (1992). Head trauma: Strategies for educational reintegration. San Diego, CA: Singular Pub. Group. Savage, R. C., & Wolcott, G. F. (1994). Educational dimensions of acquired brain injury. Austin, TX: Pro-Ed.

Resources for Families Schoenbrodt, L. (2001). Children with traumatic brain injury: A parent’s guide. Bethesda, MD: Woodbine House. Senelick, R. C., & Dougherty, K. (2001). Living with brain injury: A guide for families (Second ed.). Birmingham, AL: HealthSouth.

Chapter 4

A Slippery Descent: Adolescent Traumatic Brain Injury Peter L. Stavinoha

With prom approaching in the spring, Judy’s high school planned several fundraisers to cover the cost of decorations. This included an annual fall carnival that typically attracted several hundred schoolchildren from small towns across several rural counties. Judy, a beautiful 15-year-old sophomore, was working atop the inflatable slide, where she was in charge of making sure that children safely made the transition from the top of the steps to the slippery descent. Judy gladly paid her dues by working the carnival, because she knew that her junior and senior proms would be supported by the efforts of underclassmen similar to herself. As Judy grew a bit weary of transferring one grade schooler after another from steps to slide, a breeze began to pick up. Hardly anyone paid much attention to the dark gray-blue line of clouds coming from the north, and those who did welcomed the cool air accompanying the cold front that was supposed to pass through that afternoon. However, as often happens on the plains, there was no warning that the front would bring with it a wall of wind at least 40 miles per hour strong. Run largely by amateurs, the carnival was not adequately prepared for this sudden change in weather. Feeling the cool breeze begin to pick up, Judy stood and arched her back to take advantage of a brief moment of respite between sliders. She had a great view of the rest of the carnival from her perch at least 25 ft. in the air. At that moment, the full force of the cold front hit, and the wind easily broke the slide from its moorings. Bystanders watched in horror as Judy fell backwards as the slide flipped, and she was subsequently thrown an estimated 150 ft. through the air. Judy landed on an awning attached to a trailer parked nearby, and she then bounced to the ground. Moments later, several adults surrounded Judy as she lay unconscious on the ground. Emergency medical services (EMS) were on the way, and the adults felt helpless as they stood and watched Judy’s nearly lifeless body. After just a few minutes, experienced EMS staff tended to Judy, quickly stabilizing her and racing to the hospital. Within 15 minutes of the fall, Judy began to regain consciousness and tried to speak to the EMS attendant riding with her in the back of the ambulance. Groaning in pain, Judy was agitated and made little sense when she spoke. The EMS technician made sure that Judy remained stable during the trip to the

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hospital, but he did not react to Judy’s agitation or confused speech because he had seen this before as a result of a head injury. Cranial computerized tomography (CT) scans completed on the day of the fall revealed a small hemorrhage in the medial aspect of the right frontal lobe near the midline. Judy’s brain was bleeding in the middle part of her right frontal lobe. A subdural hematoma was also observed and monitored over the left temporal lobe, and there was evidence of a small hemorrhage in the occipital horn of the right lateral ventricle. In addition, Judy experienced three broken ribs on her right side, a cracked pelvis, partial collapse of her right lung, and multiple breaks in her left arm. Judy remained in intensive care for 4 days. During the first 3 days following the fall, Judy remained disoriented and generally did not speak. When she did begin speaking, she was abrupt, curt, extremely demanding, and often did not make sense. Over the course of her 3-week hospital stay, Judy’s parents watched their daughter go from practically mute to unusually talkative. The doctors and the hospital staff had warned Judy’s parents that she may have some cognitive and behavioral difficulties as a result of her brain injury and that she may behave in a manner unfamiliar to them. Indeed, Judy’s parents were struck by the significant personality and behavior changes that they observed in their typically straight-laced daughter. At times Judy was irritable and agitated, while at other times she was loud, obnoxious, and lacking in personal modesty. Her speech had become somewhat more fluent as time went by, but it was often peppered with risque´ ideas and curse words. Judy’s parents found themselves shielding their 12-year-old daughter from Judy, because they found it very difficult to explain this behavior to their younger daughter. Judy was fortunate not to require surgery for any of her injuries, and she seemed to be making good physical recovery by the end of her hospital stay. No inpatient or day rehabilitation facilities were within 200 miles of their rural home, so Judy’s parents were handed the reins for her care as she continued her recovery at home. Practically from the time of Judy’s initial admission to the hospital, her primary care physician, who was also a friend of the family, had kept a close eye on her recovery and her care. Although closed head injury had not been a common malady that he had faced in his practice, Judy’s doctor knew that she may be in for a long and possibly incomplete recovery. Two weeks after coming home, Judy was adamant that she return to school, and her parents were unsure how to handle this. They had talked to school staff who had told them to give Judy time to heal before worrying about a transition back to school. Judy became more insistent that she return to school, and her parents were finding it increasingly difficult to distract her from this idea. They called her doctor for advice, and he suggested a consultation with a neuropsychologist. The closest neuropsychology services were approximately 300 miles away, but Judy’s parents were willing to make the trip to ensure that they were doing all they could to care for their daughter and promote her full recovery. During the initial interview with the neuropsychologist, Judy’s parents described her significant behavioral changes. In addition to the personality change characterized by a much more demanding and irritable tone, Judy seemed unconcerned with modesty. Her parents described her as having difficulty with memory and using odd

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speech patterns at times. They also felt that she had trouble understanding what people said to her, though they were not sure whether this was disorientation or truly a problem with comprehension. Judy continued to have trouble putting thoughts into words, and her parents described her as frustrated and irritable when she did not get what she wanted. At the time of the evaluation, Judy continued to experience difficulty with frequent headaches, dizziness, and occasional nausea. She was hypersensitive to noises and could not stay awake for more than 5 or 6 hours at a time. In fact, her parents said that Judy had been sleeping about 15 hours per day since coming home from the hospital. Judy’s parents told the neuropsychologist that they felt they needed help with ‘‘everything.’’ They did not know how to respond to, or understand, their daughter’s behavior, and they feared that these personality and cognitive changes would be permanent. They knew that she had to go back to school, but they could not see how she possibly could attend in her current condition.

Test Results Judy’s parents were seeking help across a number of dimensions. They wanted to understand the changes they had seen in their daughter, and they wanted to know whether these changes would be permanent. They wanted to know when and how to send Judy back to school, particularly given that she did not seem capable of staying awake for an entire school day. Judy’s parents expressed over and over, ‘‘We just want to know how to help our daughter.’’ Given the broad nature of the issues that needed to be addressed and the somewhat diffuse nature of Judy’s documented brain injury, evaluation procedures that could provide information about a wide range of neurocognitive domains were selected. Judy’s parents wisely traveled on the day before the evaluation so that Judy would have plenty of time to rest before undergoing testing. Judy was very friendly and sociable upon the initial meeting. She talked frequently throughout the day, and at times she spoke quickly and mixed her words together, making her speech output difficult to understand. However, when prompted, Judy would slow down and repeat what she had said, and this output was usually of improved quality. Although Judy was very pleasant during direct interaction with the examiner, her parents reported that during breaks she was irritable, oppositional, and even angry, frequently commenting that she did not want to return to testing. During testing, Judy was cooperative and appeared to put forth good effort. On more difficult tasks, she was not always able to sustain a high level of effort for very long, and she fatigued very easily over the course of the evaluation. Later, her parents reported that Judy slept almost 14 hours straight after she had completed the evaluation. Results of neuropsychological testing are summarized in Tables 4.1–4.3. Measurement of Judy’s overall intelligence only 7 weeks after her brain injury indicated average ability across both verbal and nonverbal domains. However, Judy’s subtest performances suggested increasing verbal difficulty as expressive language demands increased. Auditory working memory was in the low average

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Table 4.1 Intellectual Wechsler Intelligence Scale for Children, Third Edition Full Scale IQ Verbal IQ Performance IQ Verbal Comprehension Index Perceptual Organization Index Freedom from Distractibility Index Processing Speed Index Information Similarities Arithmetic Vocabulary Comprehension (Digit Span) Picture Completion Coding Picture Arrangement Block Design Object Assembly (Symbol Search) a Standard score, (scaled score), [T score], {z score}. Table 4.2 Academic achievement Woodcock Johnson Psychoeducational Battery – Revised: Tests of Achievement Letter-Word Identification Calculation

Scorea 90 92 90 95 91 87 91 (12) (8) (7) (10) (6) (8) (11) (8) (10) (8) (5) (8)

Percentile 25th 30th 25th 37th 27th 19th 27th

Score

Percentile

111 103

77th 58th

range, and Judy’s overall nonverbal information processing composite fell in the low end of the average range. Again, there was variability among individual subtests such that Judy experienced increasing difficulty as time demands increased and as the structure of the task decreased. Simple processing speed was in the low end of the average range, though qualitative observation suggested that Judy worked slowly and had difficulty persisting on longer tasks. Judy’s capacity to take in, store, and later recall newly presented verbal information in the form of a word list that she was to learn over repeated trials was significantly below average for overall learning. She also was noted to make a number of intrusion errors in that she had a tendency to say words that were not actually on the list more often than most individuals her age, and she had difficulty with the longer-term retention of this verbal information. Judy also experienced difficulty with memory for verbal information in the form of stories. Nonverbal, visual-spatial memory was also below average, and her aggregate memory performance suggested significant difficulty across memory modalities. Performance on tasks tapping language functions suggested difficulty with aspects of both receptive and expressive language functioning. Judy had difficulty with word retrieval and naming, and she also exhibited problems with oral language

4 A Slippery Descent: Adolescent Traumatic Brain Injury Table 4.3 Other cognitive functions Tests Clinical Evaluation of Language Fundamentals Concepts and directions Recalling sentences Controlled Oral Word Association Test Boston Naming Test Total Correct with phonemic cue California Verbal Learning Test – Children’s Version List A total trials 1–5 List A trial 1 free recall List A trial 5 free recall List B free recall List A short-delay free recall List A short-delay cued recall List A long-delay free recall List A long-delay cued recall Recognition Children’s Memory Scale Dot locations Learning Total score Long delay Stories Immediate Delayed Delayed recognition Faces Immediate Delayed Wisconsin Card Sorting Test Total errors Perseverative responses Perseverative errors Nonperseverative errors Trailmaking Test Part A Part B Beery–Buktenica Developmental Test of Visual-Motor Integration Benton Judgment of Line Orientation Test

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Raw score

Score

(6) (8) 81 31/60 12/29 [31] {1.0} {2.5} {1.0} {2.0} {1.5} {2.0} {1.5} {1.0}

(7) (6) (5) (6) (5) (8) (6) (5) 82 74 71 87 86 75 96 {0.33}

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comprehension, particularly as the complexity of the information given to her increased. Her basic visual perceptual functions were intact, and she also performed adequately on a task tapping visual-motor integration. Problem-solving and concept formation were difficult for Judy, because she had trouble self-monitoring her performance, flexibly developing and testing new problem-solving strategies, and altering her own solving approach based on direct feedback. She also was observed to have significant difficulty on a task of divided attention, which required her to simultaneously shift back and forth between familiar sequences. During an interview with Judy to gather information about her emotional adjustment and current coping, she reported that she believed her attitude has changed significantly since her fall. Specifically, she said that she now believes that life is short and needs to be lived to its fullest. Judy’s interview responses suggested that she did not have a grasp of the gravity of her fall, and in fact she tended to deny any injury aside from obvious physical injuries (e.g., broken arm, broken ribs). She felt that she was still the same person and was still as capable as ever. She could not understand why her parents were ‘‘smothering’’ her, but at other times during the interview she acknowledged feeling frightened by all of the significant and sudden changes in her life.

Formulation and Recommendations Judy exhibited a diffuse pattern of neurocognitive deficits that are generally congruent both with the experience of closed head injury in general as well as with the specific areas of brain damage that she experienced. Ongoing difficulties with low stamina, fatigue, and somnolence are not unusual in the early phase of recovery following closed head injury. Judy also continued to experience short episodes of disorientation and occasional dizziness, along with persisting headaches. Again, these symptoms are not unusual during the initial weeks and months of recovery from brain injury, and these are all conditions that need to be considered when making appropriate rehabilitation and educational plans. Cognitively, Judy appeared to exhibit a general suppression of overall IQ performance. Measurement of IQ is not always warranted during the initial months of recovery because it is often not particularly stable, but it was important to have an overall measure of ability for Judy both for later comparison to document recovery and for educational planning. Judy exhibited significant difficulty with memory functions as well as with aspects of both expressive and receptive language, likely related to diffuse brain injury and specific injury to the left temporal lobe. Deficits in executive functions including problem-solving, self-monitoring, and self-regulation of behavior likely stemmed from Judy’s right frontal lobe damage observed on initial CT scans. In addition, Judy intermittently exhibited difficulties with attention, concentration, cognitive speed, and efficiency, neurocognitive effects that are relatively common following traumatic brain injury. Emotional and personality issues were prominent and particularly troubling for Judy’s family. Their once good-natured and pleasant girl had become an irritable,

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impulsive, and even obnoxious teenager in the course of a single afternoon at a carnival. Personality changes also are not unusual following damage to the frontal lobe, and on the surface Judy gave the impression that she lacked much insight into her functional deficits. However, in talking to Judy at length, she seemed to vacillate between wanting to be taken care of and needing to prove to herself and others that she was still the successful and independent young woman that she had always been. During the feedback conference held with Judy’s parents, the discussion began with the rapid trend of Judy’s recovery since the time of her fall. Judy’s parents described her improvement as dramatic during the first several weeks and somewhat less rapid currently, though they still felt she was making noticeable improvements from one day to the next. Judy’s parents were reassured to learn that she was still relatively early in recovery from her brain injury and should continue to exhibit noticeable improvements in the weeks and months to come, even though there was still a good chance that Judy would exhibit some long-term effects of her brain injury. Because there were no rehabilitation facilities available to the family, it was determined that Judy would likely best be served by returning to school, where she would have access to structured activities that could help promote recovery of function. Additionally, returning to school was encouraged to help Judy get back into a structured daily routine. From a physical perspective, it seemed clear that Judy would not have the stamina to return to school full-time. Therefore, a transition plan was constructed that allowed Judy to attend school only part-time at first, with an eventual full-time return as the final goal. Specifically, it was determined that initially Judy would attend school for only 2 hours per day for several days, and during this time she would not be expected to produce any academic work. The priority was physical attendance at school to build her stamina, not educational performance. A graduated schedule was put into place by which Judy would eventually return to school full-time within several weeks, though academic demands were to increase at a much slower rate, based solely upon Judy’s demonstration of her capacity to manage these demands. Additionally, academic work that had accrued since the time of Judy’s fall was to be set aside for completion at a later time if necessary. Some students who have missed a great deal of work become almost obsessed with completing that work, seemingly as a demonstration to themselves and others that they are not experiencing any aftereffects of their brain injury. Although it is admirable that a student would be so interested in completing missed work, this can be a huge energy drain on the student during recovery to the point of interfering with longer term reintegration and adjustment. In Judy’s case, she was told that the work would be waiting for her the following summer, and she would have ample opportunity to complete fully any outstanding work prior to starting her junior year in high school. To allow for such flexible scheduling and alteration of curriculum expectations, Judy was formally identified by the school’s multidisciplinary special education team as eligible for services under the handicapping condition of Traumatic Brain Injury (TBI). Speech and language evaluation and monitoring were recommended to address lingering language issues. Judy’s school psychologist offered to provide

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case management, teacher consultation, and oversight to ensure that Judy did not reach excessive frustration or fatigue during her lengthy transition back to school and recovery from brain injury. Fortunately for Judy, the school psychologist seemed well aware of the balance that needed to be struck between having Judy participate in normal, everyday school activities while at the same time protecting her from feeling overwhelmed and incompetent. A number of modifications and accommodations were brainstormed to address specific difficulties with memory, attention, processing speed, and organizational capacity. These are summarized in Table 4.4. It is not unusual for an adolescent to experience a strong psychological reaction to a potentially life-threatening incident. For Judy, this was further complicated by the fact that she did not entirely remember the fall and instead had to trust the stories others had told her about what happened. As with many adolescents, during the course of her recovery Judy vacillated between yearning for full independence while also wanting her parents to take care of her. Additionally, Judy’s parents were struggling with their reaction to nearly losing their daughter and with changes in communication patterns between themselves and Judy resulting from the personality changes secondary to her traumatic brain injury. Although it was anticipated that some of these personality changes would resolve to an extent given time, it was recommended that they work with a psychotherapist on these issues in the interim as Judy continued her recovery. Resources in the family’s geographic region were sparse, so a therapist was identified who would spend some time with Judy individually as well as with Judy and her parents together. Finally, given the fluid nature of recovery from traumatic brain injury, it was anticipated that Judy would experience significant recovery of function over the next several months. Neuropsychological consultation was recommended as a checkup at 2-month intervals for the next 6 months, with a full evaluation scheduled approximately 12 months after injury. The 12-month interval would allow the bulk of significant recovery to occur, and this would permit greater clarity in the identification and planning for any neurocognitive deficits that would potentially be long-standing. Table 4.4 Classroom strategies Reduce or eliminate time demands on assignments and tests Begin with smaller assignment sizes/demands and gradually return to normal academic demands as Judy demonstrates mastery Present new information in a structured and organized manner, multiple times, and in multiple modalities when possible Provide opportunities for Judy to rest during her school day Help Judy prioritize and organize her assignments so that these are broken into discrete parts that she can complete one at a time in a sequential manner Help Judy establish realistic assignment completion goals, and help her track progress toward these goals Provide prompts and cues as needed to ensure that Judy is understanding directions and what is expected for specific assignments Teacher notes should be provided to Judy so that she is not expected to take notes in lecture classes, a task that likely would exceed her working memory and processing speed capacity

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Update Judy was seen for evaluation once again approximately 12 months after her closed head injury. Her parents reported that she was bubbly and active and seemed to be ‘‘her old self.’’ She continued to sleep longer and more soundly than she had before the fall, and occasionally she would exhibit mild disorientation upon awakening. Judy progressed well at school during that year, and by the end of her sophomore year, she was back to school full-time, taking a full academic load. She continued to experience difficulty with more academically challenging activities, and these continued to cause her some frustration. However, with ongoing support, she was managing her assignments and seemed to be coping adequately. Updated evaluation indicated a 16-point increase in IQ and high average performance across academic achievement tasks. Judy continued to exhibit mild difficulties with working memory and retention for new information. She also continued to exhibit a slight weakness with information processing speed that became much more prominent as the level of task difficulty increased. Psychologically, Judy seemed to have assimilated her difficult experience and long, effortful recovery reasonably well, and she seemed to be coping positively with new life challenges with ongoing support and help from her family, educators, and therapist.

Additional Resources Key Concepts and Terms Hemorrhage Bleeding due to ruptured blood vessels. Somnolence A state of being drowsy or sleepy. Subdural hematoma A pooling of blood beneath the dura, one of the protective coverings for the brain under the skull. The blood collects and pools such that it expands like a balloon filling with air, and it causes pressure on the brain.

References Resources for Clinicians Semrud-Clikeman, M. (2001). Traumatic brain injury in children and adolescents: Assessment and intervention. New York: The Guilford Press. Yeates, K.O. (2000). Closed head injury. In K.O. Yeates, M.D. Ris, & H.G. Taylor (Eds.), Pediatric neuropsychology: Research, theory, and practice (pp. 92–116). New York: The Guilford Press.

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Resources for Families Brain Injury Association, Inc., www.biausa.org. Promotes awareness, understanding, and prevention of brain injury and provides education, advocacy, and community support services for those affected by brain injury. 1776 Massachusetts Ave., NW, Washington, DC 20036‐1904. Family Helpline 1-800-444-6443. Brain Injury Information Network, www.tbinet.org. An Internet-based network of electronic mailing lists, information, and support groups for those affected by brain injury. Brain Injury Society, www.bisociety.org, (718) 645–4401. An organization that works with clients, families and caregivers to identify strategies and techniques to improve outcome and recovery from any type of brain injury. Traumatic Brain Injury Survival Guide, www.tbiguide.com. An online publication that provides easy-to-understand language for survivors and families coping with a traumatic brain injury.

Chapter 5

Steamrolled: Sports-Related Concussions Michael Sharland and Thomas A. Hammeke

Stephen played inside linebacker for his high school junior varsity football team. His teammates called him the Steamroller because he knocked other players flat. During the final scrimmage prior to the regular season, Stephen sustained a concussion during play. He was not rendered unconscious but was immediately dizzy and later had nausea. He was removed from play and allowed only limited practice for the week. His symptoms resolved, and he returned to play in early September, 2 weeks following his injury. He did not have a clear concussion in this return game but did strike his head on the ground on several occasions while diving for the ball. His symptoms returned and he felt markedly fatigued; consequently, he was again removed from play. His parents were concerned because this was not Stephen’s first concussion, nor even his second. In fact, according to their count, Stephen had sustained four concussions since childhood (Table 5.1). Stephen’s primary care physician saw him for follow-up care and released him to return to play in mid-September. But his parents elected to withhold him from play until they obtained a second opinion from a pediatric neurologist. The neurologist saw Stephen in late October, 8 weeks following his injury, and at that time Stephen’s parents noted that his grades had suffered. Magnetic resonance imaging (MRI) of the brain was ordered and interpreted as normal. Additionally, Stephen’s neurological exam was normal. The neurologist referred Stephen to the Mild traumatic brain injury (TBI) Clinic at a regional academic medical center, where Stephen was seen in November. The Mild TBI Clinic is staffed by a physiatrist and a neuropsychologist. Its purpose is to evaluate and assess individuals shortly after sustaining a mild brain injury with the goal of early intervention preventing longer-lasting complications. For example, the Mild TBI Clinic doctors will make recommendations about readiness to return to work, treat mood disturbances associated with TBI, and provide education and follow-up monitoring of common TBI-related sequelae. The neuropsychological battery in the Mild TBI Clinic is quite brief, taking approximately 20 min to administer and including measures of memory, attention, processing speed, and executive functioning, domains which have shown to be sensitive to mild TBI. Stephen lived with his mother and father and was attending tenth grade. He had a cumulative grade point average of 2.75 with a 2.90 grade point average over

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Table 5.1 Stephen’s concussion history Age Nature of concussion (in years) 6

12

14

15

Collided with another child while running around the corner of his house Accidentally hit in the head while playing baseball Collision during practice for freshman football resulting in helmetto-helmet contact with another player Hit head while playing in scrimmage for junior varsity football

Loss of consciousness Yes, briefly (less than 1 min)

Symptoms following concussion Headache, sleepy

No

Dizziness, headaches

Yes, briefly (reportedly 5–10 s)

Brief amnesia for events following, headaches, sensitivity to noise Dizzy, nauseated, easily fatigued, headaches

No

the past semester. He averaged mainly Bs and Cs and struggled with Spanish and Geometry. These grades were slightly lower than his performances in ninth grade. His performances on standardized academic testing were consistently in the average range. A more thorough interview of Stephen’s parents revealed mild concerns regarding inattention since childhood; however, he was never diagnosed with an attentional disorder. Developmentally, he was born 2 weeks premature and delivered by C-section. He reached all developmental milestones within normal limits. He never received special education services or repeated a grade. He was active in various athletics. Past medical history was significant for Von Willebrand disease, resulting in reduced blood-clotting ability, and the aforementioned four concussions. Family medical history was significant for dementia/memory loss in a maternal great-grandparent and great-uncle in late life. It was not clear whether this was related to vascular disease or a dementing process such as Alzheimer’s disease. Past psychiatric history was unremarkable, and Stephen’s mood was reported as normal. He was not currently on any medication. TBI can be divided roughly into three levels of severity. Individuals who sustain a mild TBI typically have little or no loss of consciousness, brief or no loss of memory for events around the time of the injury, and are alert and oriented on arrival to the emergency department. People who sustain moderate and severe traumatic brain injuries will have loss of consciousness, posttraumatic amnesia, visible damage to the brain on neuroimaging, and be less lucid and alert immediately after the injury. Although strict delineations between injury severity are not always clear, medical professionals typically use an individual’s performance on the Glasgow Coma Scale, a gross measure of level of arousal and lucidity, on arrival to the emergency department as a means of estimating injury severity. Scores that range from 3 to 8 on this scale are identified as severe TBI, 9 to 12 as moderate TBI, and 13 to 15 as mild TBI.

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Test Results On presentation to the Mild TBI Clinic, Stephen stated that his physical symptoms had fully resolved; however, he did note difficulty sustaining his focus in the classroom and stated that he had to work harder mentally to do his homework. He reported that his mental stamina had not fully recovered. He was not aware of any specific problems with memory, speech, or reading. Taste and smell were said to be normal and his appetite had been good. Sleep had been good and perhaps increased over his normal amount. He was not troubled by nightmares. No significant change in mood or temperament was reported by Stephen or his parents. Stephen was evaluated by both a physiatrist and a neuropsychologist. His physical exam was unremarkable. He was administered the short screening battery of neuropsychological measures. Mild deficits were noted on a wordlist learning task and on measures of verbal working memory and information processing speed. These skill weaknesses, although mild, were consistent with his complaint of difficulty in sustaining concentrated focus in his class work. Additionally, the deficits implied that he must work harder mentally to complete routine tasks, perhaps accounting for his complaint of diminished mental stamina. Stephen’s test performance (Table 5.2) raised concerns about persistent effects from his concussion or, possibly, cumulative effects from the four concussions he had incurred. Stephen was advised to avoid contact sports at the time because repeated concussions without full recovery have shown cumulative effects. A repeat examination with a more expanded set of tests was suggested for the following spring to better gauge recovery versus persistent deficits. Additionally, the estimated hazards of contact sports would be reconsidered and a recommendation about playing football provided. Stephen was allowed to continue competing in swimming and track. Stephen returned for a more thorough neuropsychological evaluation and assessment in February, 5 months following his original injury. He arrived for his appointment accompanied by his mother and father. He was an alert, well-dressed, and pleasant young man. On interview, Stephen denied any cognitive deficits. He spoke fluently, with no abnormalities in the volume, rate, or tone of his voice. Table 5.2 Initial screening: Other cognitive functions Tests Hopkins Verbal Learning Test Total recall Delay Recognition Wechsler Adult Intelligence Scale – Third Edition Digit span Symbol digit Animal Naming Trailmaking Test Part A Part B a Standard score, (scaled score), [T score], {z score}.

Scorea

Percentile 5th–16th 5th 16th–25th

90 85 84

25th 16th 14th

99 114

47th 82nd

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His ability to understand complex instructions and conversations appeared normal. His thinking ability was clear and to the point. His emotional expression was appropriate, and he was able to appreciate and use humor. He did not exhibit any behaviors that would raise concern for a thought disorder. His ability to walk was normal, and his insight and judgment appeared age appropriate. Neither he nor his parents reported any problems with his memory, attention, judgment, speech, or other cognitive areas. Still, his father stated that Stephen had difficulty motivating himself to complete homework assignments and try his best in difficult courses. His father reported that these problems had partially remitted in the past month. Stephen stated that his energy level had improved and that he had not experienced any headaches recently. The only exception to this was a headache that occurred when shaking his head during a dancing exercise a few weeks earlier. His parents also expressed their opinion that he had fully recovered. They brought Stephen’s medical and academic records for review. Stephen was administered a more comprehensive battery of neuropsychological measures than during his visit to the Mild TBI Clinic. While his performance from the Mild TBI Clinic acted as a screening for potential problems, his current neuropsychological assessment was designed to more fully explore his pattern of strengths and weaknesses. The battery consisted of tests of verbal reasoning, spatial reasoning, verbal and nonverbal memory, verbal fluency, mental flexibility, concept formation, sustained attention and distractibility, and depression. In general, Stephen performed in the average to above average range in almost all cognitive domains. He worked hard on difficult tasks, did not give up easily when faced with challenges, and did well on tests specifically designed to detect poor effort. As such, Stephen’s performance was considered a reliable and valid representation of his current neuropsychological functioning. On a wordlist learning task, he showed a mildly slow learning curve (2nd percentile) with delayed recall being mildly below expectation (5th percentile). A measure of recognition memory was also mildly below expectation (16th percentile). In contrast, Stephen exhibited good immediate and delayed memory for orally presented stories and visually presented figures, suggesting good memory functioning in general. Measures of attention, span memory, and category fluency were below average, with a performance profile that suggested attentional deficits. On a brief depression screening measure, Stephen did not endorse items reflective of depression. Compared to his prior screen, his performance was essentially unchanged, except for improved processing speed and mental flexibility on a visual sequencing task (Tables 5.3–5.5).

Formulation and Recommendations Stephen’s cognitive profile revealed persistent subtle weaknesses in attention and span memory. His performance was also weak on an effortful memory task (learning a word list); however, his pattern of performance on this task was more

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Table 5.3 Intellectual Wechsler Adult Intelligence Scale – Third Edition Similarities Information Block Design Digit Span

Score 110 110 125 90

Table 5.4 Academic achievement Wide Range Achievement Test – Fourth Edition Word Reading Spelling

Table 5.5 Second testing session: Other cognitive functions Tests Wechsler Memory Scale – Third Edition Logical memory I II Recognition Visual reproduction I II Recognition Hopkins Verbal Learning Test Total recall Delayed recall Discrimination Symbol Digit Modalities Test Written Oral Paced Auditory Serial Addition Test I II Gordon Diagnostic System Vigilance task Distractibility Controlled Oral Word Association Test Animal Naming Trailmaking Test Part A Part B Wisconsin Card Sorting Test Categories Perseverative responses Loss of set Errors

Score 103 106

Percentile 75th 75th 95th 25th

Percentile 58th 66th

Score

Percentile

100 105

50th 63rd 72nd

115 110 100

84th 75th 50th 2nd 5th 16th

85 83

16th 13th

88 90

21st 25th

95 88

50th 50th 37th 21st

111 133

77th 99th

127 120

>16th 96th >16th 91st

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reflective of attentional weakness. He showed little or no change on the tasks he had completed in November when he was seen through the Mild TBI Clinic. Thus, his neuropsychological performance was more likely reflective of his baseline cognitive functioning. The pattern of performance was consistent with subtle longstanding attentional difficulties reported by his parents, but they were not severe enough to warrant a clinical diagnosis of an attention deficit disorder. In this case, it was very important to compare Stephen’s neuropsychological performance across time, examine his previous academic history, and obtain a thorough interview with his parents. Subtle attentional difficulties can appear very similar to the acute effects of a mild TBI. However, his pattern of performance did not change during the interval between testing sessions. If the effects were truly due to his most recent concussion, one would expect some improvement on measures. It was also possible that his neuropsychological performance reflects the cumulative effects of multiple concussions versus attentional problems. In this instance, the information provided by his parents and academic records were helpful in differentiation. If Stephen’s current neuropsychological performance were truly due to the cumulative effects of concussion, one would expect to see a step-wise decline in his academic and cognitive abilities with each successive concussion. Instead, Stephen’s standardized academic testing suggested a relative weakness in reading and verbal cognitive abilities dating back to the second grade. Additionally, his parents stated that he had had symptoms of inattention since childhood. Hence, his history was inconsistent with the cumulative effects of concussion. In addition, all of Stephen’s concussions were generally mild and spaced apart by years. This makes any cumulative effects of concussion much less likely, because severity and temporal clustering best predicts cumulative cognitive effects of concussion. These conclusions were shared with Stephen and his parents, including the opinion that he had made a good recovery from his concussion. It appeared Stephen was safe to return to all athletics, should he choose. However, he was cautioned to minimize further risk of concussion. It was also recommended that he return for a repeat neuropsychological evaluation should he sustain an additional concussion. His parents decided that he should not participate in contact sports at this time. Stephen accepted his parents’ decision and focused on participating in other sports such as baseball, soccer, swimming, and track. Although the current test findings and conclusions did not necessitate this decision, the decision was nonetheless the safest course of action.

Additional Resources Key Concepts and Terms Concussion A mild traumatic brain injury characterized by a transient alteration in mental functioning that is often associated with at least a brief loss of consciousness or brief interval of posttraumatic amnesia.

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Posttraumatic amnesia A period of anterograde amnesia in which new memories cannot be consistently made and recalled following recovery of consciousness in head injury or other neurological trauma. Traumatic brain injury Brain dysfunction caused by an external mechanical force such as a blow to the head, concussive forces, acceleration–deceleration, or projectile missile. Von Willebrand disease The most common inherited bleeding disorder. It results from a lack of Von Willebrand factor, which helps blood clot. People with this disorder can exhibit prolonged bleeding after an injury.

References Resources for Clinicians Barr, W. B., & McCrea, M. (2001). Sensitivity and specificity of standardized neurocognitive testing immediately following sports concussion. Journal of the International Neuropsychological Society: JINS, 7(6), 693–702. Echemendı´a, R. J. (2006). Sports neuropsychology: Assessment and management of traumatic brain injury. New York: Guilford Press. McKeever, C. K., & Schatz, P. (2003). Current issues in the identification, assessment, and management of concussions in sports-related injuries. Applied Neuropsychology, 10(1), 4–11.

Resources for Families Centers for Disease Control and Prevention National Center for Injury Prevention and Control. TBI – traumatic brain injury home page, http://www.cdc.gov/ncipc/tbi/TBI.htm United States Department of Health and Human Services Centers for Disease Control and Prevention. Heads up: Concussion in high school sports, http://www.cdc.gov/ncipc/tbi/Coaches_ Tool_Kit.htm

Chapter 6

Bad Signs: Sickle-Cell Disease and Stroke Kimberly Rennie Weissgerber and Amanda Epping

Johnny was a delightful little boy who was born with sickle-cell disease, an inherited genetic disorder that affects hemoglobin, a protein in the red blood cells responsible for transporting oxygen. Normal red blood cells are soft and round. In sickle-cell disease, the red blood cells are hard and sickle in shape. Consequently, they are likely to get stuck within blood vessels, causing severe pain and other medical complications. Specifically, sickle-cell disease is associated with increased incidence of vascular occlusions (pain crisis), cerebrovascular occlusions (stroke), infections, pulmonary difficulties, and organ damage. Some individuals with sicklecell disease experience few complications, while others experience many. Unfortunately, for Johnny and his family, he experienced many sickle-cellrelated complications. Johnny spent much of his 10 years of life in and out of hospitals. During his first year of life, he experienced a condition called dactylitis, the swelling of the fingers and toes. Many pediatric hematologists (doctors who work with children who have blood diseases like sickle-cell disease) consider dactylitis to be a ‘‘bad’’ sign (i.e., individuals who experience dactylitis in their first year of life are at higher risk for more severe complications of the disease than those who do not). Johnny also experienced a condition called acute chest syndrome (a new abnormal finding on a chest X ray and associated with a low oxygen level), a severe complication of sickle-cell disease that may increase the risk of stroke. If sickle-cell disease was not enough, Johnny also suffered from asthma, something not uncommon in children and adolescents with sickle-cell disease. Despite his illnesses, Johnny and his family maintained a positive outlook on life. They were all quite close and got along well. Both his mother and his siblings were quite supportive of him. Aside from his sickle-cell disease and asthma, Johnny was a relatively healthy little boy. However, his mother did have some concerns about Johnny’s development. Johnny had some trouble learning to walk, and although he began speaking at a developmentally appropriate age, he had persistent difficulty pronouncing words correctly. Although he was doing well academically and did not require any special services at school, his mother remained concerned about the impact of Johnny’s sickle-cell disease on his overall development. Thus, his mother brought Johnny in for an evaluation.

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Approximately 2 years after being seen for his initial evaluation, at the age of 10 years, Johnny had a brain magnetic resonance imaging (MRI) test that confirmed the presence of a focal silent ischemic infarct (defined as the presence of an abnormal imaging study of the brain with a normal neurological exam and no prior history of any physical findings) in the prefrontal cortex, with greater prominence on the left side of his brain. His mother also reported that Johnny was not doing as well academically as he had in the past. His grades decreased from As and Bs to Cs and Ds, and Johnny was going to be repeating the fourth grade because he had failed a school district assessment. New concerns were also noted about Johnny’s behavior at school. Results of Johnny’s initial and follow-up evaluations are presented below.

Test Results Initial Evaluation At the time of his initial evaluation, Johnny was almost 8-years old. As the testing session began, he was somewhat shy but he eventually warmed up and became quite talkative with the examiner. Johnny seemed to enjoy the testing and laughed and smiled with the examiner throughout the session. He had difficulty staying on task and was easily distracted, but he was a hard worker. Johnny and the examiner worked out a system whereby he would earn stickers for his efforts. He seemed particularly fond of this method. Johnny became a bit restless toward the end of the session and had some trouble sitting still. At times, he would move around quite a bit in his chair and stick his head under the armrest. He was always easily redirected back to the task at hand, however. It was difficult to understand Johnny as, when speaking, he tended to cover his bottom teeth with his bottom lip. He responded well when asked by the examiner to repeat himself. Overall, Johnny was a pleasure to work with and seemed to put forth his best effort (Tables 6.1 and 6.2). Although a comprehensive evaluation was recommended for Johnny at the time of his initial evaluation, only measures of intellectual functioning and academic achievement were completed. Attempts to complete the evaluation were unsuccessful because his mother was unable to bring Johnny back. On a measure of intellectual functioning, Johnny’s performance fell within normal limits. He exhibited a relative weakness on measures of perceptual reasoning, as well as on tasks of verbal comprehension. In working memory, Johnny’s ability fell solidly within the average range. Likewise, Johnny performed within normal limits in processing speed. Overall, results indicated that Johnny generally had well-developed intellectual functioning with relative weaknesses in some aspects of verbal comprehension and perceptual reasoning. On measures of academic achievement, Johnny’s reading skills fell within the average range, with equally well-developed sight-word recognition and reading fluency skills. Johnny’s

6 Bad Signs: Sickle-Cell Disease and Stroke Table 6.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal Comprehension Index Similarities Vocabulary Comprehension Perceptual Reasoning Index Block Design Picture Concepts Matrix Reasoning Working Memory Index Digit Span Letter-Number Sequencing Processing Speed Index Coding Symbol Search Full Scale IQ a Standard score, (scaled score), [T-score], {z-score}. Table 6.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition Letter-Word Identification Reading Fluency Calculation Math Fluency Math Calculation Skills

53

Scorea 87 (8) (5) (10) 90 (5) (11) (9) 99 (10) (10) 97 (9) (10) 89

Score 99 96 91 83 89

Percentile 19th

25th

47th

42nd

23rd

Percentile 47th 39th 27th 13th 23rd

basic math skills fell within the average range. His lowest score was on a measure of math fluency and fell within the upper end of the below average range, suggesting that Johnny had not yet mastered basic math skills (i.e., basic math concepts such as multiplication tables were not yet automatic for him). Results of Johnny’s achievement skills were generally consistent with his overall level of intellectual functioning.

Follow-up Evaluation At the age of 10, Johnny was seen for a follow-up evaluation to assess the impact of his infarction on his neuropsychological functioning. Similar to his first evaluation, Johnny presented as a quiet but friendly child. He remembered ‘‘playing the games’’ from his initial evaluation, and was eager to begin testing. He put forth good effort and it was a delight to work with him. In light of Johnny’s infarction and associated changes in academic and behavioral functioning, he was administered a comprehensive neuropsychological battery designed to assess areas of cognitive functioning that may have been impacted by his infarction. Language testing was not conducted because this was being done by his school district (Tables 6.3 and 6.4).

54 Table 6.3 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal Comprehension Index Similarities Vocabulary Comprehension Perceptual Reasoning Index Block Design Picture Concepts Matrix Reasoning Working Memory Index Digit Span Letter-Number Sequencing Processing Speed Index Coding Symbol Search Full Scale IQ

K.R. Weissgerber, A. Epping

Score 79 (7) (5) (7) 82 (7) (9) (5) 83 (7) (7) 91 (8) (9) 78

Percentile 8th

12th

13th

27th

7th

On Johnny’s follow-up evaluation, his overall level of intellectual ability fell within the below average range, with a relative strength in processing speed. Compared with previous testing, Johnny’s level of intellectual functioning decreased noticeably. Johnny did not exhibit any deficits on a measure of sustained visual attention in the structured one-on-one testing setting. His performance on measures of rote verbal and visual memory fell within normal limits. Johnny’s performance on a measure of executive functions fell within gross normal limits, suggesting intact planning, foresight and flexibility, and an ability to utilize feedback to generate problem-solving strategies. However, difficulties were reported with Johnny’s ability to regulate his behavior, inhibit inappropriate behavioral responses, and initiate tasks. Johnny’s performance fell within the below average range in graphomotor ability, visual processing, and motor coordination. Significant impairments in Johnny’s fine motor speed and dexterity in his right hand while maintaining a performance level within gross normal limits with his left hand were noted. Concerns with depression and withdrawal were also noted.

Formulation and Recommendations On his initial testing, results generally indicated that Johnny had intact intellectual functioning and average academic performance. No concerns were noted with emotional and behavioral regulation. At that time, he was also doing well in school, and aside from articulation difficulties, no significant concerns were noted regarding his overall development. In his follow-up testing, there was a notable decrease in Johnny’s performance on a measure of intellectual functioning. This decrease was likely a result of his infarct. Although the exact date of his infarction was unknown, a usually

6 Bad Signs: Sickle-Cell Disease and Stroke Table 6.4 Other cognitive functions Tests Conners’ Continuous Performance Test – Second Edition Omissions Commissions Hit RT Hit RT standard error Variability Detectability Response style Perseverations Hit RT block change Hit SE block change Hit RT ISI change Hit SE ISI change California Verbal Learning Test – Children’s Version Recall measures List A total trials 1–5 List A trial 1 List A trial 5 List B free recall List A short-delay free recall List A short-delay cued recall List A long-delay free recall List A long-delay cued recall Recognition hits Recall errors Perseverations Free-recall intrusions Cued-recall intrusions Intrusions (total) Children’s Memory Scale Faces Immediate Delayed Wisconsin Card Sorting Test Categories achieved Total errors % perseverative errors % conceptual responses Failure to maintain set Behavior Rating Inventory of Executive Function Inhibit Shift Emotional control Behavioral regulation index Initiate Working memory Plan/organize

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Score

Percentile

[46] [37] [54] [45] [43] [45] [53] [50] [35] [45] [43] [45]

[50] {0.5} {0.0} {1.0} {0.5} {0.0} {1.0} {0.0} {0.5} {0.5} {0.5} {1.0} {0.5}

(10) (10) >16th 91 87 93 >16th [66] [64] [62] [66] [69] [56] [50] (continued)

56 Table 6.4 (continued) Tests Organization of materials Monitor Metacognition index Global executive composite Beery-Buktenica Developmental Test of Visual-Motor Integration Visual perception Motor coordination Grooved Pegboard Test Dominant hand (left) Nondominant hand (right)

K.R. Weissgerber, A. Epping

Score [45] [59] [57] [61] 74 77 78

Percentile

85 51

developing child, with intact intellectual functioning, typically maintains his or her level of cognitive functioning over time. Moreover, silent infarctions are associated with an overall dampening of general cognitive ability and weaknesses in graphomotor ability, such as seen on Johnny’s follow-up evaluation. Thus, it is likely that the decline observed in his intellectual ability was secondary to his silent infarction. In the absence of his infarct or other injury to his brain, Johnny’s intellectual functioning would have likely remained in the average range. Additionally, the new concerns regarding Johnny’s emotional and behavioral development are likely related to his infarction. Johnny’s stroke was located in the prefrontal cortex, which is located at the front part of the brain and associated with the regulation of behaviors and emotions. Difficulties in response inhibition, task initiation, mood regulation, and behavior that Johnny’s mother reported during his follow-up evaluation were generally consistent with the difficulties faced by individuals who suffer from injuries to the prefrontal cortex. Finally, the new onset of motor difficulties noted in Johnny’s right hand was also consistent with the location of his left-sided infarction. Given Johnny’s medical history and the cognitive difficulties that arose as a result of his stroke, a number of recommendations were advanced. Specifically, it was recommended that an eligibility meeting be held to determine his qualification for an Individualized Education Plan (IEP) under the Other Health Impairment (OHI) category, because his silent infarction may have played a role in his academic failure. Because Johnny’s new onset of academic difficulties likely also stemmed from his stroke, it was recommended that he not be retained. Instead, recommendations included special education services under the OHI category to include specialized instruction in a small group setting. Because of the change in his level of intellectual ability, Johnny would likely have a more difficult time acquiring and mastering new skills and concepts. It was important to get those working with Johnny to understand that although he looked the same, expectations for him had to be modified and extra academic support would be required for him to have academic success. Given the presence of fine motor and graphomotor difficulties, recommendations were made that Johnny receive an occupational therapy evaluation at school to

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determine his need for services in this area, with a goal of increasing his writing efficiency. In the meantime, it was recommended that he be provided with classroom notes to limit the demands on his already strained graphomotor skills. Consultation with a mental health professional to address emotional and behavioral concerns was also advised. Specifically, consultation with a child psychologist with expertise in working with children who have suffered from brain injuries was suggested to address anger management and coping skills. At this point, medication was not recommended; however, if emotional and behavioral concerns did not improve over time, medication management would become an option. Finally, in light of his sickle-cell disease and silent infarction, it would be important to continue to monitor Johnny’s functioning over time. Most children with sickle-cell anemia are followed closely by their pediatric hematologists at least once a year. Those who have more severe complications, such as Johnny, are followed more closely (approximately once a month when they come in for their monthly blood transfusions). In addition to his medical needs, it would also be important to monitor his neuropsychological functioning over time. Unfortunately, as was the case with Johnny, silent infarctions are typically not noticed right away. Thus, children may suffer ‘‘silently’’ for years without anyone knowing that they have experienced a stroke. By monitoring their neuropsychological functioning, changes can be detected and accommodations can be made and/or adjusted as necessary.

Additional Resources Key Concepts and Terms Executive Functions Include self-regulatory skills that affect an individual’s planning, flexibility, generation of information, inhibition of impulses, and working memory (i.e., the ability to hold information in memory while performing some operation on it or manipulation using it). The ability to regulate emotions and attention are also key hallmarks of executive functioning.

References Resources for Clinicians Day, S., & Marion, S. B. (1996). Educator’s guide to sickle cell disease. Memphis, TN: St. Jude Children’s Research Hospital. Kirkham, F. (2007) Stroke and cardiovascular disease in childhood. Cambridge, MA: Blackwell. National Heart Lung Blood Association, www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs. html. Provides information about sickle cell disease and engages in research for sickle cell disease.

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Sickle Cell Disease Association of America, (1998). Parent/teacher guide: How parents and teachers can work together to achieve school success for children with sickle cell anemia. Baltimore, MD.

Resources for Families Children’s Hemiplegia and Stroke Association, www.chasa.org/. A non-profit support group for parents and families of children who have had a stroke. Earles, A., Lessing, S., & Vichinsky, E. (Eds.). (1993). Parents’ handbook for sickle cell disease: Part II: Six to eighteen years of age. Berkley, CA: State of California Department of Health Services. Lessing, S., & Vichinsky, E. (1998). Parents’ handbook for sickle cell disease: Part I: Birth to six years of age. Oakland, CA: State of California Department of Health Services. Sickle Cell Disease Association of America, (1998). Parent/teacher guide: How parents and teachers can work together to achieve school success for children with sickle cell anemia. Baltimore, MD: Sickle Cell Disease Association of America. Sickle Cell Disease Association of America, www.sicklecelldisease.org/. Dedicated to finding a cure for sickle cell disease. The Web site provides general information, research updates, and links to additional resources.

Chapter 7

Life Interrupted: Medulloblastoma Peter L. Stavinoha

Anthony’s parents had been planning a family trip to the coast for months. Anthony, a precocious 4-year-old with excellent language skills, had been to the beach once before and could not stop talking about the upcoming trip. On a cool Friday morning in autumn, Anthony’s mother was busy packing the family’s things for their 2-week vacation. She had decided to take the day off from work while Anthony was in day care so that she could take care of all the last-minute details. When the day-care staff called to report that Anthony did not seem right, she figured he might have come down with an illness that would make the plane ride less pleasant, but she certainly did not anticipate that the vacation would never happen. When she arrived at the day care, she learned that Anthony had been having problems with balance all morning and had fallen several times. She also noticed that his eyes were moist and that his head was tilted to one side. ‘‘Tears are falling from my eyes, Mommy,’’ said Anthony with only a slight hint of distress in his voice but still looking to his mother to somehow make it stop. Anthony’s mother felt her heart jump and quickly scooped up Anthony and shuttled him to the emergency department. Within an hour, Anthony had been sedated and was undergoing a Magnetic Resonance Imaging (MRI) scan. Within 4 hours, a neurosurgeon was describing to Anthony’s parents the lemon-sized mass that had shown up on the MRI in the posterior fossa, the area of the brain that houses the cerebellum and critical brainstem structures. The neurosurgeon was personable but very serious, and Anthony’s parents consented to the recommended treatment, which consisted of creating a hole in the back of Anthony’s skull to remove the tumor. By Sunday morning, the family’s vacation was a distant memory, and the neurosurgeon told Anthony’s parents that the surgery had gone well. They held Anthony’s hand while he was in the intensive care unit, counting their blessings that their child had survived, without realizing that the battle was not over. Samples of the tumor tissue were analyzed and identified as medulloblastoma, a malignant and aggressive type of Primitive Neuroectodermal Tumor (PNET). As Anthony recovered from neurosurgery and became increasingly alert and active, his parents were learning about the risks and benefits of the proposed treatment plan. Specifically, they learned that Anthony would need to undergo a course of radiation

J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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therapy in which radiation would be delivered directly to his brain and spine, and they also learned about the months of chemotherapy that Anthony would have to endure following cessation of radiation. Still in shock from their realization of how close Anthony had been to death, his parents did not really process some of the risks stated to them with regard to radiation and chemotherapy, including declines in IQ, learning problems, hair loss, and growth suppression. They also did not heed, or perhaps even hear, the recommendation that they should have Anthony evaluated by a neuropsychologist in the coming years in order to keep close tabs on his neurocognitive development in light of the tumor and its treatments. Fast-forward 8 years, and Anthony was in the middle of fifth grade and struggling significantly. For years, Anthony underwent routine MRI scans and endocrine treatments (e.g., growth hormone), and his parents were always relieved that there was no recurrence of the tumor. For kindergarten through fourth grade, Anthony’s parents had enrolled him in a small private school, where they believed that he would get any extra attention he might need. The teacher-to-student ratio was low, and Anthony had a series of kind teachers who always seemed to have a soft spot for this first child they had ever known who had experienced a brain tumor. Anthony’s parents felt that they were not being overprotective but simply providing Anthony with a school placement that would give him plenty of time to mature as they harbored lingering concerns about his ability to integrate successfully in an overcrowded public school system that they perceived as overwhelming. They always felt reinforced when his report cards said that he was making satisfactory progress and underscored what a sweet and compliant child he was. Anthony’s parents were well-educated professionals who had an inkling that he was not as strong in math as perhaps other students in his grade might be, and they also noticed that he moved a little slower and seemed a little more awkward than many other children of his age, but otherwise the consistently positive teacher comments reinforced their belief that Anthony was progressing well. At the end of fourth grade, Anthony’s father was transferred by his employer, and the family had to move. Anthony’s parents were devastated that he would no longer be able to attend the private school where he had seemed so comfortable, but circumstances dictated that they seek a new educational placement for Anthony. After exhausting all options, Anthony’s parents determined that the only placement for him would be their local public elementary school. They felt confident that Anthony had progressed well over the years, and they knew that the elementary school was highly rated. So with only a little trepidation, Anthony’s parents enrolled him in the fifth grade. Within the first few weeks, it was clear that things were not going well. Anthony’s teacher already had sent notes home about unfinished work in the classroom, and Anthony was complaining that he had no friends and that the work was very hard for him. He wanted to do well and please his teachers, so he was adamant about completing all of his homework each night, though this was taking hours, to the point of significant fatigue. Anthony’s parents were particularly distressed after receiving results of benchmark testing showing that Anthony was not keeping up with his classmates, particularly in math.

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A parent–teacher conference was called 5 weeks into the school year, and Anthony’s parents were shocked to learn how far he was behind typical fifth grade students. School staff indicated that they would like to do some testing with Anthony to see whether he needed more intensive school services, such as those offered through special education. Anthony’s parents were dismayed at the suggestion and could not even fathom that their child could be a special education student. After a few days of soul-searching and watching Anthony become more and more unhappy and even fearful of going to school, Anthony’s parents recalled the neurosurgeon’s suggestion of a thorough neuropsychological evaluation. They discussed this with the school, and it was determined that, given Anthony’s history of brain tumor, a neuropsychological evaluation would be the most appropriate starting point for determination of needs at school.

Test Results Dual priorities dictated the scope of the neuropsychological evaluation. First, Anthony had never undergone neurocognitive evaluation in the past, even though this had been recommended to his parents. Because several patterns of findings in children who have experienced medulloblastoma and subsequent radiation and chemotherapy treatments are common (such as decreases in IQ, slower processing speed, and difficulties with attention and memory), a broad group of tests was selected. Second, the practical issue of identifying Anthony’s educational needs as well as helping to elucidate the most appropriate educational placement for Anthony within the public school dictated that the evaluation includes measures that would satisfy the multidisciplinary committee that would ultimately plan Anthony’s educational placement. The initial interview with Anthony’s parents illuminated their conflicted feelings about his progress. On the one hand, they seemed sensitive to the fact that he was not as well-developed cognitively, socially, and academically as many other children of his age, but on the other hand, they were holding dearly to their pretumor picture of Anthony as a bright, energetic, and creative boy with limitless potential. Anthony was a bit wary of the testing situation at first, but he quickly settled in and seemed to enjoy the one-to-one attention. He possessed physical characteristics common to children who have undergone craniospinal radiation during early childhood, including hair loss and short stature. His movements tended to be relatively slow and cautious, but he seemed to be in a good mood. He fatigued relatively easily, and was given more breaks than is typical during the course of an evaluation. In fact, he worked rather slowly and needed so many breaks that testing had to be completed over two separate sessions. Anthony appeared to have mild difficulty with attention, though his behavior was not at all impulsive. He seemed very eager to please the examiner and to perform well on testing, and at times he even seemed mildly anxious when he was not able to complete a task within time limits.

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Parent and teacher behavioral ratings indicated social difficulties that seemed more prominent at school than home. In addition, Anthony was judged to be more withdrawn and anxious in his classroom environment than most boys of his age, though Anthony’s parents did not raise the same concern about anxiety at home except when it came to homework activities. Mild difficulties with attention and concentration were also noted at school, though again these difficulties were not reported as prominent at home. The evaluation (Tables 7.1–7.3) revealed that Anthony was experiencing a number of common late effects of treatment for medulloblastoma. In particular, his neurocognitive pattern was characterized by a significant discrepancy between verbal information processing abilities and nonverbal abilities, with his composite IQ falling below average. In contrast to average verbal abilities, Anthony exhibited significant weaknesses across tasks involving visual-spatial information processing as well as processing speed and cognitive efficiency. Auditory working memory was low average, with Anthony exhibiting deterioration of working memory with increasing complexity of information. Evaluation of learning and memory functions suggested a pattern congruent with measurement of Anthony’s overall cognitive ability. Specifically, his capacity to take in, store, and later retrieve languagebased information was much better developed than his nonverbal, visual-spatial memory functions. Visual-motor skill development was well below average, and Anthony had difficulty both with visual-perceptual functions as well as bilateral motor dexterity. His performance on tasks tapping executive functions was suggestive of difficulty with both abstract concept formation and cognitive flexibility. Anthony tended to have difficulty generating appropriate problem-solving

Table 7.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Full Scale IQ Verbal Comprehension Index Similarities Vocabulary Comprehension (Information) Perceptual Reasoning Index Block Design Picture Concepts Matrix Reasoning Working Memory Index Digit Span Letter-Number Sequencing Processing Speed Index Coding Symbol Search a Standard score, (scaled score), [T-score], {z-score}.

Scorea 77 96 (9) (10) (9) (11) 73 (5) (7) (5) 83 (8) (6) 73 (4) (6)

Percentile 6th 39th

4th

13th

4th

7 Life Interrupted: Medulloblastoma Table 7.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition Academic skills Academic fluency Academic applications Broad reading Letter-word identification Reading fluency Passage comprehension Broad math Calculation Math fluency Applied problems Broad written language Spelling Writing fluency Writing samples

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Score

Percentile

Grade Equivalent

93 78 85 90 101 82 92 78 77 74 82 87 97 76 91

33rd 7th 15th 25th 52nd 12th 29th 7th 6th 4th 11th 20th 42nd 5th 26th

5.2 3.6 3.9 4.7 6.7 3.6 4.5 3.5 3.5 3.7 3.5 4.5 5.7 3.6 4.3

strategies and benefiting from direct feedback in order to modify his problemsolving approach. Academically, Anthony exhibited patterns of relative strengths and weaknesses that correlated with his underlying neurocognitive capacity. Specifically, his overall reading skill development was measured in the low end of the normal range, with his composite written expression performance falling in the low average range. Interestingly, Anthony’s strongest language-based performances were on tasks that are amenable to rote memory (e.g., sight word recognition and written spelling). In contrast, Anthony struggled significantly on math tasks such that his overall math development was well below average and more in line with his visual-spatial and nonverbal information processing weaknesses. A prominent weakness emerged when the added dimension of speed of processing intersected with academic skill performance. Congruent with his slow cognitive efficiency, which was documented in testing and informally observed during the evaluation, Anthony struggled with academic testing that required quick performance. This was in keeping with both parent and teacher reports that Anthony takes a very long time to complete classroom and homework academic activities.

Formulation and Recommendations Anthony’s test result pattern illustrated a number of issues relatively common in children treated for medulloblastoma with radiation and chemotherapy, and his treatment before 6 years of age raised his risk for neuropsychological deficits. Functional deficits commonly seen following radiation and chemotherapy treatment for medulloblastoma include difficulties with nonverbal, visual-spatial

64 Table 7.3 Other cognitive functions Tests California Verbal Learning Test List A total trials 1–5 List A trial 1 free recall List A trial 5 free recall List B free recall List A short-delay free recall List A short-delay cued recall List A long-delay free recall List A long-delay cued recall Recognition Children’s Memory Scale Dot locations Learning Total score Long delay Stories Immediate Delayed Delayed recognition Faces Immediate Delayed Wisconsin Card Sorting Test Total errors Perseverative responses Perseverative errors Nonperseverative errors Grooved Pegboard Test Preferred hand (right) Nonpreferred hand Beery-Buktenica Developmental Test of Visual-Motor Integration Benton Judgment of Line Orientation Test

P.L. Stavinoha

Score [39] {1.0} {1.0} {2.0} {1.0} {0.5} {1.0} {0.5} {0.0}

(6) (6) (5) (8) (7) (8) (5) (6) 71 79 78 73 {2.88} {3.29} 72 {2.84}

information processing, motor dexterity, executive functions, and speed/efficiency of information processing and are presumed to be due to white matter damage. Radiation by itself, and in combination with chemotherapy, is toxic and often results in damage to the protective coating of the long nerve projections that make up the white matter. Younger children appear to be particularly susceptible to this damage and associated functional deficits. Compromise to these main communication pathways that let one brain region efficiently ‘‘talk’’ to others results in a pattern of deficits that is very consistent with Anthony’s weaknesses. Anthony’s effort and reasonably well-developed rote verbal memory ability likely propped up his academic performance over the years. This was magnified by his participation in a private school setting that did not effectively document or

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communicate his slower progress compared with peers in feedback to Anthony’s family. He typically perceived himself to perform reasonably well because he did not have a clear standard to which to compare himself. Consequently, his immersion in a regular education, public school setting resulted in a mismatch between Anthony’s previously unidentified neurocognitive deficits secondary to treatment for medulloblastoma and an increasingly demanding curriculum emphasizing abstract thinking, synthesis, and information organization. Anthony wanted to perform well but found that even his best efforts produced mediocre results. This was frustrating for Anthony as he tried to assimilate into a new school and new peer group, and he started to feel anxious about his performance to the point of being somewhat fearful of going to school. Anthony’s attempts to socialize with same-age peers were not fruitful for several reasons. He showed significant physical stigmata of early radiation treatment (e.g., hair loss, short stature) that his elementary school peers had never seen before. Anthony also had difficulty perceiving social cues and responding quickly to typical middle-school social interactions secondary to his visual-spatial information processing deficits and generally slow pace of cognitive processing. During an extensive feedback conference with Anthony’s parents as well as consultation with staff at school, a number of recommendations were made to address the prominent issues with which Anthony presented, including difficulties at school, underlying neurocognitive weaknesses, and difficulties with social interactions and emotional adjustment. Because Anthony had not already been identified as a student requiring special education services, this was the top priority. He already had begun showing anxiety about his capacity to successfully perform schoolwork and difficulty fitting in with peers. Consequently, the recommendation was made to the multidisciplinary team determining special education eligibility that it swiftly act to develop an appropriate Individualized Education Plan (IEP) and supportive educational placement for Anthony on the basis of his academic and cognitive deficits secondary to brain tumor and its treatment. Because Anthony had experienced a significant health condition that clearly resulted in significant educational need, the handicapping condition of Other Health Impairment (OHI) seemed most appropriate, because it captured the link between Anthony’s deficits and corresponding medical etiology. In particular, emphasis was placed on ensuring that Anthony received modified instruction in math given that his skill development in that core subject area lagged so significantly behind that of his same-age peers. In addition, strategies for dealing with Anthony’s slow cognitive processing speed and fatigability were provided that included reduction in the amount of homework assigned to Anthony, availability of classroom support to assist with completion of assignments during the school day, and a limit on the time that Anthony would be permitted to spend on homework each evening. A number of strategies were suggested related to deficits in visual-spatial information processing. Because Anthony exhibited a relative strength in verbal memory and verbal cognitive ability overall, rehearsing information out loud in order to better digest and remember it was recommended so as to take advantage of this strength. This oral rehearsal technique included reading or reciting information

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aloud as a primary study method for remembering information later. Subsequently, oral testing procedures could be utilized judiciously given Anthony’s handwriting deficits. Compensatory strategies for organizing and dividing visual information also were recommended. For example, Anthony could receive direct instruction in using a highlighter to divide a worksheet into smaller, more manageable parts. Using spacers or place markers could also reduce complex visual stimuli such as worksheets or pages of text to more manageable amounts. An occupational therapy evaluation was recommended because of Anthony’s difficulties with fine motor dexterity and handwriting. Difficulties with speed of information processing compound problems with handwriting, so it was recommended that Anthony have access to teacher or peer notes and that he not be required to copy large amounts of information from the board. It was stressed that Anthony likely expends a great deal of energy on tedious tasks such as copying and handwriting, and this energy could be much better spent on higher level cognitive/academic activities. The lack of understanding of the full range of implications of Anthony’s brain tumor and its treatment extended far beyond the academic domain. Anthony’s peers had little to do with him because of differences in physical appearance as well as his slower speed of processing. Although Anthony was not bullied, he certainly was not included, and this magnified any normal adjustment issues that would be associated with starting a new school. As such, two methods were advised. First, a Child Life Specialist, a professional whose role includes assisting patients and families coping with medical illness and medical procedures, was asked to speak to Anthony’s class at school in order to talk more about the difficult experiences and treatments that he had endured. Second, Anthony was selected to participate in a friendship group facilitated by the school guidance counselor focusing on social interaction and social skill development. Although Anthony’s parents had come a long way in their understanding of his deficits and the long-term implications, it was clear that they had always struggled with the potential that Anthony would have certain neurocognitive limitations, and they seemed to be facing these issues at this time only out of necessity. As an extension to the feedback conference, a series of additional consultation appointments were scheduled with the neuropsychologist to help provide guidance with IEP implementation, education regarding late effects of brain tumors and their treatment, and psychosocial support for these parents who were struggling to fully acknowledge and appreciate their child’s neurocognitive assets and weaknesses.

Update As difficult as it had been for Anthony’s parents to finally seek out neuropsychological evaluation in the first place, they seemed eager to get on the neuropsychologist’s calendar for a follow-up evaluation 18 months later. Through the process of being confronted with the late effects of Anthony’s treatment for medulloblastoma

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as he integrated into a new school setting, Anthony’s parents caught a glimpse of the numerous developmental challenges that awaited Anthony in the coming years. In the short-term, with appropriate educational placement and planning, Anthony’s anxiety about school and his classroom performance had reduced significantly. He seemed happier in his new school placement, and his participation in the social group had yielded benefits in terms of peers including him in activities more often, though he still did not have a ‘‘close’’ group of friends. Even with these positive changes, Anthony’s parents began to recognize that there would be tough days ahead as Anthony transitioned into junior high, high school, and young adulthood.

Additional Resources Key Concepts and Terms Child Life Specialist A professional typically employed in a hospital setting that works to support the psychosocial development and emotional coping of children who are ill or who are undergoing medical treatment. Medulloblastoma A type of malignant brain tumor that grows in the region of the posterior fossa. Treatment for this type of aggressive brain tumor in children typically requires surgical removal of the tumor followed by treatment with radiation and chemotherapy in order to minimize risk for regrowth of the tumor. Posterior Fossa This is a region inside the skull that is low and at the rear of the skull. This vault houses important brain regions including the cerebellum and the brain stem. Primitive Neuroectodermal Tumors Sometimes referred to as PNET, these are a group of malignant and rapidly growing brain tumors that have a high rate of recurrence. Medulloblastoma is a type of PNET. White Matter Consists of long projections from nerve cells that are covered in a protective coating called myelin, which is white in color. White matter acts as the communication pathway so that one region of the brain can communicate efficiently with another.

References Resources for Clinicians Butler, R. W., & Mulhern, R. K. (2005). Neurocognitive interventions for children and adolescents surviving cancer. Journal of Pediatric Psychology, 30(1), 65–78. Mulhern, R. K., Merchant, T. E., Gajjar, A., Reddick, W. E., & Kun, L. E. (2004). Late neurocognitive sequelae in survivors of brain tumours in childhood. The Lancet Oncology, 5(7), 399–408.

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National Cancer Institute. (2006). Late effects of treatment for childhood cancer (PDQ1). Retrieved October 16, 2006 from http://www.cancer.gov/cancertopics/pdq/treatment/lateeffects/HealthProfessional/page2. Schatz, J., Kramer, J. H., Ablin, A., & Matthay, K. K. (2000). Processing speed, working memory, and IQ: A developmental model of cognitive deficits following cranial radiation therapy. Neuropsychology, 14(2), 189–200.

Resources for Families American Brain Tumor Association. Provides funding for brain tumor research and offers educational and support services for patients and families. Address: 2720 River Road Suite 146, Des Plaines, IL 60018-4110; phone: 1-800-886-2282; Web site and e-mail: http://www.abta.org; [email protected] American Cancer Society. Dedicated to eliminating cancer through research, education, advocacy, and service. Phone: 1-800-ACS-2345; Web site: http://www.cancer.org The Brain Tumor Society. Provides information, resources, and support for people affected by brain tumors. Address: 124 Watertown Street, Suite 3H, Watertown, MA 02472; phone: 1-800770-TBTS; Web site: http://www.tbts.org The Children’s Brain Tumor Foundation. Committed to improving treatments, quality of life, and long-term outcomes for children with brain and spinal cord tumors through research, support, education, and advocacy. Address: 274 Madison Avenue, Suite 1004, New York, NY 10016; phone: (914) 238-7658; Web-site and e-mail: http://www.cbtf.org; [email protected] National Brain Tumor Foundation. Provides information about brain tumors and their treatment. Information is available on clinical trials and medical centers specializing in brain tumors. Address: 22 Battery Street, Suite 612, San Francisco, CA 94111-5520; phone: 1-800-934CURE; Web site and e-mail: http://www.braintumor.org; [email protected]

Chapter 8

The Mystery of the Falling Grades: Seizure Disorder Lynn Bennett Blackburn

Working with children with epilepsy is like reading a good mystery. When learning or emotional problems occur, the neuropsychologist’s job is to help identify ‘‘whodunit.’’ The list of likely suspects from the epilepsy perspective include whatever is atypical about the brain that is the basis of the seizures, seizures themselves, abnormal electrical discharges between seizures (also known as subclinical seizures), and side effects of medications used to treat the seizures. These suspects may set the stage for problems with attention, learning, memory, and emotional functioning. The reactions of family and friends to the epilepsy diagnosis and/or seizures may lead to feelings of anxiety and depression. Children with epilepsy can also develop learning or emotional problems for the same reasons that other children do, such as genetics or life experiences. John was referred for neuropsychological evaluation to sort through these suspects to find the cause of his falling grades. John was attending seventh grade at the time of referral. His parents reported that he started off well in school. He began reading prior to starting kindergarten. He was placed in a program for gifted students in first grade and continued in this program throughout elementary school. John developed complex partial seizures between fourth and fifth grade. John would become confused during seizures and was sleepy after a seizure ended. Since seizures were only occurring at home, his parents informed the school nurse, but no one else knew about his epilepsy. His antiepileptic drug (AED) supported good seizure control. During fifth grade, John seemed to work harder for his grades but continued to do well. Just before John was to start sixth grade, he began to experience frequent seizures. His parents reported that sixth grade was very hard for John because of frequent changes in medication and in dose. John was often sleepy as he adjusted to each change. When seizures occurred in school, John was teased by peers. To make matters worse, his best friend throughout elementary school moved away, leaving him with no one to come to his defense. According to his parents, other friends just drifted away, failing to include John in activities. His grades dropped to Bs and Cs. An effective AED combination was identified over the summer following sixth grade, raising his parents’ hope that seventh grade would be better. However, at the J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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time of referral, John was in danger of failing seventh grade, with Ds and Fs in most subjects. At the time of referral, John’s parents expressed concern about his memory. John would forget what he was told to do. Even when he would begin a task, he would get distracted and fail to complete it. He needed prompts to do things that should be habit, such as brushing his teeth or taking his medication. He had trouble finding possessions he had put away. Regarding schoolwork, John’s parents reported that he often forgot to write down assignments and to turn in homework he had completed. They tried organizing John’s binder for him, providing color-coded folders for each class. They put reminder notes on his completed work. However, even with this help, he was still inconsistent in turning in work. Late assignments were one factor in his poor grades. John’s teachers noted inconsistency in memory, stating that John would appear to understand a concept one day but not the next day. Poor performances on tests were also contributing to his poor grades. However, his parents indicated that he could remember material for tests if he studied for them. John could remember upcoming events that were important to him and past events he had enjoyed. He was doing relatively well in his Spanish class, remembering new vocabulary and grammar as it was introduced. Although his seizures were once again well controlled, John did not make new friends. His school counselor reported that he sat with other students during lunch but did not talk to them. John’s parents described his interests as more ‘‘immature’’ than peers. They also indicated that John had difficulty adjusting to change, such as the move to junior high. Although his parents provided no further examples, his difficulty adjusting to change became a very important clue in understanding John. When John arrived for testing, he was like many adolescents referred for testing. He responded to the questions with one-word answers, providing little help in understanding his perspective on his problems. He knew school was hard. He complained that teachers went too fast, making it hard to take notes or write down assignments. John spent the rest of the interview staring at his shoes and offering ‘‘I don’t know’’ or shoulder shrugs as answers to the questions, all while his parents were struggling to remain quiet, barely controlling the urge to jump in and answer for him. John’s behavior during testing provided two important clues regarding his problems. First, his affect remained flat throughout testing. He showed no reaction to praise or pride in his accomplishments when successful. He appeared to have little confidence in his skills, becoming easily overwhelmed. He gave up on some tasks (saying ‘‘I don’t know’’) before he had even heard the complete question or direction. However, with encouragement to listen again and then take a guess, he often did know the correct answer. He became visibly anxious with the introduction of each new task. Second, he seemed to have problems with expressive language. He knew lots of facts but had difficulty organizing them to effectively answer test questions. He could engage in conversation if asked a series of yes/no questions but could not elaborate on answers in a way that continued the conversation. He also had trouble communicating in writing. Generation of written sentences

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was painstakingly slow. While his parents and teachers were focused on his memory, his behavior during testing suggested that emotional factors and language skills were also likely suspects in his falling grades.

Test Results Although John might have been expected to struggle with tasks involving understanding and remembering language, his achievement on these tasks fell in the average to high average range (Table 8.1). John retold stories in a somewhat disorganized fashion but captured the main theme and most details. He used effective strategies to organize his recall of items from a shopping list. Rote learning, that is, learning facts through repetition, emerged as a relative strength for John. This strength may have supported his rapid learning of early academic information. John had trouble remembering some types of visual Table 8.1 Other cognitive functions Tests Scorea Clinical Evaluation of Language Fundamentals – Fourth Edition Understanding paragraphs (9)

37th

California Verbal Learning Test – Children’s Version Total learning Long-delay free recall Recognition

115 115 108

84th 84th 70th

(13) (12) (12)

84th 74th 74th

(2) (7)

<1st 16th

(9) (11)

37th 63rd

68 65 94

<1st <1st 35th

<40 104 82 96

<1st 60th 12th 45th

Children’s Memory Scale Stories Immediate recall Delayed recall Recognition Dot Locations Learning Delayed recall Faces Immediate recognition Delayed recognition Rey-Osterrieth Complex Figure Test Immediate recall Delayed recall Recognition Test of Variables of Attention Omission errors Commission errors Response speed Variability a Standard score, (scaled score), [T-score], {z-score}.

Percentile

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information. He could remember faces but had difficulty remembering spatial position and orientation. For example, he could recognize details that made up a complex geometric design he had copied, but he could not remember where to put the details when he attempted to draw the design from memory. Overall, results of memory testing suggested that John should be able to do many of the everyday memory tasks that had his parents concerned. His relative weakness in spatial memory might affect achievement in a few subjects, but John should have been able to use his strength in verbal memory to compensate. Results suggested that something was getting in the way of John’s use of his memory skills. Attention and emotions remained likely suspects. John’s attention was assessed through parent and teacher ratings, as well as through a computerized attention task. John’s parents and all but one of his teachers rated his attention as age appropriate. Scores from the computer task suggested that John was having problems remaining vigilant. However, his behavior during the task was not consistent with an attention deficit-hyperactivity disorder. John would give up on the game and stop responding for a period of time after he had made a mistake. He never stopped paying attention to the task, continuing to watch the screen and muttering to himself about what was being presented. At the end, he described the task in the same terms he used to describe school: ‘‘It was too hard because it went too fast and I just could not do it.’’ His performance appeared to reflect his pattern of coping rather than his level of development of attention skills. Emotional functioning was assessed through questionnaires completed by John and his parents and teachers. Not surprisingly, John denied that he was having any problems, worries, or concerns. Most teachers described him as a typical adolescent, while one teacher noted problems with social skills. Although John was not earning good grades, he was not causing problems in the classroom. His parents’ ratings depicted John as moody and isolated. They noted that he coped with challenges by attempting to withdraw from or avoid the challenging situation. John’s behavior during testing reflected low self-esteem, with symptoms of anxiety and depression. Children with epilepsy are at increased risk to develop significant problems with anxiety and depression, but it appeared that John’s problems coping with change were evident before he developed epilepsy and became worse since seizure onset. When the testing results were shared with John’s parents, they reported a new piece of information. Even though they had been asked if anything else was going on in John’s life, they had failed to mention that they were planning to move to a new house in the same community and John had indicated that he would not go. Clearly the adjustment to the impending family move and the other changes in his life (epilepsy, best friend moving away, beginning middle school) had overwhelmed John. The unfortunate handling of his epilepsy by school staff when seizures began to occur in school set John up to become isolated from peers. He had no support network to help him through the challenges his epilepsy presented. His parents were encouraged to get John into psychotherapy immediately. Involvement in a social skills group in the school setting was also recommended to help John reconnect with his peers.

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Often for children with epilepsy, the ‘‘whodunit’’ is not a single suspect but a gang of suspects. It was important to follow up on the other clues in John’s behavior during testing. The results of John’s intellectual screening came as a surprise (Table 8.2). His achievement fell in the average range, not the gifted range. His pattern of relative strengths and weaknesses was even more concerning. Junior-high-level instruction occurs primarily through language (lectures, discussion, reading), and John’s language-based reasoning skills fell in the low average range. Given his early academic history, his teachers and parents were still expecting gifted work from a child who was no longer functioning in the gifted range. His teachers would need to provide more explanation in order for John to understand concepts fully. Concepts are more likely to be retained from day to day when fully understood. John was right—his teachers were going too fast. Clearly, John also needed to work harder than he had in the past so as to keep pace with his peers. The results of John’s achievement testing provided further evidence of a change in his rate of development (Table 8.3). His reading and math skills fell in the average range. He was no longer making gains in these areas at a faster rate than his peers. His scores on writing tasks fell in the low average range. Qualitatively, his writing was even worse. John struggled with the very act of handwriting and was most legible when printing. He had difficulty generating a complete thought when writing, just as he had when speaking. He left out capitalization and punctuation, omitted letters from words, and occasionally omitted words from sentences as he struggled to keep in mind what he wanted to say. This struggle to organize and keep track of his thoughts was also evident when John spoke. Upon observing his writing speed and content, it made perfect sense that John was struggling to take meaningful notes in lecture classes and failing to record assignments in the time allotted. John was right—his teachers did not give him enough time. Table 8.2 Intellectual Wechsler Abbreviated Scale of Intelligence Vocabulary Similarities Block Design Matrix Reasoning Verbal IQ Performance IQ Full Scale IQ

Score (8) (7) (11) (11) 87 104 95

Table 8.3 Academic achievement Woodcock Johnson Psychoeducational Battery – Third Edition Reading fluency Passage comprehension Math fluency Calculation Writing fluency Writing samples

Percentile 25th 16th 63rd 63rd 20th 60th 36th

Score 103 98 95 104 83 89

Percentile 58th 24th 36th 60th 13th 24th

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Formulation and Recommendations John’s difficulty organizing his thoughts, both when speaking and writing, suggested an expressive language disorder. Failing grades were occurring in applied subjects where lecture and class discussion were the primary means of teaching. In these classes, note-taking was essential, because test questions were often based on lecture material. Demonstration of learning in these classes often involved at least some questions requiring the generation of sentences. It was recommended that John have assistance with note-taking (an assigned note-taker or a copy of the teacher’s notes). John’s teachers also were encouraged to monitor his recording of assignments to ensure that John had enough time to get all the information. Accommodations for test-taking were also recommended, including extra time to complete tests and grading responses based on content rather than on writing mechanics (grammar, punctuation, spelling). It was recommended that the speech and language pathologist serving John’s school evaluate John’s verbal and written expressive language skills. The quality of John’s answers suggested that he was having difficulty with higher order language skills such as inference, comparison, and decoding the meaning of ambiguous words (e.g., figures of speech, words with multiple meanings). At his grade level, learning in applied subjects is often assessed through questions involving these skills. The speech and language pathologist was encouraged to look beyond John’s obtained test scores, to consider the speed and qualitative aspects of his responses and not just the accuracy of the eventual response. If John performed well on verbal memory tasks, why was he failing to remember everyday things? His test results suggested that the inconsistency in his memory was reflecting the disruptive effects of depression on attention and motivation. At home and in school, John likely was often preoccupied with his worries and concerns, rather than listening carefully to what adults were saying. Even when he remembered directions, he demonstrated little motivation to attempt many of the tasks presented but felt immediately overwhelmed and certain he would fail. ‘‘I forgot’’ became a convenient explanation. His parents were reassured that John’s everyday memory would improve as he gained confidence in himself through psychotherapy, and in the meantime, they were encouraged to give him singlestep commands (one thing to do at a time). John’s teachers were encouraged to check his understanding of directions by having him demonstrate what he should do. This would also address John’s concerns about making mistakes. Three suspects were identified as responsible for John’s falling grades: a change in rate of intellectual development, emergence of an expressive language disorder, and emotional distress. The key to helping John was to recognize that all of these factors were interrelated. John needed both academic accommodations and psychotherapy to stop the failing grades. One mystery was solved for John, while another was identified: What caused the change in John’s rate of development of intellectual skills and language skills? Only time will help to solve that mystery. It was possible that whatever was underlying

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John’s epilepsy was also responsible for the changes in his pattern of skill development. Other suspects remained, including his medications and emotional distress. The best way to understand the change was to follow John with repeated assessments over time. John’s story was not done, but testing had gotten him the help he needed to stop the failing grades.

Additional Resources Key Concepts and Terms Complex Partial Seizures: Partial seizures arise from a small area of the brain. Simple partial seizures involve a sensation such as a feeling, taste, or smell. These events do not disrupt consciousness. Complex partial seizures spread to involve more brain tissue but still remain contained in a single brain region. They are characterized by alteration in consciousness and may be accompanied by staring, semipurposeful movements (e.g., picking at clothing, walking in a circle), sensations, and/or oral automatisms (lip smacking, repetitive swallowing). Depending on the brain regions involved, a person may be able to understand what others are saying and/or speak during these events. Expressive Language Disorder: Expressive language disorders involve delays in the development of the language organization, sentence structure, and grammar to support communication of desired meaning. This does not include the ability to accurately produce speech sounds (articulation). Higher Order Language Skills: Higher order language skills require the integration of information as the basis for a language response, including the ability to flexibly, based on situation, interpret word meaning, body language, and tone of voice (e.g., interpreting figures of speech, jokes, or words with more than one meaning). Higher order language skills also include integrating current information with past learning as the basis for making inferences, making comparisons, and drawing conclusions.

References Resources for Clinicians Austin, J. K., & Caplan, R. (2007). Behavioral and psychiatric comorbidities in pediatric epilepsy: Toward an integrative model. Epilepsia, 48(9): 1639–1651. Baker, G. A., Spector, S., McGrath, Y., & Soteriou, H. (2005). Impact of epilepsy in adolescence: A UK controlled study. Epilepsy and Behavior, 6: 556–562.

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Dunn, D. W., Austin, J. K., & Huster, G. A. (1999). Symptoms of depression in adolescents with epilepsy. Journal of the American Academy of Child and Adolescent Psychiatry, 38: 1132–1138. Plioplys, S. (2003). Depression in children and adolescents with epilepsy. Epilepsy and Behavior, 4: 39–45.

Resources for Families Blackburn, L. B. (2003). Growing up with epilepsy: A practical guide for parents. New York: Demos Medical Publishing. E-communities: Entitled to Respect – Straight Talk for Teens about Epilepsy and Acceptance and Teen Group. Educational Materials: Epilepsy in the Teen Years (video). Epilepsy Foundation, www.epilepsyfoundation.org.

Chapter 9

When Half a Brain is Better than One: Recurrent Seizures Lynn Bennett Blackburn

Betsy looked just like any other child as she sat between her parents in the waiting room. She greeted the examiner with a polite smile and a shy hello. As she walked down the hall to the testing room, her right arm tended to hang at her side, not moving like the left. Her gait was awkward. These were the only visible signs that Betsy was very different than other children. No one suspected when Betsy was born that her brain had developed in an atypical manner. During infancy, her parents noticed that she was not using her right arm. Her doctor thought that she might have damaged a nerve in the shoulder area at birth and recommended occupational therapy. The occupational therapist found that muscle control in Betsy’s right leg was also abnormal. Magnetic resonance imaging (MRI) demonstrated that the left side of her brain had a cleft, a deep valley in the brain’s surface rather than the normal wrinkles. The cells around the cleft were disorganized, a brain malformation. Physical and occupational therapy were provided in her home until she was 3 years of age and then continued through an early childhood special education program. Speech therapy was added when it became clear that Betsy had difficulty making some speech sounds (articulation problems). Betsy’s parents were pleased with her progress. When Betsy was aged 4, she experienced another challenge. She developed epilepsy. Initially, seizures occurred during sleep and involved drooling, lip smacking, and movement of her arms and legs. Numerous medications, as well as a ketogenic diet, failed to control her seizures. Betsy continued to make good progress in developing cognitive skills, so her parents adapted to her seizures. Seizures often evolve, changing over time. Betsy developed daytime seizures during which the muscles on her right side would suddenly stiffen, causing her to fall. Because of the risk for injury, she had to be closely supervised all the time. She was provided with a one-to-one aide in school. One of her parents was always with her at home. She learned to ask permission before doing anything. Her parents or her aide could then determine whether what she wanted to do was ‘‘safe.’’ At this point, seizures were significantly interfering with Betsy’s social development and physical safety. Betsy’s parents faced a very difficult decision. The only hope of controlling her seizures and allowing her to live a normal life was neurosurgery. For some children J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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with epilepsy, surgery involves removal of only a small portion of the brain. For Betsy, a hemispherotomy was recommended. When she was 5-years old, a large portion of the malformed left hemisphere was removed, and the nerves allowing communication between the left and right hemispheres of the brain were severed. Betsy would function using only the right side of her brain (Figs. 9.1 and 9.2). Betsy’s surgery was successful; the seizures were controlled. Although she took multiple medications prior to surgery, most were tapered over the year after surgery. When Betsy arrived for assessment 1 year later, she was taking only one

Fig. 9.1 The MRI scans demonstrate the extent of tissue removed during a hemispherectomy, specifically a left hemisphere resection (Note: MRI right is left and left is right). Scan A reflects removal of the top of the brain looking down on brain structure

Fig. 9.2 Scan B reflects a cross section created by removal of the front portion of the brain, looking into the brain

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antiepileptic medication to support seizure control. Betsy was referred for neuropsychological assessment to assess the impact of surgery and her change in seizure control on her cognitive development. Her parents wanted to get a better picture of her strengths and weaknesses to assist them in setting expectations for her at home and at school. For most children undergoing epilepsy surgery, results are compared with presurgical testing to determine whether surgery changed the child’s skill pattern or rate of development. Betsy had been too ill prior to surgery to participate in testing. Postsurgical testing would only provide information about current skills and would not tell Betsy’s parents if her skill pattern had changed. Although test performance is one important source of information in neuropsychological assessment, information is also gained from other sources. Betsy’s parents were interviewed and completed questionnaires, her teacher completed a rating scale, her Individualized Education Plan (IEP) was reviewed, and her behavior was observed as she performed tasks and played. Her parents reported that Betsy’s expressive language skills had improved following surgery. Her production of speech sounds improved, making her easier to understand, and her sentence length increased. Her ability to concentrate also improved. Her parents had been warned that her hemiparesis might worsen, but Betsy showed no change in her ability to use her right arm or leg. It appeared that her brain had shifted control of her right arm and leg to the right hemisphere early in her development. During testing, Betsy was observed to use her right arm to anchor materials, such as holding down her paper when drawing. Because her right fingers moved in an approximation of what the left hand was doing (mirror movements), she still was unable to use both hands together in a coordinated fashion. Surgery did affect a nerve involved in the coordination of eye movements. Betsy’s parents reported that she had improved, but her behavior during testing suggested that she continued to experience some problems in this area. In the waiting room, Betsy was observed to continue to have problems with depth perception as she attempted to negotiate stairs carpeted in the same color as the surrounding floor. She also had difficulty scanning lines of material and needed the examiner to use a sheet of paper to block material below the line she was scanning. Her surgery also created a visual field deficit. Betsy could see things immediately in front of her and to her left, but she needed to turn her head or move her eyes to the right to see things off to the right. Her parents noted that she would occasionally forget to look to the right and then bump into things on her right side. During testing, it was necessary to position test materials so that the right edge of the materials lined up with the midline of her body to ensure that she saw all the response options on the right side of the page. The examiner would also use gestures to draw attention to these options when Betsy became overly focused on the left half of the page. Betsy’s parents reported that the years of needing to wait for permission before acting seemed to have taken a toll on her social functioning. Betsy continued to be hesitant to act in new situations, looking to adults for guidance. Although her parents indicated that Betsy was becoming more spontaneous in new situations, her teacher continued to describe her as somewhat passive in the classroom. Betsy’s behavior during testing was consistent with this description. In the waiting room,

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Betsy would look to her parents before approaching and playing with any of the toys available to her. This behavior did not change as she became familiar with the testing setting.

Test Results Initially Betsy was very shy with the examiner, but she became more interactive as she gained comfort in the testing setting. By the end of the morning, Betsy was initiating conversation with the examiner, relating stories about her family and her experiences. Her manner of telling stories was quite tangential and disorganized. She would abruptly change topics and relate the ending of a story before providing enough information to understand what had led up to the ending. Although it was possible to understand what Betsy meant to say, she would often omit sounds (e.g., ‘‘pooter’’ for ‘‘computer’’; ‘‘welve’’ for 12). One might logically conclude that her articulation problems were a result of her brain malformation, but her parents noted that other family members had similar problems with articulation during childhood. During testing it was also noted that Betsy had difficulty inhibiting well-learned patterns. When shown a picture of an arrangement of dots that she was to remember, she insisted on counting the dots, paying little attention to their relative positions. On a task where she was told to put a slash mark through her answer, she circled her answer. Her parents reported that most school worksheets involve circling answers. On tasks involving yes/no questions, she tended to only respond yes. This continued use of a response even when one is aware that it is no longer appropriate is referred to as perseveration. Betsy had difficulty understanding verbal directions during testing. She would sit quietly, offering no response when confused. Once directions were repeated, rephrased, or demonstrated, Betsy got right to work. At the time of evaluation, Betsy was attending a regular kindergarten classroom. According to her IEP, she was making satisfactory progress in reading and math but had a difficult time sequencing events to relate a story. Classroom modifications included preferential seating, shortening assignments, providing extra time to complete tasks, and repeating or modeling directions to support understanding. She was receiving speech therapy with IEP goals focused on her articulation problems. She no longer received direct occupational therapy services, but the occupational therapist at her school continued to monitor her progress. Her IEP indicated that no additional tutoring was necessary to support her learning of basic academic skills. Performance on the Wide Range Achievement Test – Fourth Edition, given as part of her neuropsychological assessment, confirmed the presence of age-appropriate academic skills (Table 9.1). Betsy’s test results highlighted the unevenness of her skill development. Although she was doing remarkably well in some areas, she had significant weaknesses in others. Her ability to reason using language fell solidly in the average range. Visually-based reasoning skills were less well developed (borderline range). Betsy relied on language, labeling the materials and talking herself through pro-

9 When Half a Brain is Better than One: Recurrent Seizures Table 9.1 Academic achievement Wide Range Achievement Test – Fourth Edition Word reading Spelling Math computation a Standard score, (scaled score), [T-score], {z-score}.

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Scorea 91 87 88

Percentile 27th 19th 21st

blems when visual materials were presented. Further testing of her ability to interpret visual information was conducted. Betsy was able to accurately integrate details into a meaningful whole perception, as well as visually match material on the basis of size and spatial orientation. Her average performance on these measures demonstrated that she could accurately process visual material as long as she was focused on a single object or was choosing from only a few clearly distinct response options. Her low scores on visually based reasoning tasks appeared to reflect problems with effectively scanning and integrating large amounts of visual information, discriminating relevant from irrelevant details, and sequencing actions to reproduce what she had seen. Her achievement on measures of receptive vocabulary (associating labels with pictures) and measures of expressive vocabulary (word definition, object labeling) fell in the average range (Table 9.2). She was able to understand language concepts in directions that sequenced and defined actions. Her sole low score occurred on a task where she had to keep in mind the content of an entire sentence while scanning four detailed pictures to find the picture representing the sentence’s meaning. Visual scanning skills and verbal memory capacity appeared to play a role in her low achievement on this task. Betsy’s scores on the information subtest from the Wechsler (Table 9.3) and scores from the Wide Range Achievement Test – Fourth Edition (Table 9.1) demonstrated that Betsy was able to learn material through repetition. An attempt was made to quantify her memory skills through formal tests. This attempt was met with limited success. Betsy rapidly became bored and/or frustrated with memory games. Her low scores reflected her difficulty with sequencing verbal information for relating an event (stories and sentences) and her focus on counting dots rather than attending to spatial location. Her tendencies to perseverate on a yes response when given true/ false questions on material invalidated attempts to measure recognition of faces, words from a list learning task, and story content. The results of formal memory testing were felt to be a poor estimate of her functional memory capacity. Adaptive behavior refers to the everyday application of language skills, fine motor skills, and gross motor skills. Betsy’s parents completed a questionnaire rating her skills. Social skills include play skills and the knowledge of good manners. Her average score reflected her age-appropriate range of play interests, ability to maintain play with friends, and knowledge of manners. Her lower ratings in conceptual skills reflected her continued delays in the development of articulation skills and her limited ability to relate an event. Practical skills include

82 Table 9.2 Other cognitive functions Tests Kaufman Assessment Battery for Children – Second Edition Gestalt closure Woodcock Johnson Psychoeducational Battery – Third Edition Block rotation Beery-Buktenica Developmental Test of Visual-Motor Integration Peabody Picture Vocabulary Test – Fourth Edition Clinical Evaluation of Language Fundamentals – Fourth Edition Expressive vocabulary Sentence structure NEPSY Comprehension of instructions Verbal fluency Children’s Memory Scale Dot locations Learning Stories Immediate recall Delayed recall Word list Learning Woodcock Johnson Psychoeducational Battery – Third Edition Memory for names Learning Delayed recall Memory for sentences Adaptive Behavior Assessment System – Second Edition Conceptual Social Practical General adaptive composite

Table 9.3 Intellectual Wechsler Intelligence Scale for Children – Third Edition Information Vocabulary Word Reasoning Block Design Matrix Reasoning Picture Concepts Symbol Search Verbal IQ Performance IQ Full Scale IQ

L.B. Blackburn

Score

Percentile

(14)

91st

100 100 101

50th 50th 53rd

(9) (4)

37th 2nd

(7) (11)

16th 63rd

(6)

9th

(6) (6)

9th 9th

(1)

<1st

79 86 57

8th 12th 0.2nd

80 93 48 67

9th 32nd <0.1st 1st

Score (11) (8) (8) (6) (6) (7) (7) 93 77 82

Percentile 63rd 25th 25th 9th 9th 16th 16th 32nd 6th 12th

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grooming and dressing as well as helping out with household tasks. In part, delays in this area reflected the challenges created by her hemiparesis. For Betsy to be independent, she needed to learn one-handed approaches to tasks most children do with two hands. However, her delays in this area also reflected the lingering effects of the year in which it was unsafe for Betsy to do things on her own. Betsy’s parents were encouraged to reevaluate her need for them to do many things for her that she could now safely do independently.

Formulation and Recommendations Betsy’s school program was reviewed in the context of test findings. Test results suggested that Betsy was receiving appropriate classroom accommodations. It was recommended that these accommodations be continued. Betsy had done well in kindergarten, and it was recommended that her IEP be expanded to support continued success. Given the difficulty she had scanning, tracking, and dealing effectively with complex detailed visual information during testing, her parents were encouraged to talk with Betsy’s ophthalmologist to determine whether occupational therapy services would be helpful in developing these skills. It was also recommended that the occupational therapist monitoring her school progress consult with her classroom teacher regarding modification of worksheets and use of adaptive devices (e.g., line guide or shield exposing one line at a time [work window]). It would be important that her teacher avoid ‘‘high interest worksheets’’ where an academic task is embedded in an interesting background. Preferential seating was recommended, placing Betsy at the right side of the classroom, so that most of the classroom would be in her intact left visual field. Given Betsy’s need for frequent repetition of directions during testing, her teachers were encouraged to monitor her understanding of classroom directions by having Betsy demonstrate what she should do before working independently. Her parents were also encouraged to monitor Betsy’s development of skills, such as being able to copy material from the blackboard (visually tracking between paper and blackboard) and to maintain column alignment in math as multidigit numbers are introduced. Because of her tendencies to perseverate, her parents were also warned that she might have difficulty shifting between math operations, for example, if she is asked to complete a page involving some addition and some subtraction problems. Changes in directions for recurring classroom tasks may also be a problem for Betsy (e.g., being told to underline answers instead of circle them). It was explained to Betsy’s parents that she was at risk for greater problems in learning as she progressed through school. Although her overall intellectual ability fell in the low average range, she demonstrated a pattern of doing well on tasks involving single facts and doing less well when needing to integrate facts and flexibly apply them. Her progress to this point has reflected an intact ability to learn individual facts when taught in a multisensory fashion (combining visual cues with verbal description and hands-on experience). As Betsy progresses in school, the

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amount of material expected to be learned at one time increases, repetition decreases, and the emphasis shifts to learning through language. Although the lower elementary grades maintain a primary focus on fact learning, expectations to flexibly apply learning gradually increases. It was impossible to predict from the current data whether or not Betsy would be able to keep up with all these changes. Close monitoring of her development through repeated neuropsychological assessment was recommended in 1 year to track her progress. In addition to determining the need for adjustments in educational programming, results from repeated testing would allow the neuropsychologist to begin to estimate Betsy’s developmental trajectory, that is, her rate of cognitive growth. This information could be used to help her parents set appropriate long-term goals for her. In addition, it was hoped that Betsy would be better able to tolerate the challenges of memory testing, allowing for a better estimate of her memory skills. Betsy was a child whose developmental story was in the early chapters. It was clear that her parents made the right decision. Betsy’s frequent seizures and her high risk for injury would have significantly limited her development of cognitive skills and social skills. Betsy was doing a remarkable job of learning, using only one hemisphere of the brain. Only time would tell if half a brain would provide her with a normal life, but clearly it already had provided her with a better life.

Additional Resources Key Concepts and Terms Epilepsy A diagnosis of epilepsy is given when a person has experienced two or more unprovoked seizures. Unprovoked seizures are seizures that occur without an immediate cause such as high fever, brain infection, or brain injury. Gait The pattern of movement made by the arms and legs when walking. Hemispherotomy A neurosurgical procedure in which a large portion of one hemisphere of the brain is removed and the remaining connections between the two hemispheres are severed. Hemiparesis A reduction in or loss of muscle control is referred to as paresis. A hemiparesis involves changes in muscle control for one side of the body (arm and leg). Muscles may become limp (flaccid) or may show an increase in muscle control (spastic). Ketogenic Diet A very high fat, carbohydrate-restricted diet effective in reducing seizure frequency in some individuals with epilepsy. The Atkins Diet, made popular as a method of weight loss, is a less restrictive form of this diet. The Atkins Diet has also been shown to reduce seizure frequency for some young adults with poorly controlled epilepsy. Mirror Movements Involuntary movements in one hand that approximate the voluntary movements carried out by the other hand.

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Perseveration The repetition of a response even when the person making the response is aware at some level that it is no longer appropriate. Perseveration may take the form of repeating an action or procedure such as adding all the problems on a page containing both addition and subtraction. Perseveration may take the form of a verbal response, such as repeatedly asking a question even though it has been answered. Seizure A burst of abnormal electrical activity in the brain that produces an observable change in consciousness and/or behavior. This burst may involve the whole brain (generalized seizure) or only a small area of the brain (partial seizure). Seizures may present as sudden sensations (e.g., a funny taste, feelings of fear), staring, semipurposeful movements (e.g., picking at clothing), or as abnormal movements (e.g., stiffening, jerking). Visual Field Deficit A limitation in peripheral vision. Visual fields are divided into four quadrants (upper/lower for right and left). Deficits may involve one side (e.g., all information to the right or left) or one quadrant (e.g., upper right or lower right). A person can compensate for this deficit by moving the eyes or turning the head.

References Resources for Clinicians Basheer, S. N., Connolly, M. B., Lautzenhiser, A., Sherman, E. M., Hendson, G., & Steinbok, P. (2007). Hemispheric surgery in children with refractory epilepsy: Seizure outcome, complications, and adaptive function. Epilepsia, 48, 133–140. Griffiths, S. Y., Sherman, E. M., Slick, D. J., Eyrl, K., Connolly, M. B., & Steinbok, P. (2007). Postsurgical health-related quality of life (HRQOL) in children following hemispherectomy for intractable epilepsy. Epilepsia, 48, 564–570. Pellock, J. M., Dodson, W. E., Bourgeois, B. F. D., Nordli, D. R., & Sankar, R. (2007). Pediatric epilepsy: Diagnosis and therapy (3rd ed.). New York: Demos Medical Publishing. Pulsifer, M. B., Brandt, J., Salorio, C., Vining, E. P. G., Carson, B. S., & Freeman, J. M. (2004). The cognitive outcome of hemispherectomy in 71 children. Epilepsia, 45, 243–254.

Resources for Families Blackburn, L. B. (2003). Growing up with epilepsy: A practical guide for parents. New York: Demos Medical Publishing. Freeman, J. M., Freeman, J., & Kelly, M. (2000). The ketogenic diet: A treatment for epilepsy (3rd ed.). New York: Demos Medical Publishing. Freeman, J. M., Vining, E. P. G., & Pillas, D.J. (2002). Seizures and epilepsy in childhood: A guide for parents. Baltimore: Johns Hopkins University Press.

Chapter 10

Pathological Left-Handedness: Stroke and Seizures Elizabeth N. Adams

Lily weighed 7 pounds, 6 ounces at birth and, apart from mild jaundice, was a healthy infant. Her proud parents were optimistic about their firstborn’s new life and, if asked, would have said that they did not expect her to have medical problems, because no one on either side of the family had any chronic medical conditions. When Lily was around 2 years of age, her parents observed that she seemed to prefer her left-hand for tasks such as coloring and eating. They were somewhat surprised by this because they knew that handedness tended to be a hereditary trait and were not aware of any relatives who were left-handed. Then, as their daughter started preschool, Lily’s parents observed that her left-hand preference seemed particularly pronounced. In fact, it seemed as though Lily were not using her right hand much at all. Curiosity led to closer observation, which revealed a subtle dragging of her right leg with a tendency to ‘‘throw’’ it outward when she walked and ran. Lily’s parents sought advice from their pediatrician, who referred their daughter to a neurologist for an evaluation. The neurologist assessed the strength and mobility of Lily’s arms, hands, and legs and told her parents that she had a mild right hemiparesis (i.e., weakness or limited use of the right side of the body). The neurologist then ordered two brain imaging studies, which showed that, unbeknownst to her parents or other treating providers, Lily had experienced an episode of bleeding on the left side of her brain before birth (i.e., an intrauterine cerebral vascular accident (CVA), commonly known as a stroke). Because the left side of the brain, which controls the movements of the right side of the body, was damaged, Lily demonstrated an early preference for using her left hand. Apart from this, Lily’s development had been normal to this point. At the expected times she reached milestones in language development, including use of first single words, vocabulary increase, and use of two to three words in phrases. Shortly after Lily turned 3 years of age, her parents observed a recurring behavior lasting several seconds during which she suddenly turned her head to the right and began shaking her right arm and leg. Concerned and wondering if this had anything to do with the intrauterine stroke, they took her to see the neurologist again. The behaviors were described as seizures, and an electroencephalogram J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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(EEG) showed that they were indeed coming from the part of the brain where the intrauterine stroke had occurred. Lily was prescribed an antiepileptic medication to control the seizures. She did not have another seizure for 5 years, but then at the age of 8 years the same set of recurring behaviors (i.e., seizures) was observed and the frequency increased. Despite attempts to control the seizures with medication over several years, Lily continued to have them. The neurologist explained to Lily’s parents that after several unsuccessful medication trials, another antiepileptic medication was quite unlikely to be effective in stopping the seizures. The next best option would be to surgically remove the portion of her brain from which her seizures were originating. Lily was age 13 years at this time. Thus, Lily’s neurologist began to consider her as a possible candidate for epilepsy surgery. As was explained to her parents, surgical candidacy typically involves a several-month process of collecting and integrating data from various sources, including structural and functional brain imaging, long-term video-EEG monitoring, and neuropsychological testing. The neurologist referred Lily to the neuropsychology clinic in the hospital for a presurgical evaluation of her cognitive and behavioral functioning. The evaluation was specially designed to provide information that would be beneficial to the neurologist in planning for surgery as well as to Lily and her family in continuing to support her academic and psychosocial development through her challenging medical condition. At the time of the neuropsychological evaluation, Lily was 14-years old and in the seventh grade at a middle school, where she received special education support for all academic subjects. She also was receiving occupational therapy services to address her right-sided hemiparesis. Lily was described by her parents as being immature. Although she was seen as a good-natured, kind girl who was outgoing and wanted to fit in, she had difficulty because of social awkwardness (e.g., restricted interests, lack of social awareness, and difficulty interpreting nonverbal and other subtle cues). Nevertheless, Lily did have a few friends at school and generally was accepted by the other children in her class. Lily’s parents also reported that she had difficulty sustaining attention in most situations and was easily distracted.

Test Results The neuropsychological evaluation consisted of a review of available medical and academic records; interviews with Lily and her parents; and administration of tests of intellectual abilities, higher executive functions, sustained attention and inhibition of impulsivity, selected language and visual-spatial functions, memory functions, motor and tactual-perceptual functions, and academic achievement. Lily arrived on time for the evaluation, with her parents. She was a pleasant teenaged girl, who demonstrated obvious right-sided hemiparesis (i.e., rigid gait favoring the left side and limited right hand manual motor movement). Lily was able to talk with the examiner fluently and without errors. However, the manner in which she spoke resulted in the content of her speech seeming tangential and circum-

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locutious. That is, she demonstrated word-finding difficulties and often described characteristics of the word or concept she was attempting to talk about without addressing the target word or concept directly. Lily demonstrated an extremely slow rate of mental processing, especially noticeable on two tasks (one oral, one written) that required her to formulate a response with appropriate content in response to a question. Her approach to these tasks was very deliberate, and the content of her responses was appropriate and well thought-through. Thus, despite her extremely slow processing, when given ample time she successfully answered a set of quite challenging questions. Lily remained polite and cooperative throughout the entire evaluation. She was noticeably distractible but was easily redirected to the task at hand. In general, Lily’s compliance was excellent throughout the duration of the assessment; her effort and cooperation during testing were viewed as adequate to ensure that the reported results were reliable and valid (Tables 10.1–10.3). Lily’s test results revealed intellectual functioning in the mildly cognitively deficient range overall. The majority of the test scores in her neuropsychological profile fell within or below this broad range of impairment. Within this range, her performance on measures of verbal reasoning, verbal working memory, and discrete language functions was slightly less impaired than on measures of nonverbal reasoning and visual-spatial functions. In addition, her memory for verbal material consistently was stronger than her memory for nonverbal material. Lily’s parents’ description of her attentional difficulties, also observed informally during the testing, was corroborated by the finding of poor sustained attention on a continuous performance task. Measures of higher executive functions including speed and flexibility of mental processing and abstract problem solving yielded significantly impaired scores. Academic achievement testing indicated relative Table 10.1 Intellectual Wechsler Intelligence Scale for Scorea Children – Fourth Edition Full Scale IQ 63 Verbal Comprehension Index 75 Similarities (6) Vocabulary (3) Comprehension (6) Perceptual Reasoning Index 69 Block Design (4) Picture Concepts (5) Matrix Reasoning (6) Working Memory Index 80 Digit Span (5) Letter-Number Sequencing (8) Processing Speed Index 56 Coding (3) Symbol Search (1) a Standard score, (scaled score), [T-score], {z-score}.

Percentile 1st 5th 9th 1st 9th 2nd 2nd 5th 9th 9th 5th 25th 34th 25th 50th

90 Table 10.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition Broad reading Letter-word identification Passage comprehension Reading fluency Basic reading skills Letter–word identification Word attack Broad math Calculation Applied problems Math fluency Broad written language Spelling Writing samples Writing fluency Academic skills Academic applications Academic fluency

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Score

Percentile

Grade equivalent

77 85 70 77 88 85 90 80 83 78 91 71 78 84 62 79 72 72

6th 16th 2nd 7th 20th 16th 26th 9th 13th 7th 27th 3rd 7th 15th 1st 8th 3rd 3rd

3.8 4.8 2.7 3.7 4.8 4.8 4.7 4.9 5.3 3.8 7.1 3.5 3.8 4.8 2.8 4.6 3.4 3.8

strengths in basic skills (i.e., single-word reading, phonetic analysis, simple arithmetic) and relative weaknesses in the application of those skills. Although Lily’s fine motor speed, dexterity, and grip strength were significantly impaired with her dominant left hand, she was completely unable to perform measures of these functions with her right hand. The same pattern was observed on testing of finger graphesthesis. Testing of finger localization demonstrated bilateral finger agnosia that was more prominent with the right hand than with the left hand.

Formulation and Recommendations The next step in the evaluation was to consider the results of the cognitive and motor testing in conjunction with the findings available from other sources including neuroimaging, prolonged video-EEG, and neurological examination. Before discussing the case-specific findings, it is important to understand one of the basic rubrics for interpretation of neuropsychological test data. Several decades of scientific research have yielded a tenet that is especially applicable in assessment of individuals with neurological conditions. In a typically developing brain, language, verbal reasoning, and verbal memory typically are mediated by left hemisphere structures, while visual-spatial organization, nonverbal reasoning, and nonverbal memory are mediated by right hemisphere structures. A simplified form of this general principle is ‘‘left-side language, right-side spatial.’’ When a neurological insult occurs in a mature brain (i.e., during late childhood and beyond), this general principle applies and specific types of cognitive dysfunc-

10 Pathological Left-Handedness: Stroke and Seizures Table 10.3 Other cognitive functions Tests Gordon Diagnostic System Vigilance task (12 min) Correct Commission errors Symbol Digit Modalities Test

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Score

Percentile

54 76 86

<1st 5th 18th

Trailmaking Test Part A Part B

74 <1

4th <1st

Wisconsin Card Sorting Test Categories Perseverative errors Verbal Fluency Test (FAS) Animal Fluency Boston Naming Test

45 34 74 64 37

<1st <1st 4th 1st <1st

Multilingual Aphasia Examination Token Test Sentence Repetition Test Benton Judgment of Line Orientation Test Benton Facial Recognition Test

106–107 62–72 32 43

67th 1st–3rd <1st <1st

Wide Range Assessment of Memory and Learning – Second Edition Story memory Recall Recognition Verbal learning Recall Recognition Design memory Recognition Visual learning Delayed recall

(7) (6) (6) (10) (10) (10) (4) (8) (4) 74

16th 9th 9th 50th 50th 50th 2nd 25th 2nd 4th

Finger Tapping Test Left hand

49

<1st

Grooved Pegboard Test Trial 1 left hand Trial 2 left hand

39 39

<1st <1st

Grip strength Left hand

77

6th

Finger Gnosis Right hand Left hand Right hand (errors) Left hand (errors) Finger Graphesthesis Left hand Left hand (errors)

<1 19 <1st <1st 44 <1st (continued)

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Table 10.3 (continued) Tests

Score

Percentile

Achenbach Child Behavior Checklist Parent Form Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior

[63] [60] [67] [84] [72] [81] [62] [60]

90th 84th 96th >97th >97th >97th 89th 84th

Teacher’s Report Form Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior

[71] [67] [69] [68] [79] [68] [63] [65]

>97th 96th 97th 97th >97th 97th 90th 93rd

tion can be expected on the basis of the site of the insult or damage. However, when the insult occurs before birth or during early infancy, the cognitive outcome is not as predictable. Because the sets of structures that work together to mediate language and spatial thinking abilities are not fully differentiated in these early stages, we typically do not see the expected pattern of deficits following brain compromise that can be expected when a more mature brain is damaged. This case provides a helpful example of what can happen when the expected pattern is changed because of timing and location of neurological insult. Lily’s left-hemisphere stroke occurred before birth, thereby impacting the subsequent trajectory of her brain development. As a consequence, she does not demonstrate the typical pattern of cognitive dysfunction (i.e., impaired language and intact visual-spatial functions) that would be expected following a later-onset left-hemisphere injury. Rather, Lily’s cognitive testing reveals an intriguing pattern characterized by more severely impaired visual-spatial than language functions and more severely impaired nonverbal than verbal memory functions. These findings are the opposite of what would be expected following a left-hemisphere insult in an older individual with a more mature brain. Together with her right-sided hemiparesis, Lily’s neuropsychological profile is consistent with what has been termed a ‘‘pathological left-handedness’’ syndrome. Pathological left-handedness is a pattern of left-handedness without family history of left-handedness, relatively poorer right hand motor function along a continuum of severity, and better developed language than visual-spatial functions. It is seen as preceded by an early left-hemisphere insult (or possibly by a developmental anomaly

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of or within the left cerebral hemisphere). Like Lily, some people with the syndrome may develop seizures originating from the left hemisphere. This syndrome is consistent with a construct of language development that remains partially theoretical yet is commonly accepted. Within this theory, language is the dominant cognitive function because it is most integral to adaptive functioning and participation in our highly language-dependent world. As such, the dominant (left) hemisphere is primed even before birth to house language functions. This hemisphere is particularly good at processing discrete pieces of specialized information in a temporal manner, aiding language use, and also is the hemisphere with the greatest degree of control over finely coordinated motor movements such as those required by the speech output system. When an early left-hemispheric insult is sufficient to compromise the development of language in the left hemisphere, which is the dominant hemisphere for approximately 94–96% of people, theory with support from clinical research suggests that the brain reorganizes to delegate language development to the more intact structures in the nondamaged (right) hemisphere. Because this process of reassignment of language functions to the right hemisphere often seems to take place at the expense of the full development of the visual-spatial functions that ordinarily would be represented in that hemisphere, it is referred to as crowding. Coincident with this process of language representation moving to the right hemisphere, thus establishing the dominance of this hemisphere, is the development of left-handedness without family history of such. This essentially provides behavioral evidence of the level of severity of the early left-hemisphere insult, which was marked enough to cause this shift of hand dominance counter to the odds of heritability. The results from this evaluation were provided to Lily’s neurologist, who integrated the data with other clinical and laboratory findings to ensure optimal understanding of her brain functioning as part of her candidacy for resective brain surgery. In addition, several recommendations were offered to Lily’s family that focused on decision-making about school programming, suggested accommodations for this girl in the school setting, and improvement of her psychosocial adjustment in the context of a chronic medical condition and anticipation of surgery. Lily’s academic skills represented a relative strength in her overall profile. This was discussed with her family and explained as being likely related to her strong verbal memory as well as to her supportive family and educational environments, which were encouraging and challenging her to continue learning. In planning Lily’s curriculum, school personnel were advised to consult her test scores in order to establish an appropriate starting point for her in each academic domain. In addition, it was noted that Lily’s deficit in higher-order conceptualization was expected to translate into difficulty with the applied aspects of academic skills. Similarly, her slowed processing speed, combined with her known fine motor difficulties, was expected to result in less efficient academic fluency. Thus, it was emphasized that Lily’s actual school performance could represent an underestimate of her knowledge of basic academic skills. Recommended school accommodations for Lily included shortened assignments, extended time on tests, and use of a word processor for writing assignments.

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Several compensatory strategies were also suggested. For example, Lily was encouraged to use her strength in rote memory by mastering basic skill sets (e.g., analytic phonics, math facts, spelling rules). Then, when she had demonstrated adequate knowledge in a basic skill set within a particular academic domain, Lily’s teachers and parents were encouraged to challenge her to apply the skills further or to use them fluently. Finally, Lily’s parents were given several sets of suggestions for managing her attentional difficulties, as well as contact information for psychologists who could provide social skills training and therapy for Lily in light of her parents’ concerns about her inattention and social immaturity. It was anticipated that, with the appropriate supports and accommodations, Lily’s prognosis would be positive. She demonstrated a relative strength in verbal memory, which often serves an important purpose in the process of acquiring new skills and consolidating new to-be-learned information. Also, Lily had a supportive family environment.

Additional Resources Key Concepts and Terms Electroencephalogram A diagnostic test used by neurologists to assess electrical activity in the brain. It is a noninvasive procedure that involves temporarily gluing sensors to the scalp; the sensors send output to a computer, which shows on a graph the pattern of electrical discharges in the corresponding cerebral areas. Abnormal patterns are sometimes associated with seizures. Hemiparesis Weakness or limited use of one side of the body, usually caused by a neurological injury in the contralateral hemisphere. Intrauterine Stroke A cerebral vascular accident (CVA), that is, an episode of bleeding in the brain, which occurs before birth. Jaundice A condition occurring during the postnatal phase and sometimes extending through early infancy that is caused by elevated levels of bilirubin (i.e., ‘‘hyperbilirubinemia’’). Severe jaundice can cause bilirubin encephalopathy, which has been shown to have effects on neurodevelopment, and sometimes ongoing cognitive deficits are observed. Mild jaundice is quite common and most often benign.

References Resources for Clinicians Dodrill, C. B., & Matthews, C. G. (1992). The role of neuropsychology in the assessment and treatment of persons with epilepsy. American Psychologist, 47, 1139–1142.

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Foldvary-Schaefer, N., & Wyllie, E. (2003). Epilepsy. In C. Goetz (Ed.), Textbook of clinical neurology (2nd ed.). Philadelphia, PA: Saunders. Hertz-Pannier, L., Gaillard, W. D., Mott, S. H., Cuenod, C. A., Bookheimer, S. Y., Weinstein, S., et al. (1997). Noninvasive assessment of language dominance in children and adolescents with functional MRI: A preliminary study. Neurology, 48, 1003–1012. Miller, J. W., Dodrill, C. B., Born, D. E., et al. (2003). Atypical speech is rare in individuals with normal developmental histories. Neurology, 60, 1042–1044. Strauss, E., Satz, P., & Wada, J. (1990). An examination of the crowding hypothesis in epileptic patients who have undergone the carotid amytal test. Neuropsychologia, 28, 1221–1227. Woods, R. P., Dodrill, C. B., & Ojemann, G. A. (1988). Brain injury, handedness, and speech lateralization in a series of amobarbital studies. Annals of Neurology, 23, 510–518.

Resources for Families Ellis, N., Upton, D., & Thompson, P. (2000). Epilepsy and the family: A review of current literature. Seizure, 9, 22–30. Epilepsy Foundation. (2005). Answer place: Introduction, http://www.epilepsyfoundation.org/ answerplace/quickstart/forparents/index.cfm.

Chapter 11

Moving Forward: Psychotherapy with a Youth After Brain Injury Mary C. Kaven

Shaun was sitting quietly on the couch in the dayroom of the residential treatment center (RTC), listening intently to a book on tape with a headset while his peers were talking or playing games. Just under 5 ft and 100 lbs, with curly blond hair and fair, even facial features, he looked more like his 12-year-old peers on the unit than his chronological age of 15 years. Shaun was pleasant and cooperative in conversation. His vocabulary was quite good, and he talked about the Harry Potter books he had heard on tape. He was open about not being able to read or tell time. He could read short words and the numbers on a digital watch, but he did not understand what time his activities and meals took place. He knew his colors, could count aloud into the double digits, and print his first name with difficulty. He asked for help in constructing an animal from a novel building set he saw on a shelf, but he knew what looked realistic and what did not. He denied having any auditory or visual hallucinations, and there was no mention of such in his medical record. He admitted being very picky about food and having trouble ending activities and said he wanted help to tell his schoolteacher how to understand him and help him learn. In a session with his parents, Shaun was teary, more regressed, and oppositional. He told his parents that he does not belong in this world and should die. Shaun had already received lengthy psychiatric treatment in the partial hospital program, followed by acute care for longstanding problems with aggressive outbursts, oppositionalism, suicidality, impulsivity, problems with peers, and learning problems. He had a history of seizures. Now on two mood stabilizers, among other medications, Shaun was having relatively fewer seizures but had not made enough progress to ensure safety at home and school. His behavior in the RTC was similar to what his family had experienced at home and what was reported at school. It was very difficult to get Shaun out of bed and ready for school in the morning. He cursed at staff and sometimes threw things at them. One morning he punched a staff member and held a pencil to the staff member’s chest. During other times of the day, Shaun sometimes kicked a peer—even his closest friend—or hit staff when he was frustrated. With each incident, Shaun was remorseful afterwards and apologized, but he felt worse about himself and more depressed. Consequences of earning

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or losing toys and privileges did not seem to control his behavior even if they were important to him. It was difficult for Shaun to think ahead more than a few hours and, therefore, difficult for him to make any positive changes in his behavior. It was generally agreed that Shaun’s intractable seizures, neuropsychological problems, and emotional and behavioral dysregulation were primarily a consequence of brain injury secondary to viral encephalitis contracted on a family trip shortly after his first birthday. Until that time, his development had been normal. He was born 10 days past term without any complications or exposure to alcohol or drugs, and he was reportedly an easy, content, happy, and cuddly infant. He had begun to walk and speak his first words before he developed influenza-like symptoms and a seizure on the trip, which escalated into status epilepticus. An electroencephalogram (EEG) suggested a low seizure threshold, with a seizure focus in the right central region of the brain. He was hospitalized for 1 month, then again for 2 weeks shortly thereafter, while his seizures were stabilized with medication. He then received physical therapy and occupational therapy for residual left hemiparesis and visual and auditory impairments. Shaun’s difficulties in hearing and seeing resolved within a few months. However, he did not walk or speak again until he was 2½-years old, and then he started speaking in sentences at the age of 4. At the age of 8, a neuro-ophthalmologic exam suggested the presence of a bitemporal field defect and an incongruent left hemi-field deficit. Antiepileptic medications were eventually discontinued, and he was largely seizure free until age 13, when seizures reoccurred regularly in clusters and included drop spells without warning. No medication or combination of medications after the reemergence of his seizures were totally successful in controlling them. In first grade, Shaun was hyperactive, inattentive, impulsive, and aggressive to peers. He was frustrated that he could not learn to read or write, and he craved attention. He was placed in a special education class for children with traumatic brain injury. At age 9, he was still aggressive and demanded help for daily self-care such as dressing and toileting. He was violent to family members, particularly toward his older brother when his brother tried to protect their mother from him when he was acting out. Shaun stopped asking for help cleaning himself after toileting at age 15, but he still could not tie his shoes. He continued to be oppositional, impulsive, distractible, aggressive, and easily frustrated. In addition, Shaun was frequently irritable, labile, and overtly depressed. His mother described him as too dependent on her, saying, ‘‘he would like to sit on the couch and have me serve him.’’ She realized that the family had been doing too much for Shaun for too long. Following several urgent visits to the emergency department for threats of suicide, uncontrollable sobbing, and barricading himself in his room, Shaun was admitted to acute psychiatric care and then to the RTC for further help. The primary treatment goals were for Shaun to handle his feelings safely, without causing harm to himself or others, and to get up most mornings with reasonable but not heroic effort from adults. To accomplish those goals, Shaun needed help with depression and becoming more independent. To assist with stabilization and treatment planning for emotional and behavioral dyscontrol, Shaun received a neuropsychological evaluation, magnetic resonance imaging

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(MRI), and an updated EEG. The neurologist described Shaun’s seizure disorder as having features of both complex partial and generalized tonic-clonic seizures. The EEG showed a nonspecific abnormality in the right central/parietal region with slowing, and a lesser degree of focal abnormality in the left temporal region. The MRI was similarly abnormal, showing bilaterally symmetric high T2 signal intensity, involving the inferior temporal lobes and posterior occipital and parietal lobes and sparing the visual cortex. Focal gyral atrophy and gliosis in these regions were, presumably, secondary to Shaun’s history of viral encephalitis as an infant.

Test Results In previous evaluations at age 7 years, 10 months, and age 9 years, 0 months, Shaun’s performance on intelligence and achievement tests had shown his verbal IQ to be in the borderline range and his performance IQ as moderately deficient, with his achievement scores similarly low, including particularly low arithmetic scores. So, although Shaun had difficulty in all areas, he was significantly better on auditory verbal tasks than on visual and constructive tasks. The neuropsychological battery at age 15 was designed to update these previous findings and to provide more comprehensive views of his mental processing skills and adaptive functioning (Table 11.1). Partly due to his psychiatric problems, Shaun’s adaptive skills were comparable to that of a 5-year-old across motor, social and communication, personal living, and community living domains. He still had significantly greater difficulty with visual and performance tasks than auditory verbal ones, but he showed some important changes since the age of 9 years. Although he still could not read, auditory verbal Table 11.1 Intellectual Wechsler Intelligence Scale for Children—Fourth Edition Verbal Comprehension Index Vocabulary Similarities Comprehension Perceptual Reasoning Index Block Design Picture Concepts Matrix Reasoning Working Memory Index Digit Span Letter-Number Sequencing Processing Speed Index Coding Symbol Search Full Scale IQ a Standard score, (scaled score), [T-score], {z-score}.

Scorea 85 (8) (8) (5) 57 (4) (3) (2) 52 (2) (1) 50 (1) (1) 54

Percentile 16th 25th 25th 5th <1st 2nd 1st <1st <1st <1st <1st <1st <1st <1st <1st

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tests were now in the low average to average range compared with his same-age peers. Expressive vocabulary was low average, receptive vocabulary average, and the ability to express and comprehend both simple and complex language was low average to average. Visual processing, reasoning, and problem solving were still quite impaired, as were working memory and processing speed. He also showed severe executive functioning deficits with initiating, sustaining, and shifting attention, maintaining set, and learning from feedback. Shaun’s ability to encode and learn a list of words was in the low average to average range, although his source memory was poor. His performance on sensory-perceptual and motor tests was impaired as well, with a pattern suggesting relatively greater lateralized right hemisphere dysfunction.

Formulation and Recommendations The pattern of test results indicated severe generalized cortical dysfunction and bilateral dysfunction in specific neuropsychological abilities, with greater involvement of the right hemisphere than the left. Shaun demonstrated relative sparing of expressive and receptive verbal skills and verbal learning and memory. Because his neuropsychological pattern coincidentally was similar to those described in the previous chapters on epilepsy, further discussion of the evaluation is deferred to focus on the nature and course of Shaun’s psychosocial treatment. During the 4 months of residential treatment, Shaun received individual, family, group, milieu, and experiential therapy, pharmacotherapy, and special education. Shaun’s depression, oppositional behaviors, and aggression were seen to be related primarily to negative self-comparisons to others, feeling stuck and conflicted about growing up, and anxieties about his brother growing up and leaving the household. After initial sessions with Shaun and his family, observing Shaun in the milieu, and reviewing the neuropsychological evaluation, EEG, and MRI, the developmental and contextual resources and challenges that would shape the direction and course of treatment were identified. Shaun was an attractive (nondysmorphic), young-looking teen with some good verbal skills, a sense of humor, and the abilities to play and understand metaphor. He was attached to his family, interested in social interaction, and capable of learning and caring about other people. He had a history of showing remorse in some situations. He demonstrated a range of affect, some ability to identify feelings verbally, and no problems with reality-testing. He enjoyed listening to music and books on tape (with earphones) for significant amounts of time. He remembered the stories he heard, and he liked to make up poetic, personally expressive songs. His family was intact and motivated to be supportive of him. Shaun’s parents encouraged him to develop his own interests and had a wide range of interests themselves that they tried to share with him. But his parents were also weary and stressed emotionally, mentally, and financially. Both parents had to work full-time and share the extensive supervision and caretaking of Shaun, sometimes doing shift work to

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manage. Shaun had no friends, and it was hard for his family to find time to help Shaun learn to make those connections. Shaun’s older brother was getting ready to graduate from high school and travel abroad for 6 months—an exciting but major transition in the family structure. Shaun was an adolescent struggling with growing up and becoming more independent. When he was younger, he had tried hard to learn new things and was proud of his accomplishments. He was now discouraged about his progress, afraid of failing to be able to go out on his own like his older brother, and angry about being dependent and lonely. Both his health and neuropsychological deficits contributed to this struggle. He had trouble developing and holding on to a sense of himself. Problems with memory, visual perception, sensory‐motor awareness, and time made it difficult for him to encode, or recall, autobiographical memories, let alone to integrate them into a coherent schema of a self. Likewise, Shaun had difficulty with understanding the transformations that led to the future. His problems with visual memory and associative learning also interfered with his utilizing any object to remind him enough of his parents and his home when he was not there with them so he could feel secure. His sense of time was so impaired that, outside a few hours, he could not feel certain when he last saw his family, when he would see them next, or certainly what it meant for his brother to be gone for 6 months. Shaun also could not be left alone or unsupervised because of his risk of falling unexpectedly from a seizure. Very limited access was available systemically to skilled nonfamily members who could help Shaun explore his interests and the world outside his home safely. When he was away from his family, he felt too insecure— even devastated—and when he was with his family, he felt too dependent. The neuropsychological testing had shown significant improvement in Shaun’s verbal skills since he was 7-years old, compared with his same-age peers, which was very encouraging. Not only had he continued to learn and improve skills to maintain the same standard score as he got older; he improved enough to raise his standard score by at least one standard deviation. Unfortunately, other skills did not improve at the same rate. Because oral skills are so salient to others, it became easy for others to misunderstand and overestimate what Shaun could do personally, socially, and academically. At ages 7 and 9 years, Shaun struggled with nonverbal tasks more than oral tasks. At age 15, this split was even wider. Children with significantly higher verbal than visual-spatial scores often struggle with social skills, feeling secure, handling novel situations, and meeting the expectations of others. In general, this puts them at risk for acting out when they are younger and for becoming depressed and anxious when older. Psychoeducation, including learning to identify and label one’s own emotions, and social skills training are usually helpful. Cognitive behavioral therapy can also be helpful, provided it is geared to a cognitive and developmental level appropriate to the child’s skills. The marked neuropsychological split between Shaun’s performance on verbal and nonverbal tasks likely created a challenge to Shaun’s development. His marked improvement in verbal skills suggested Shaun might be able to utilize more cognitive behavioral strategies than expected for an adolescent with so many cognitive deficits. But this was uncertain and would need to be tried.

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Behavioral interventions were already turning out to be of limited effectiveness, probably for several reasons. Shaun was not sure of what he liked, and he was too depressed to think that earning anything would make a difference. His memory for events and his ability to anticipate something in the future were impaired. Taking the program down a level developmentally, which often works, was still problematic. Earning tokens or toys immediately, for example, did not link up well enough or long enough to make a difference in getting him what he wanted or keep him from acting out impulsively. Not being able to use behavioral interventions reasonably to shape Shaun’s behavior was a challenge. Conflict over dependency is common in adolescents like Shaun who have significant problems with executive functioning. A lot of external regulation is needed at a time when adolescents usually move toward more autonomy. Selfregulation was not a realistic goal for Shaun, and a more appropriate goal was to actively cooperate with his caregivers to regulate himself. Instead of feeling that his only choices were ‘‘Do what I want’’ and ‘‘Do what you want,’’ Shaun would assume enough control and responsibility to say, essentially, ‘‘Both of us are important. I want your help to consider what I want and what you want and to make a good decision.’’ Such a shift represented a big step in developing perspective. Shaun needed to have a strong enough sense of identity as well as self-efficacy. Finding a way to improve Shaun’s sense of time and understanding of calendars would open up a lot of possibilities. Separations might be less devastating. Behavior therapy techniques could become more effective. He might better understand how his behavior affects others. He might be better able to encode, access, and integrate his own memories. Many children with developmental problems have difficulty telling time and understanding how to know what will happen when. Building on strengths and trying out creative techniques usually helps to keep them oriented in a meaningful way. But Shaun’s deficits related to understanding time were particularly challenging. Neither verbal nor nonverbal explanations or metaphors were helpful. Although Shaun had developed some significant verbal strengths, he also had profound difficulties with working memory, sequencing, and perception. He said, in fact, that he did not utilize visual imagery at all when using his imagination. Considering the acuity of his behavior and out-of-home placement, this direction in therapy was tabled in favor of others that were more productive. Cognitive behavior therapy (CBT) can be very helpful in building mood regulation skills in a wide variety of situations. One useful indicator of success in using CBT to treat children with neuropsychological problems is their ability to rate the intensity of their feelings, typically on a scale from 0 (not at all) to 10 (the most intense). Children with less mature cognitive ability will typically describe a feeling as all or none—10 or 0, with nothing in between. Being able to identify feelings and rate their intensity on a graduated scale seems to be associated with greater cognitive maturity, as well as ease in understanding how feelings can change, reverse in intensity, or be mixed. With careful instruction and rehearsal, Shaun was able to rate the intensity of his feelings on such a scale and to notice how the intensity went down after collaboratively identifying and processing what was bothering him. In addition to helping Shaun feel better, this exercise, with practice,

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seemed to help him understand more about the process of change in general and be more hopeful that things could change positively for him. One particular challenge was managing Shaun’s reactivity, high levels of arousal, and slow recovery time. When upset, his resources for information processing were limited. Shaun felt very frustrated when adults tried to get him to process verbally about what was bothering him, which increased the risk for an aggressive outburst. He found it helpful to focus first on calming down with the help of a reassuring adult and a well-practiced, low-demand strategy. When Shaun clearly confirmed that the intensity of upset was no more than 4 out of 10, he could engage in more usual verbal CBT strategies to regulate his mood and solve problems. Shaun was encouraged to notice and let adults know when he was just starting to feel upset, so they could help him intervene early on and be more successful. CBT was helpful in addressing several problem areas related to depression, growing up, family relationships, and self-concept. For example, Shaun struggled with both understanding and accepting growing up. He did not understand puberty and that growing up was inevitable. It was as though he thought that if he refused to be independent, he would therefore not really get older. Yet he envisioned that soon he would be expected to leave home like his brother and support himself, which was overwhelming. Understanding more about puberty and what level of independence would be expected of him when he became an adult, along with information about how he would be supported economically, allayed many of his anxieties. In another example, Shaun said he expected his parents ‘‘to be there for him,’’ which they were a reasonable amount of the time. But his understanding of what that meant was for his parents to attend to him more as one would an infant or toddler. The state of being grown up was hard enough to understand. The process of getting there step by step was conceptually even harder, particularly with regard to himself. Communicating more regularly with another person about his feelings with the structure of a list of feelings and rating scales had an added benefit. ‘‘I feel’’ statements helped to create a personal perspective and sense of self. Shaun became clearer about himself and more capable of taking responsibility for his actions. He also began to construct the feelings of others. When he was very calm, he could construct both his own feelings and the feelings of others at the same time. But he could not tolerate these feelings emotionally. Capable of empathy, shame, and guilt, he became overwhelmed with how his behavior affected others. Group exercises that focused on cooperation, flexibility, empathy, and perspective-taking were all helpful. Additional medications to help him manage his reactivity and level of arousal were helpful as well. Shaun then became more aware of how his own thoughts and feelings fluctuated. He had already begun to construct his own perspective along with the perspective of someone else and to look at them together. Now he was able to look at himself under two different circumstances and to compare them. Shaun described feeling like a ‘‘split personality,’’ understanding that to be metaphorical. He said he usually felt weak and vulnerable and as if he were 12-years old or older. In the destructive mood, by contrast, he felt stronger physically, mentally, and emotionally and as though he were 8 or 9-years old. He began to think of himself as feeling stronger on

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a regular basis and more like his current age. Importantly, he was developing a more integrated sense of himself. This, in turn, seemed to facilitate his memory for personal experience. Shaun was continuing to develop his verbal skills for social and emotional purposes and used them gradually to mature. Shaun was in residential care, while his brother embarked on a 6-month journey overseas. Shaun could not understand what 6 months was or compare that time to anything else, such as how long he had been in the hospital or how long it was between birthdays. Shaun said his brother was the only young person who would play with him, and he experienced the separation as a real loss. Shaun recalled the many fights he had with his brother and talked about his intense anger at his brother for those fights—something Shaun had done previously with little resolution. This time was different. Shaun could now put more of the pieces on the table, stand back, and look at them without decompensating. Shaun had a better sense of himself as an agent—one who could take responsibility for his actions and tolerate it emotionally. He could also construct and reflect on more than one perspective. Shaun now accepted that he had been violent toward his family members. Finally, he could understand and accept that his brother had not started the fights but had tried to keep Shaun from hurting their mother and had intended to be protective instead of hurtful. Throughout the RTC treatment, Shaun was prescribed multiple medications to target seizures, impulsive aggression, depression, reactivity, irritability, and inflexibility. The benefit was sometimes marginal, and the side effects were significant, leading to frequent medication changes. Unfortunately, some antidepressants appeared helpful at first but soon became too activating and required discontinuation. Other medications reduced aggression but increased depressive symptoms or were too sedating, an even bigger concern for Shaun because of his already significant risk of falling from uncontrolled seizures. However, even the discontinuation of some medications created opportunities to work on depression, posttraumatic stress disorder, mood regulation, and separation and individuation. These led to developmental gains that were maintained overall. Shaun’s medications at his discharge included two anticonvulsants and a neuroleptic. Unlike many other young people with neuropsychological problems, Shaun benefited more from the cognitive than the behavioral interventions. His difficulties with visual perception, encoding context, and understanding time were particularly challenging. But when medications helped to keep his reactivity and level of arousal within a workable range, Shaun could collaborate in building on his verbal strengths. This facilitated progress in the development of a more organized and stable sense of self and more acceptance of the process of growing up and becoming more independent. He was then more able to construct the perspective of other family members. Most significantly, he could finally process and revise his understanding of the conflict in his relationship with his older brother and cope safely with fewer medications. The external systems issues were also challenging and unfortunately had a negative impact on Shaun’s progress at several points. Imagining what services

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he needed did not guarantee their availability. Shaun was discharged when his aggression remitted, his mood and morning functioning improved, and outpatient resources were adequate. Everyone hoped he would find it easier to improve his self-care, self-regulation, and interpersonal skills and welcome growing up. A neuropsychological evaluation can be valuable when treating a child or adolescent with neuropsychological problems. However, like a topographic map, the evaluation helps to prepare the neuropsychologist but does not tell where to go or exactly how to get there. That is a journey best undertaken collaboratively with the individual child and family. Sometimes a bridge expected to be there is out, like Shaun’s difficulties with time. Other times a shortcut or a store of hidden resources can be discovered. This quest is a constructive and dynamic process that might even change the landscape. There was urgency, so with help Shaun took an alternate route home. There are still some theoretical issues to ponder. For example, what was Shaun’s imagination really like, and how did he experience it? Also, what neurobiological and neuropsychological problems were responsible for Shaun’s difficulties with time? Perception of time is a complex area of study. A variety of brain mechanisms appear to be involved depending on the time scale being studied—milliseconds, seconds, hours, or days—and there is little understanding of how mechanisms at any of these levels may be related. Several studies have implicated the right hemisphere, particularly the frontal area, as particularly important in the perception of passage of time. But several researchers, including Alexander, Cowey, and Walsh (2005), suggest there is a role for a right frontal-parietal network. Of note, the results of Shaun’s neuropsychological evaluation were consistent with particular difficulties in right hemisphere functioning, including frontal and parietal areas. Abnormalities in the right parietal area were also very clear on his EEG and MRI.

Update Shaun, no longer a minor, and his mother freely gave permission for his history and therapy to be described because they thought other young people in similar circumstances might be helped. More services are available to Shaun and his family now, which has reduced much of his family’s stress. Shaun’s mother helps him make jewelry, which he sells through the Association for Retarded Citizens (ARC). He also likes to ‘‘mess with’’ electronics, enjoyed taking a shop class, and trains dogs for others with disabilities. Shaun’s mobile phone is ever present, like a transitional object. He has memorized all the icons and menus on the phone, and the background screen has pictures of each of his family members. He likes to put video clips from family vacations on his phone and surprise his mother. Understanding time continues to be a struggle, but when his parents take a weeklong vacation, for example, Shaun is comfortable when a respite worker stays with him, even if he

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cannot understand or track when his parents will return. He now has a vagus nerve stimulator (VNS) to help control his seizures but still needs close supervision by a caretaker to help him manage his seizure activity. His mother thinks the VNS has also helped with Shaun’s depression, focus, and memory. Developing same-age friends is still a problem, primarily because it has been hard to find others of his age with similar abilities and interests. His older brother has continued to travel overseas since the big first trip that was so hard for Shaun to comprehend and tolerate emotionally when he was in the RTC. When asked how he felt about his brother’s trips, Shaun said, ‘‘I was happy for him and upset.’’ Shaun and his brother no longer fight; neither is Shaun aggressive. His mother described, ‘‘Maybe a door slam or curse word, and he apologizes afterwards and communicates better. He might say, ‘I didn’t like the tone of voice you were using.’’’ A small detached residence on the family’s property is available for Shaun to use when he feels ready. In retrospect, his mother feels that the RTC was instrumental in helping Shaun and his family separate from each other and in assisting Shaun to become more independent.

Additional Resources Key Concepts and Terms Hemiparesis. Weakness on one side of the body, involving both the arm and leg and sometimes the face. It is associated with a variety of medical conditions; among the most common are stroke and head injury. In such cases, the brain insult or injury is often on the opposite side from the weakened arm and leg. Hemiparesis may be a temporary condition. Prognosis will depend on the medical condition. Source Memory. The aspect of episodic memory related to the context of the item or experience being recalled. For example, if the content of a girl’s memory is ‘‘Grandma is coming for my birthday,’’ the context would include how and when she found out her grandmother was coming. Did her mother tell her, did she read it in a letter, or did she just imagine it? That is, what was the source of the information being recalled? The capacity for source memory normally increases during early childhood. Status Epilepticus. A seizure or series of seizures lasting at least 30 min without complete recovery of mental functioning. It is considered to be a medical emergency because it may lead to a variety of medical difficulties, including hypoxia, brain swelling, very low blood pressure, difficulties breathing, or even death. Seizures may be either convulsive or nonconvulsive. An EEG may be required to confirm the presence of nonconvulsive seizure activity.

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References Resources for Clinicians Alexander, I., Cowey, A., & Walsh, V. (2005). The right parietal cortex and time perception: Back to Critchley and the Zeitraffer phenomenon. Cognitive Neuropsychology, 22(3/4), 306–315. Childswork/Childsplay. Web site: http://www.childswork.com Friedberg, R. D., & McClure, J. M. (2002). Clinical practice of cognitive therapy with children and adolescents: The nuts and bolts. New York: Guilford Press. Johnson, S. M. (1994). Character styles. New York: Norton. Kendall, P. C. (2006). Child and adolescent therapy: Cognitive-behavioral procedures (3rd ed.). New York: Guilford Press.

Resources for Families Academy of Cognitive Therapy, http://www.academyofct.org Stewart, K. (2007). Helping a child with nonverbal learning disorder or Asperger’s disorder: A parent’s guide. Oakland, CA: New Harbinger.

Chapter 12

Family Matters: Psychosocial Factors on Neuropsychological Outcome Grace W. Fong

Jamie was a typically developing, healthy, and active teenaged girl who enjoyed life. She did well in school, especially in math and science, and dreamed of becoming a science teacher. Jamie and her family were not initially alarmed when she seemed to come down with the flu after returning from a family vacation in Maine. However, when the usual medicines just did not seem to work and Jamie’s health instead began declining rapidly, her mother decided it was time to see the doctor. A blood test led to other tests, and soon, without warning, Jamie was diagnosed with leukemia, a form of cancer in the blood (Box 12.1). Jamie’s world was immediately turned upside down. She barely had time to even think about her new diagnosis before having to start an aggressive chemotherapy treatment. Her parents were devastated and scared as they tried to sort through the impact of this diagnosis on their daughter’s future. Over the next few weeks, Jamie’s health problems multiplied as she suffered a left-sided stroke that resulted in right-sided hemiparesis (weakness) and aphasia (language problems). Jamie was rushed to the hospital and underwent multiple surgeries to address complications related to the stroke. After her surgeries, Jamie was transferred to an inpatient rehabilitation unit to learn to function independently again. At that point, the right side of Jamie’s body was paralyzed, and she could not speak, control her bladder, or eat independently and thus required a feeding tube. During inpatient rehabilitation, Jamie received speech and language and occupational therapies. By the time she was discharged, she had made significant progress in her ability to walk, speak single words, and eat independently. Arrangements had been made for her to continue to receive speech and language, occupational, and physical therapies in addition to special education placement through her local public school. Unfortunately, when Jamie was transferred to a different school later that school year, she was discharged from all of her therapies, although she retained her special education placement at her new school. Jamie rarely had attended classes because of a home-bound medical classification that excused her from attending school when she was feeling ill due to side effects from chemotherapy. However, Jamie continued to remain home from school on most days even after her chemotherapy treatments were completed. This was

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Box 12.1 Leukemia is a general name for cancer in the blood. There are four main types of leukemia: l l l l

Acute Myelogenous Leukemia (AML) Acute Lymphocytic Leukemia (ALL) Chronic Myelogenous Leukemia (CML) Chronic Lymphocytic Leukemia (CLL)

Leukemias are classified as either acute cancers that develop rapidly or chronic cancers that develop slowly. Most leukemias in children are acute. Leukemia affects cells in the bone marrow that undergo a leukemic change and multiply until they eventually outnumber normal cells. Lymphocytic (or lymphoblastic) leukemia affects the marrow cell that forms a type of white blood cells called lymphocytes. In myelogenous leukemia, cellular changes occur in marrow cells that form red blood cells, some other types of white blood cells, or platelets. ALL is the most common cancer in children, especially in those between ages 2 and 8 years. For unclear reasons, ALL is more common in Caucasian children than African-American children, although it appears to be the most common in Hispanic children. Prenatal exposure to X rays or later exposure to high doses of radiation appears to increase the risk of ALL. Individuals with Down syndrome or other genetic conditions appear to have increased risk for ALL. Signs and symptoms of leukemia are not specific to leukemia but include fatigue, tendency to bruise easily, and recurrent minor infections. Diagnosis of leukemia typically begins with a physical exam and blood test, which may be followed by additional tests. Treatment options include chemotherapy and/or radiation treatments.

due in large part to Jamie being teased and occasionally bullied by her special education classmates who were behaviorally disruptive. Her teacher had failed to intervene at any level. Jamie was very concerned about being teased, and even the thought of having to interact with her peers was overwhelming. Jamie’s lack of confidence was a significant change from her previously assertive and confident nature. Given her mother’ desire to protect Jamie from any further pain, Jamie had been allowed to avoid negative classmate interactions by spending her days with her mother at work. Jamie also had to contend with a number of cognitive issues in addition to her social problems at school. Her mother noticed that Jamie appeared more distractible and forgetful than usual and was having problems focusing. Moreover, despite regaining the ability to speak, Jamie continued to experience language difficulties. Jamie’s language problems appeared to be exacerbated by the headache medicine she was taking, and she complained about this side effect to her neurologist. Unfortunately, little could be done because she was still having chronic headaches. These cognitive issues were a significant change from the girl who had been an honor-roll student prior to her medical issues. Jamie and her family were having great difficulty accepting and coping with the trauma of her medical issues and their subsequent impact on her day-to-day life. Jamie often felt frustrated and angry about what had happened to her. She was very self-conscious of her hair loss from the chemotherapy and her unresolved speech problems. Jamie had been prescribed an antidepressant, which she found somewhat helpful. However, she continued to feel sad and hopeless most of the time. She felt

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she had become a different person. She felt slower than usual, had trouble focusing, and lacked confidence in her daily decision making. Although Jamie was no longer interested in her friends or going out, when she was forced to go to fun activities, she was able to enjoy them. Jamie also had been having problems sleeping and often woke up tired. In addition, her appetite was affected, and she had lost 16 pounds in 2 weeks. Even with all of these issues, when questioned, Jamie adamantly denied having thoughts of self-harm. Then, just when she and her family thought things could not get worse, they lost their home in an unexpected tragedy. Luckily, they were able to rely on the support of friends who took them in as they searched for a new home. Jamie’s neurologist referred her for a neuropsychological evaluation of her cognitive and emotional functioning in light of her emotional issues and ongoing cognitive problems. When Jamie arrived for the evaluation, she had just finished chemotherapy treatment and had not required radiation therapy. She was soon found to be in remission for her leukemia. Despite the discontinuation of chemotherapy, Jamie continued to require a heavy regimen of medications for ongoing headaches, pain, and concerns about possible seizures.

Test Results In light of Jamie’s complicated medical history and lack of previous evaluations, a broad approach was taken in assessing all aspects of Jamie’s cognitive functioning. Although a more comprehensive evaluation of Jamie’s language abilities would normally have been conducted, only a brief screen of this area was conducted because of time restrictions. Jamie was also to complete a speech and language evaluation with her school to determine her eligibility for services. Individual tests administered included measures of intelligence, academic achievement, attention, executive functions, visual-motor integration and visual processing, fine motor functions, language abilities, learning and memory, and emotional functions. Jamie’s mother also completed several child behavior checklists rating her daughter’ emotional and behavioral functioning. Jamie initially presented as a shy and reserved girl who quickly became teary when asked about her medical condition. In fact, Jamie and her mother both cried easily each time they discussed Jamie’s medical issues and the subsequent impact on her functioning. However, over the course of the evaluation, Jamie’s affect brightened and she would even smile shyly during casual conversation. Her responses, however, tended to be brief and limited to a few words. She appeared to have difficulty finding the right word to say and on at least one occasion resorted to describing the word she was trying to say; however, she seemed to have no problems understanding task requirements. Jamie appeared to favor her left hand but was able to use her right hand slowly to produce legible printing. Although Jamie tired easily, with short breaks she was able to resume working each time with

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renewed energy. Jamie persevered on all tasks but worked at a slow pace. In general, she was compliant, cooperative, and self-motivated. Jamie’s evaluation results placed her overall intellectual ability in the borderline impaired to below average range (Table 12.1). However, she demonstrated a significant discrepancy between her average nonverbal and visual spatial abilities and her mildly impaired to borderline impaired verbal and language abilities, working memory, and speed of information processing. Taking into account Jamie’s medical history of a left-sided cerebral stroke along with the corresponding right-sided weakness and language problems, Jamie’s solidly average nonverbal and visual spatial abilities likely were more representative of her premorbid intellectual functioning. The impact of Jamie’s language problems as well as attention and executive functioning problems was observed in her variable performance across the individual subtests. On subtests with minimal language requirements, Jamie’s performance was average. However, on subtests with language demands, Jamie’s performance was weaker as indicated by her low average to borderline impaired range performance. Consistent with her concerns about mental slowing, Jamie also demonstrated slowed speed of information processing, particularly on tasks requiring complex fine motor output. This was also noted in her borderline impaired to impaired ability to visually sequence numbers rapidly. Related to her focusing and decision-making problems, Jamie demonstrated problems with attention and executive functioning (Table 12.2). On a measure of sustained attention, she was inattentive and made excessive omission errors (missed targets). In addition, she was slow but also variable in her rate of responding to targets. Executive functioning problems were noted in Jamie’s ability to manipulate information mentally, demonstrate mental flexibility, problem-solve Table 12.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal Comprehension Index Similarities Vocabulary Comprehension Perceptual Reasoning Index Block Design Picture Concepts Matrix Reasoning Working Memory Index Digit Span Letter-Number Sequencing Processing Speed Index Coding Symbol Search Full Scale IQ a Standard score, (scaled score), [T-score], {z-score}.

Scorea

Percentile

69 (6) (4) (4) 96 (11) (7) (10) 71 (9) (1) 68 (4) (6) 71

2nd 9th 2nd 2nd 39th 63rd 16th 50th 3rd 37th 0.1st 2nd 0.4th 9th 3rd

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Table 12.2 Other cognitive functions Tests Conners’ Continuous Performance Test – Second Edition Omissions Commissions Hit RT Hit RT standard error Delis-Kaplan Executive Function System Trail Making Test Number Sequencing Letter Sequencing Number-Letter Switching 20 Questions Total Questions The Tower of London Behavior Rating Inventory of Executive Function Inhibit Shift Emotional control Behavioral regulation index Initiate Working memory Plan/organize Organization of materials Monitor Metacognition index Global executive composite Grooved Pegboard Test Right hand Left hand Beery-Buktenica Developmental Test of Visual-Motor Integration Visual discrimination Motor coordination Boston Naming Test Wide Range Assessment of Memory and Learning – Second Edition Story memory Story memory delay Story memory recognition California Verbal Learning Test – Children’s Version List A Total Trials 1–5 List A short-delay free recall List A short-delay cued recall List A long-delay free recall List A long-delay cued recall Recognition hits Children’s Memory Scale Dot locations Learning Total score Long delay Rey-Osterrieth Complex Figure Test Delayed recall

Score

Percentile

[64.91] [44.67] [84.08] [69.23]

94th 33rd 99th 97th

(4) (1) (1)

2nd <1st <1st

(3) 78

1st 7th

[55] [79] [80] [75] [74] [87] [76] [65] [64] [79] [79]

75th 99th 98th 98th 99th 99th 99th 92nd 92nd 99th 99th

<1 68 91 75 82 48

<1st 2nd 27th 5th 12th <0.1st

(6) (5) (7)

9th 5th 16th

83 85 78 78 85 78

13th 16th 7th 7th 16th 7th

(4) (4) (5)

2nd 2nd 5th

93

32nd

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efficiently, and organize information, particularly as task demands increased in complexity or involved language. Accordingly, Jamie’s mother’s ratings of her daughter’s executive functioning suggested even greater problems in Jamie’s dayto-day functioning when faced with real-world demands. Consistent with Jamie’s history of left-sided stroke, problematic fine motor dexterity and language functions were noted. Although impaired fine motor dexterity was noted bimanually, Jamie’s performance was significantly worse and in the severely impaired range with her dominant, right hand. However, when Jamie was asked to trace figures rapidly, her speeded motor coordination was low-average, and when she was asked to copy simple to complex geometric designs, her visual motor integration was average. Although Jamie’s speeded visual discrimination was borderline impaired, a review of her performance suggested an adverse impact from inattentiveness. Although a more extensive evaluation of Jamie’s language functioning was deferred to her school, Jamie’s visual confrontation naming was impaired, which was consistent with her complaints of word-finding difficulties. This appeared to be impacted in part by processing speed, because Jamie often struggled to generate a response within the designated time limit. Memory functions were also noted to be problematic, but more with regard to Jamie’s encoding of novel verbal information. With regard to verbal memory skills, difficulty was noted in Jamie’s encoding of complex, organized stories as well as her encoding of wordlist items on a verbal learning through repetition task. As such, Jamie’s delayed recall for the stories and the wordlist items were low average to borderline impaired. Her delayed recognition for story details and wordlist items were also low average to borderline impaired, suggesting memory problems primarily related to problematic initial encoding of novel verbal information. Encoding problems were also noted in Jamie’s ability to learn a visual array over multiple trials. In contrast, on a separate task, Jamie’s delayed recall of a complex geometric design was average, suggesting adequate ability to retain nonverbal information, once encoded. Consistent with the reported and observed language problems since her stroke, problematic academic achievement was noted on language-based academic functions (Table 12.3). Although her basic math calculation skills were intact, Jamie’s basic spelling skills were borderline impaired to below average, and her reading decoding skills were mildly impaired. A review of Jamie’s premorbid functioning

Table 12.3 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition Academic skills Letter-word identification Calculation Spelling

Score

Percentile

70 65 91 77

2nd 1st 28th 6th

Grade equivalent 4.1 3.0 7.3 4.1

12 Family Matters: Psychosocial Factors on Neuropsychological Outcome Table 12.4 Other emotional functions Tests Children’s Depression Inventory Total score Negative mood Interpersonal problems Ineffectiveness Anhedonia Negative self-esteem Behavior Assessment System for Children – Second Edition Internalizing problems Anxiety Depression Somatization Withdrawal

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Score

Percentile

[55] [64] [54] [45] [60] [39]

70th 93rd 68th 32nd 84th 14th

[86] [74] [80] [84] [63]

99th 98th 99th 99th 90th

suggested that this change occurred secondary to her recent medical events because her prior academic function was not noted to be problematic. Finally, consistent with presenting emotional concerns, Jamie’s ratings of her mood functioning indicated significantly elevated negative mood and anhedonia (ability to enjoy life). Maternal ratings of Jamie’s emotional and behavioral functioning were remarkable for clinically significant, internalizing problems such as anxiety, depression, somatization, and, to a lesser degree, withdrawal (Table 12.4).

Formulation and Recommendations Together, the results obtained suggested a general pattern of lateralized findings that would be consistent with Jamie’s history of a left-sided stroke (e.g., poorer right-handed fine motor speed and coordination compared with her left hand, language and verbal problems, relatively poorer verbal memory than nonverbal memory, weakness in language-based academic functions). Generally speaking, certain cognitive functions are thought to be supported by one side of the brain or the other. For example, in most individuals, the left side of the brain largely supports language functions while the right side of the brain largely supports visual-spatial abilities. Other functions are lateralized as well, such as motor functions: the right side of the brain controls left-sided motor movements, and vice-versa. On the basis of these generalizations, when an individual experiences a stroke or some other type of damage to the brain, the deficits that may occur are often reasonably predictable based on which side the insult has occurred. Even more specificity in predicting deficits often can occur by considering the exact area where the damage has occurred. In addition, consistent with Jamie’s diagnosis of leukemia that required chemotherapy treatment, Jamie demonstrated slowed processing speed and problems with attention and executive functioning. Research suggests that cognitive problems

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associated with leukemia treatments such as chemotherapy are most commonly observed in attention skills, although problematic processing speed, working memory, visual-motor skills, and arithmetic skills have also been noted. Further, the impact of leukemia treatments on cognitive functioning varies with a number of factors, such as the type of treatment received, age at the time of treatment, and gender. Finally, also concerning was the likely impact of emotional factors such as anxiety and depression and their contributions to some of Jamie’s neurocognitive difficulties. Despite Jamie’s very significant cognitive problems that required remediation and accommodations in school, the greatest area of concern was the impact of Jamie’s ability to cope with her new medical issues and her depressed mood. Mood issues were evident in parent ratings of Jamie’s emotional functioning. Symptoms of concern included Jamie’s depressed mood, decreased enjoyment of everyday activities, social withdrawal, sleep dysregulation, fatigue, poor concentration, and psychomotor slowness. After reviewing Jamie’s medical records, it appeared that her depressed mood had been an issue of concern since she was first diagnosed with leukemia and that it was continuing to have significant impact on her general well being. Therefore, the first recommendation made was to address Jamie’s depressed mood. Although she had been prescribed and was taking an antidepressant, Jamie still continued to experience symptoms of depression. Individual therapy was recommended to help Jamie find power in being able to control her emotions and cope better. Jamie seemed hesitant when therapy was first suggested to her, so time was spent introducing her to cognitive behavioral therapy techniques in order for her to better understand the basic concept of therapy. She was taught some basic principles to work on changing her thoughts to improve her emotions, and she seemed willing to try therapy. Jamie’s treatment plan also had to take into account her mother’s understandably strong desire to protect her daughter. Jamie’s mother had spent the past year fighting to protect Jamie from any pain that could be avoided, and she was reluctant to have her daughter subjected to yet another doctor’s care. Unfortunately, although Jamie’s mother clearly cared about Jamie, in playing ‘‘momma bear’’ her attempts to protect her daughter had allowed Jamie to perpetuate some maladaptive coping strategies, such as her avoidance of school. Trying to convince Jamie’s mother that she could not always be there for Jamie was unproductive – she had spent the past year heroically being there for Jamie. Instead, the focus had to be shifted to Jamie’s needs and what was in her immediate and long-term best interests. Once the emphasis was shifted back to helping Jamie cope and subsequently live better, progress could be made toward considering therapy. Thus, after a discussion of Jamie’s emotional needs, a compromise was made to pursue family therapy first to address everyone’s adjustment and coping with Jamie’s medical issues. It was hoped that this would later develop into an opportunity for Jamie to learn better coping strategies to manage her mood and her anxiety about school independently. In considering referrals, emphasis was placed on finding a therapist with whom Jamie would feel comfortable.

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Jamie still had to contend with a number of cognitive issues. Given her medical history and neuropsychological problems, an Individualized Education Program (IEP) under the Other Health Impairment classification was felt to be appropriate. With the discrepancy between her average nonverbal abilities and her problematic language abilities, her resource room placement also appeared to be appropriate. However, given the problems noted during the evaluation, both speech and language and occupational therapies were recommended in Jamie’s IEP. Recommendations were also made to provide accommodations for Jamie’s difficulties, including the provision of teacher/peer notes, taped lectures, training in the use of a keyboard for writing assignments, time-based homework assignments, and allowance for extra time on tests. Standard attention and memory recommendations were also included to help Jamie function more independently at school.

Update Jamie continued to have regular follow-up visits with her oncologist and other physicians to monitor her ongoing recovery and remission from leukemia. By her next neurology visit, Jamie’s family had found a new home, and Jamie had been enrolled in a new school. Her new classroom was more academically challenging and her classmates were friendlier. In addition, since Jamie had experienced some mild improvements with her headaches, her neurologist had been able to lower her medication dosage, which seemed to improve some of Jamie’s language problems. Jamie’s mother had also arranged for her to receive private speech and language therapy over the summer to ensure that Jamie would not have a gap in her therapy services. Despite these positive changes, Jamie’s emotional issues still lingered. Although a therapist had been found for Jamie, she and her family were awaiting the resolution of insurance issues. Nevertheless, Jamie and her mother continued to find ways to cope with the trauma of the past year. Although they still cried easily whenever discussing the events of the past year, mild improvements in Jamie’s mood were noted in what seemed to be a slight glimmer of hope in Jamie’s eyes when she relayed some of the new, positive changes in her life. Therefore, despite Jamie’s slow and probable partial recovery from her cognitive problems, her emotional recovery looked hopeful.

Additional Resources Key Concepts and Terms Cognitive Behavioral Therapy A type of therapy based on the idea that an individual’s own thoughts lead to maladaptive feelings and behaviors, rather than

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external things, like other people, situations, or events. Because of this, if individuals can modify and manage their thoughts, they can feel better even if the situation does not change. Depression A mental disorder characterized by the following symptoms: depressed or irritable mood, markedly diminished interest or pleasure in everyday activities, significant weight loss or gain, increased or decreased sleep, psychomotor agitation or retardation, fatigue, feelings of worthlessness or excessive guilt, poor concentration, and recurrent thoughts of death. Depression is diagnosed when five or more of these symptoms are present for 2 or more weeks. Lateralized Located on the right or left side of the brain. Seizure Temporary abnormal electric discharge in the brain. Clinical manifestations include sudden disruption of specific cognitive functions while this discharge is occuring. Stroke A neurological syndrome characterized by the acute onset of neurological symptoms following disturbance in the brain’s blood supply leading to cell death. The two main types of strokes are ischemic stroke, which involves blockage of a blood vessel, and hemorrhagic, which involves bleeding.

References Resources for Clinicians American Psychiatric Association, & Task Force on DSM-IV. (1994). Diagnostic and statistical manual of mental disorders: DSM-IV (4th ed.). Washington, DC: American Psychiatric Association. Baron, I. S., Fennell, E. B., & Voeller, K. K. S. (1995). Pediatric neuropsychology in the medical setting. New York: Oxford University Press. Bluebond-Langner, M. (1996). In the shadow of illness: Parents and siblings of the chronically ill child. Princeton, N. J: Princeton University Press. Butler, R. W., & Mulhern, R. K. (2005). Neurocognitive interventions for children and adolescents surviving cancer. Journal of Pediatric Psychology, 30(1), 65–78. Davis, H., Marlow, N., & British Psychological Society. (1993). Counselling parents of children with chronic illness or disability. Leicester, Baltimore, MD, U.S.A: British Psychological Society; Distributed exclusively in North America by P.H. Brookes Pub. Moore, B. D., III. (2005). Neurocognitive outcomes in survivors of childhood cancer. Journal of Pediatric Psychology, 30(1), 51–63. National Cancer Institute. Leukemia home page. Retrieved November 27, 2007, from http://www. cancer.gov/cancertopics/types/leukemia (provides information on treatment, clinical trials, research, and statistics related to leukemia) Waber, D. P., & Mullenix, P. J. (2000). Acute lymphoblastic leukemia. In Yeates, K. O. Ris, M. D. & Taylor, H. G. (Eds.), Pediatric neuropsychology: Research, theory, and practice (pp. 300–319). New York: Guilford Press.

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Resources for Families The Leukemia & Lymphoma Society, http://www.leukemia.org. Provides information on leukemia and its treatment. The ‘‘Disease Information’’ section can be particularly informative for both clinicians and families. Families may also wish to view the ‘‘Patient Services’’ section to learn more about available support. Relevant publications available at the Web site include ‘‘Acute lymphocytic leukemia’’ and ‘‘Coping with childhood leukemia and lymphoma.’’ The PACER Center, http://www.pacer.org. In St. Paul, Minnesota, a regional, Midwest center for providing information, advocacy, and training for parents and professionals, about individual’s rights, particularly in relation to education. Parent to Parent-USA, http://www.p2pusa.org. A national non-profit organization whose programs provide emotional and informational support to families of children who have special needs, most notably by matching parents seeking support with an experienced, trained ‘‘Supporting Parent.’’ NICHCY State Resources List, http://www.nichcy.org. Includes names, addresses, phone numbers, and e-mail addresses for developmental disabilities, special education, vocational rehabilitation, and parent advocacy programs for every state.

Chapter 13

Dog Attack: Physical Trauma with Associated Brain Injury Jennifer Niskala Apps

Anna was a spunky and energetic young girl who enjoyed being ‘‘mommy’s little helper.’’ She had been to her mother’s friend’s house many times and was familiar with her dogs. When the women stepped outside during one visit, 8-year-old Anna thought the two dogs might like to play together, so she took down the gate that separated them from each other. She remembers reaching for the rope toy, thinking she would play with them. She remembers the sounds as first the male full-grown dog attacked her and then the adult female joined in, biting her severely, mauling her head and neck. She remembers being dragged across the room and calling out for help. Her mother returned to the house only a few moments later. She immediately noticed the gate, and her gaze turned to the front room and the sight of her only child lying on the floor horribly injured. Running immediately to her, she noticed Anna’s breathing was shallow. Anna could not talk and did not appear fully conscious. While her friend called 911, Anna’s mother never left her side. An ambulance arrived quickly to transport Anna to the emergency department. During 9 hours of surgical intervention to stabilize her injuries, Anna experienced pulmonary distress and required resuscitation. The attack had focused on the right side of her face and neck. Her injuries included nearly complete occlusion of the right internal carotid artery and focal injuries to both vertebral arteries. Her right ear was severed, the facial nerves on the right side of her face were injured, and her right eye was dislodged from its socket. Facial bones required reconstruction, and plates were placed in the right eye socket and across the bridge of her nose. Anna also experienced two cerebral infarcts. The first was during the attack itself. Emergency computerized tomography (CT) and angiogram revealed an embolic stroke at the distribution of the middle cerebral artery on the right. The second stroke came immediately following the completion of her surgery in the right mid-parietal lobe. Within an hour of moving to postoperative recovery, Anna called for her mother and reported remembering everything. She had a tracheostomy and a gastrointestinal tube. Although she experienced some disorientation from constant use of pain

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Box 13.1 Anna’s medical complications l l l l l l l l l l

Pulmonary distress and resuscitation Occlusion of the right internal carotid artery Focal injuries to both vertebral arteries Embolism – right middle cerebral artery Embolism – right mid-parietal lobe Tracheostomy Gastrointestinal tube and feedings Constant morphine for pain Permanent occlusion of the superior left vertebral artery Encephalomalacia – right parietal-occipital

medication, Anna remained relatively cognizant and communicated through hand signs. She initially experienced full paralysis on the left side of her body. Anna remained in the hospital for 7 weeks. During her long recovery, her parents described frequent and extreme emotional highs and lows. Anna would become intensely angry and frustrated, often crying. Other times she would become very exuberant, and the smallest strides forward seemed to be major breakthroughs. Angiogram during her recovery indicated Anna continued to experience the blockage of one carotid artery. However, it was described to the family that Anna had adequate connections on the top of her brain to support full cranial circulation. Follow-up angiographies of the head and neck at 3 months and again at 1 year postinjury noted continued occlusion of the superior aspect of the left vertebral artery and resolution of the hemorrhage in the right parietal-occipital region, with some areas of resulting encephalomalacia. Four months after the injury, Anna underwent major surgery once again to reconstruct her jaw, tear duct, the bridge of her nose, eyelid, and ear canal. Three additional surgeries were performed subsequently, with more planned once Anna finished growing into adulthood. With significant physical and occupational therapy, Anna regained major muscle movements on the left side of her body. However, she continued to experience some left-sided hemiparesis and possible neglect. Although she was able to retain her eye, Anna experienced some long-term functional damage to the eye, including dysconjugate gaze. She also had some reduced hearing in her right ear. Anna had been attending second grade at the time of the injury. She had a previous history of average grades and, despite never liking to read, average achievement. She completed second grade by working with tutors, and then returned to school at the beginning of third grade. Initially, the family and a large support network of teachers believed everything was going well. However, in fourth grade, Anna began to fail in mathematics. Homework took longer and longer to complete. Fifth grade began at a new and larger school. Anna began having problems with attention, and she continued to struggle in math. When she was required to enroll in summer school in order to progress to the sixth grade, her parents became more concerned.

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Anna also was demonstrating problems with coordinated motor movements. Physical therapy had been completed some time ago; however, like most adolescents, Anna began having trouble remaining compliant with her home exercises. Her parents described her as ‘‘sassy’’ at times, with some ‘‘normal’’ adolescent defiance and occasional giddy behavior. They portrayed her as an affectionate girl, invested in her family, friends, teachers, and church. At the time of evaluation, they stated that Anna had continued concerns regarding her vision and her difficulty using her left hand. However, they clearly stated that they and Anna had no problems with her scars, her physical appearance, or remorse about the injury. It had been 3 years since the attack, and the family was interested in seeking neuropsychological evaluation only to determine whether there were any ‘‘permanent’’ cognitive sequelae.

Test Results Given Anna’s complex medical history and lack of any recent neurocognitive evaluations, a broad approach was taken in assessment, with a focus on visual and motor tasks. Anna’s parents and teacher completed several behavior checklists to assess her level of possible emotional or behavioral dysfunction. Individual tests administered included measures of intelligence, academic achievement, learning and memory, visual-motor integration and visual processing, executive functions, motor and sensory abilities, and emotional functions. Anna presented as a tall, thin young lady with obvious facial deformities on the right side of her face involving her right eye. She was cautiously friendly upon meeting the examiner, although rapport was established quickly. Anna immediately began chatting about her summer and carried easy, appropriate, and pleasant social conversation throughout the evaluation. Her sustained attention was adequate, although she was mildly distractible at times. She demonstrated left-sided hemiparesis that did not affect gait but was noted to affect significantly the use of her left hand. In reviewing Anna’s evaluation results (Table 13.1), intellectual abilities were in the overall average range, with her verbal abilities in the average range and her nonverbal abilities average to above average. Limited variation was seen among individual subtests. Anna demonstrated relative difficulties with visual-motor tasks requiring rapid processing speed, with her performances falling into the low average range. Anna performed consistently within the average range on all Table 13.1. Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal Comprehension Index Perceptual Reasoning Index Working Memory Index Processing Speed Index Full Scale IQ a Standard score, (scaled score), [T-score], {z-score}.

Scorea

Percentile

102 112 91 88 101

55th 79th 27th 21st 53rd

124 Table 13.2. Academic achievement Woodcock Johnson Tests of Achievement – Third Edition, Form A Broad reading Letter–word Reading fluency Passage comprehension Broad math Calculation Math fluency Applied problems Broad written language Spelling Writing fluency Writing samples

J.N. Apps

Score

Percentile

99 107 96 98 104 111 86 104 100 108 91 103

48th 68th 39th 45th 60th 77th 17th 60th 50th 70th 28th 58th

Grade Equivalent 6.0 7.1 5.4 5.8 6.6 8.0 4.5 6.8 6.1 7.7 4.9 7.0

academic tasks, with the exception of speeded tasks (Table 13.2). On these tasks, which required rapid visual-motor speed, she performed in the low average range. Further, among executive skills, Anna demonstrated intact performances in all areas except for visual fluency and visual problem-solving, which were low average. No significant attention difficulties were noted on formal examination (Table 13.3). Immediate and delayed memory abilities were in the average to above average range for both visual and verbal information. Anna demonstrated mild difficulties learning unstructured visual information, although her long-term recall of the information was intact. Visual-motor integration, visual recognition, and overall motor coordination were all within the average range. However, not surprisingly, significant deficits were found in motor and sensory functions in her left hand. Although Anna’s deficits in motor and sensory functioning were significant, no evidence of visual neglect was noted during evaluation. Visual field testing indicated that Anna had difficulty seeing items placed in her right peripheral vision, which appeared directly related to her continued physical complications with her right eye. Anna was able to attend and respond adequately to stimuli in all other visual fields. On tasks of visual attention, no neglect was noted for the right side of the stimuli, although Anna consistently demonstrated poorer performances on tasks requiring visual-spatial orientation and organization.

Formulation and Recommendations In formulating an explanation for these findings, Anna’s areas of relative weakness, including her continued deficits in sensory and motor functioning in the left side of her body, were thought to be consistent with her right parietal stroke and resulting encephalomalacia. Anna’s mild relative difficulties on tasks requiring rapid visualmotor speed were thought to indicate continued mild difficulties processing and responding to visual information.

13 Dog Attack: Physical Trauma with Associated Brain Injury Table 13.3. Other cognitive functions Tests

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Raw score

Children’s Memory Scale Visual immediate Visual delayed Verbal immediate Verbal delayed General memory Learning Delayed recognition

Score

Percentile

94 106 112 106 108 85 118

34th 66th 79th 66th 70th 16th 88th

Rey-Osterrieth Complex Figure Test Copy Immediate recall Delayed recall

<10th <10th <10th

Beery-Buktenica Developmental Test of Visual-Motor Integration Visual perception Motor coordination Grooved Pegboard Test Right hand Left hand

96

39th

100 99

50th 47th

102 <50

55th <1st

Finger Gnosis Right hand (errors) Left hand (errors)

1/20 11/20

76 <50

Graphesthesia (Numbers) Right hand (errors) Left hand (errors)

3/20 16/20

98 <50

Delis-Kaplan Executive Function System Verbal Fluency Test Letter fluency Category fluency Category switching Total Accuracy Design Fluency Test Condition 1 total Condition 2 total Condition 3 total Twenty Questions Test Initial abstraction Total questions Achievement Tower Test Achievement Move accuracy ratio

(11) (12) (12) (13) (8) (9) (8) (14) (9) (10) (8) (6)

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However, an additional area of concern was raised in this evaluation that was not part of the presenting complaint. Anna’s history indicated past symptoms consistent with posttraumatic stress disorder. Although these symptoms appeared to have resolved, upon emotional evaluation, Anna continued to demonstrate high levels of inappropriate guilt, depressive symptoms, and significant anger. Anna’s primary coping methods included denial and an attempt to internalize these feelings. Collected behavioral reports indicated that Anna had consistent difficulties attending in the classroom and often preferred to be alone. At home, Anna reportedly was complaining often about somatic concerns and also often withdrew from others. Although none of Anna’s caretakers felt she was demonstrating major emotional or behavioral problems, Anna’s withdrawal and somatic concerns were thought to be related to overwhelming emotional distress. It appeared through the course of working with this young woman that she had internalized significant responsibility for demonstrating that she was ‘‘okay’’ to all the adults in her life. This had left her unable to express her anger and guilt appropriately and resulted in her feeling isolated. Anna was diagnosed with encephalopathy and late effects of a brain injury, as well as an adjustment disorder. Anna likely experienced symptoms consistent with a posttraumatic reaction immediately following the event, including nightmares and emotional dysregulation. However, in the past 3 years, while she continued to demonstrate chronic emotional symptoms related to not only the trauma itself but the constant stressor of physical sequelae from her injuries, the severity of these symptoms did not meet criteria for a formal, chronic posttraumatic stress disorder. Anna was no longer reexperiencing the trauma itself or physiological reactions to reminders of the trauma, but she was continuing to demonstrate emotional adjustment issues. Recommendations initially focused on the family acknowledging Anna’s need for emotional interventions. Speaking with the family about the recommendation of therapy was delicate, because it directly challenged many of the family’s defenses. They were praised for the remarkable job they had done coping with such a tragedy. The clinician empathized with the extensive journey the family had been through during recovery. Then, a developmental perspective was introduced to the parents to help them understand that Anna was continuing to reexperience the meaning of her attack at every stage of her development. Although the adults in her life also may experience some revisiting of the loss and grief of the attack over the years, Anna must relive the impact of this event daily and in all aspects of her growth and development. Such a complicated grieving process leaves even the best of children emotionally exhausted and overwhelmed. Anna needed the opportunity to work with someone not involved in her history since the attack, someone with whom she could openly explore all aspects of her coping. A further suggestion was made for the family to pursue at least time-limited family therapy to specifically learn additional techniques for coping with emotions as a family. Further recommendations with Anna and her family focused on the physical and cognitive sequelae of her injury. Because of Anna’s increasing avoidance of using

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Box 13.2 Further modifications to Anna’s classroom curricula l l l

l

Avoid timed tests or assignments if a true representation of skills is desired Make tasks shorter in length Give assignments with similar types of questions presented together to reduce visual recognition demands Provide external structure on larger projects, breaking larger assignments into smaller, more manageable, components

her left hand, it was recommended that she return to physical and occupational therapy. This would provide a structure for her to continue to rehabilitate and learn how to better incorporate ongoing exercises into her daily life. Additional recommendations focused on Anna’s lower relative performances on tasks of a timed, visual nature. These included allowing greater time to complete visually based assignments, particularly in mathematics, and tasks requiring written output. Anna’s Individualized Education Program (IEP) through the school provided the structure for such accommodations. Reevaluation of Anna’s visual fields and recognition skills was recommended following surgical correction of her right eye. Her current visual status was impacting her vision, which was anticipated to improve with surgery but would require continued monitoring. Further, reevaluation of Anna’s cognitive development was recommended in approximately 3 years to continue monitoring her motor and sensory skills, visual processing abilities, and development of executive skills. This family continued to be invested in their child being developmentally sound, cognitively, emotionally, and socially. It remained unclear if they would return for reevaluation or follow through with the emotional recommendations suggested. However, an important aspect of this evaluation remained the attempt to help the family understand that Anna’s emotional development may require equal attention to her cognitive, motor, and sensory growth.

Additional Resources Key Concepts and Terms Adjustment Disorder A mental disorder that involves the development of emotional and/or behavioral symptoms in response to an identified stressor. Qualifiers are available to distinguish acute from chronic reactions, and symptoms are further described through subtypes of symptoms. Embolic A blockage in a blood vessel. Encephalomalacia An area within the brain of dead or destroyed cells. Hemiparesis Lack of motor coordination or paralysis on one side of the body.

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Neglect The tendency to ignore one side of the body or information presented to one side of the body. Posttraumatic Stress Disorder An anxiety disorder occurring following exposure to a traumatic event in which a person experienced intense fear or threat to life, followed by heightened physiological arousal, persistent reexperiencing of the event, and avoidance of things associated with the event.

References Resources for Clinicians American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders: DSM-IV (Text Revision, 4th ed.). Washington, DC: American Psychiatric Association. Cohen, J. A., Mannarino, A. P., & Deblinger, E. (2006). Treating trauma and traumatic grief in children and adolescents. New York: Guilford Press. James, B. (1989). Treating traumatized children: New insights and creative interventions. Lexington, MA: Lexington Books. Saxe, G. N., Ellis, B. H., & Kaplow, J. B. (2007). Collaborative treatment of traumatized children and teens: The trauma systems therapy approach. New York: Guilford Press.

Resources for Families Lowenstein, L. (1999). Creative interventions for troubled children & youth. Toronto: Champion Press. Monahon, C. (1997). Children and trauma: A guide for parents and professionals (1st ed.). San Francisco: Jossey-Bass.

Chapter 14

Back to Life: Anoxic Brain Damage in a Near Drowning John M. Oestreicher

Near drowning is a significant cause of disability in children, with one-third sustaining moderate to severe neurological impairment. The risk for near drowning is greatest among toddlers, preschoolers, and adolescent males. Younger children are more likely to drown in swimming pools (Verive, Heidemann, & Fiore, 2007). Charlie, a 7-year-old boy, was found at the bottom of a swimming pool in Singapore, where he was living with his parents, 9-year-old sister, twin brother, and 10-month-old brother. It was undetermined how long he was submerged. He was resuscitated and transferred to a local hospital, where magnetic resonance imaging (MRI) was completed, revealing no structural damage. Given the traumatic stress this event placed on Charlie’s family, they elected to return to the United States for his rehabilitative services. Charlie was referred for neuropsychological assessment at the direction of his physiatrist, 2 months after the near-drowning in order to more carefully examine his current pattern of neurocognitive strengths and weaknesses following anoxic brain injury. Charlie’s parents noted several changes in his behavior and cognitive functioning. They described concerns regarding marked changes in his processingspeed. He was much slower in his overall information processing. He also had been experiencing disturbed motor functioning, including disturbed gait, balance and coordination issues, and a tendency to fatigue quickly. He was receiving physical and occupational therapy through a local rehabilitative program. In addition, Charlie was involved in speech and language services to address ongoing concerns with word finding difficulties. The speech and language pathologist raised additional concerns about potential left-sided neglect, because Charlie often failed to attend to information on the left side of a worksheet. Charlie’s parents also described some changes in his personality, both increased emotional lability and general apathy. He previously had been rather competitive with his twin brother, but his parents noted that Charlie seemed resolved to give up on tasks that he found difficult. Associated difficulties for Charlie were relatively few. He experienced no headaches or sensory disturbances and never experienced a seizure. He had no notable

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sleep or appetite disturbances, and his additional medical history was generally unremarkable. His family medical history was significant for depression on the maternal side, because Charlie’s great-grandfather was hospitalized for this disorder. Charlie’s mother reported no difficulties with pregnancy. Charlie and his identical twin were born at 36-weeks’ gestation. Charlie was twin B and presented as breech but was delivered without further difficulty at a weight of 4 pounds, 11 ounces. Both he and his brother were mildly jaundiced and treated with biliwraps. They experienced no subsequent postnatal difficulties. Charlie was able to achieve developmental milestones in an age-appropriate manner. Charlie was homeschooled by his mother and working at a 1st grade level. Prior to his injury, he read simple sight words and performed simple math such as addition and subtraction. Math continued to be an area of strength for Charlie but reading seemed to be more difficult, and he had demonstrated greater regression in this area. Charlie’s home environment was described as generally harmonious, and the family was planning to return to Singapore after Charlie completed his rehabilitation.

Test Results In cases of near-drowning, computerized tomography (CT) studies suggest that survivors are not a homogeneous group in terms of neurocognitive impairments, which may be very specific to quite generalized. Therefore, the assessment was designed to be comprehensive, taking into account general abilities and assessing the specific areas of language comprehension and expression, simple motor and psychomotor functioning, visual-spatial reasoning, visual analysis and construction, verbal and nonverbal learning and memory, and executive functioning, including specific tasks of processing speed. Testing was completed over 2 days to avoid fatigue, and Charlie was pleasant and cooperative. He readily adjusted to the test situation, although he exhibited mild anxiety and requested that his father remain in the exam room. This did not seem to interfere with his ability to adjust to the test situation, because he formed a good working relationship with the examiner. He demonstrated appropriate affective range and stability over time. Although Charlie tended to be relatively quiet, he seemed to encounter no difficulties with language fluency. Mild articulation problems and language sequencing impairments were noted at times. Charlie exhibited no evidence of whole body overactivity, but he was fidgety during some tasks. His attention was sometimes variable during the exam and seemed to deteriorate slightly over time, interfering with his ability to carefully monitor his performance during some tasks. Charlie typically exhibited appropriate reflective behavior. Given his good level of effort, the test results appeared to reflect a fairly reliable and valid estimate of his neurocognitive functioning. On the basis of a lateral preference examination, Charlie presented as predominantly right-handed, exclusively right-footed, and right-eyed. His motor speed rated as average bilaterally. Mild bilateral slowing was evident on a task of speeded

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eye‐hand motor coordination, with these weaknesses more pronounced with his nondominant (left) hand. Although Charlie completed a task of visual imperception and suppression without error, he encountered mild to moderate weaknesses bilaterally on a task of finger gnosis, which was more pronounced with his nondominant (left) hand. Charlie completed one measure of psychometric intelligence (WISC-III) yielding a verbal IQ score within the low average range while performance and full-scale IQ scores fell within the borderline range of intellectual functioning (Table 14.1). Verbal reasoning and problem solving were primarily within the low average range, and mild to moderate impairment was evident on tasks of working memory. On the performance section of the test, Charlie’s visual spatial reasoning was variable (Table 14.2). He scored within the average range on a measure of visual scanning, the mildly to moderately impaired range on tasks of visual analysis and construction, and the moderately impaired range on a measure of visual sequencing. His processing speed rated as mildly impaired. Charlie performed inconsistently on tasks assessing verbal and psycholinguistic abilities (Table 14.3). On a measure designed to assess the symbolic and communication aspects of language, Charlie encountered three errors in reading.

Table 14.1. Intellectual Wechsler Intelligence Scale for Children – Third Edition Verbal Comprehension Index Perceptual Organization Index Freedom from Distractibility Index Processing Speed Index Full Scale IQ Information Similarities Arithmetic Vocabulary Comprehension Digit Span Picture Completion Coding Picture Arrangement Block Design Object Assembly Symbol Search a Standard score, (scaled score), [T-score], {z-score}. Table 14.2. Academic achievement Wide Range Achievement Test – Third Revision Reading Spelling Arithmetic

Score 83 81 69

Scorea 88 77 69 80 77 (8) (7) (3) (8) (8) (6) (9) (6) (4) (5) (6) (6)

Percentile 21st 6th 2nd 9th 6th

Percentile 13th 10th 2nd

Grade equivalent Kindergarten Kindergarten Kindergarten

132 Table 14.3. Other cognitive functions Tests Name Writing Right hand Left hand

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Raw score

Score

Percentile

94 75

34th 5th

Grooved Pegboard Test Right hand Left hand

78 63

7th 3rd

Finger Tapping Test Right hand Left hand

103 112

58th 79th

Age equivalent

Visual Suppression Test Right hand (errors) Left hand (errors)

0 0

106 106

66th 66th

Finger Agnosia Right hand (errors) Left hand (errors)

5 7

77 66

6th 1st

1 3 2 6

70 54 22 73 100

2nd <1st <1st 4th 50th

7–2

72

3rd

4–9

(5) 66 (6)

1st

108 76

70th 5th

92 95 105

30th 37th 63rd

(7) (6) (6) 99

42nd

Reitan Indiana Aphasia Screening Test Dysgraphia (errors) Dyslexia (errors) Dyscalculia (errors) Total (errors) Peabody Picture Vocabulary Test – Third Edition, A Expressive Vocabulary Test NEPSY Phonological processing Verbal fluency Arrows Multilingual Aphasia Examination Token test Progressive figures (time) Buschke Selective Reminding Test Recall Long-term store Consistent long term retrieval Wide Range Assessment of Memory and Learning – Second Edition Design memory Finger windows Sentence memory Beery-Buktenica Developmental Test of Visual-Motor Integration Wechsler Intelligence Scale for Children – Third Edition Digit span

134

6–6

(6) (continued)

14 Back to Life: Anoxic Brain Damage in a Near Drowning Table 14.3. (continued) Tests Coding Symbol search Test of Variables of Attention Omission errors Commission errors Response time Variability

Raw score

133

Score (6) (6)

Percentile

46 61 82 65

<1st <1st 12th 1st

Age equivalent

He also committed a simple spelling error when attempting to copy a word, and his calculation skills were inefficient. He performed within the mildly to moderately impaired range on measures of phonological processing as well as language fluency, requiring him to generate words in response to sound cues. Charlie’s expressive language skills rated mildly to moderately impaired on a measure of expressive vocabulary. His receptive language skills and language comprehension appeared strong. Charlie performed within the low average range on language-based academic tasks. His math skills fell within the mildly cognitively impaired range. Charlie performed inconsistently on tasks of verbal and nonverbal learning and memory. His performance was rated as average, when he was asked to learn a list of phonemically and semantically unrelated words in the context of a selective reminding instructional procedure. He readily transferred information from shortterm to long-term memory and performed solidly within the average range when attempting to retrieve learned information from long-term memory. Charlie performed within the low average range when attempting to recall and reproduce design information in its appropriate spatial location. His immediate memory skills for both verbal and nonverbal information rated as mildly impaired because Charlie found it somewhat difficult to repeat spoken sentences of progressively increasing length and to reproduce a series of visual-spatial patterns of progressively increasing length. Charlie’s reproductions of several simple geometric forms were generally unremarkable in visual-spatial characteristics. He performed solidly within the average range when attempting to reproduce a series of simple and progressively more complex figural stimuli. However, he encountered mild difficulties when he attempted to render judgments regarding the angular orientation of arrows directed toward a target. Mental flexibility and simple problem-solving skills rated as mildly impaired because Charlie encountered slowing when he attempted to negotiate a visualspatial pattern while utilizing size and form cues alternately for orientation and direction. Attention-based functioning rated as at least mildly impaired; that is, Charlie encountered difficulties on tasks of working memory, including measures of short-term recall of both digit strings and visual-spatial patterns. Processing speed weaknesses were evident on tasks involving quickly and accurately matching symbolic stimuli and visual search and attention. Charlie’s performance on a

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measure of sustained attention and concentration revealed his difficulties in monitoring his efforts over time because he committed a high number of inattentive and impulsive errors. His processing speed rated as low average but tended to be highly variable across the task. Charlie’s parents were asked to complete ratings of his pre and postdrowning behavior. Prior to the event, Charlie was described as exhibiting very mild difficulties with sustained attention, restlessness, and impulsiveness. He showed mild oppositional tendencies but not to the extent that would warrant diagnosis. He reportedly demonstrated appropriate social interaction with both peers and adults. He frequently had nocturnal enuresis. Following his injury, Charlie was described as exhibiting pronounced difficulties with sustained attention. He frequently encountered difficulties with organization, failed to follow through on tasks, and was forgetful and distractible. He showed increased levels of restless or fidgety behavior along with prominent impulsive tendencies and was prone to complain of physical symptoms. But he had not demonstrated any increase in oppositional behaviors. His interpersonal skills had not changed dramatically nor had his frequency of nocturnal enuresis.

Formulation and Recommendations Examination of Charlie’s performances on the battery of tests revealed a pattern of generalized weaknesses with lateralization of deficits on the left side of the body and with tasks of visual-spatial functioning. In addition, he demonstrated mild to moderate impairments in expressive language, speeded fine motor coordination, processing speed, and other aspects of attention/concentration skill. He also demonstrated a number of skills that were generally intact, including receptive language, simple motor functioning, and some components of verbal learning and memory, particularly when information was repeated for him. Charlie’s verbal reasoning and problem solving, while being low average, could be viewed as a relative strength. The noted pattern of functioning appeared to be due to his near-drowning episode, because the onset post-dated the traumatic event. Charlie would benefit from continued supportive services involving speech and language and occupational therapies to address his expressive language and fine motor weaknesses. He likely would continue to encounter word-finding difficulties, at least over the next several months as he continued his recovery. Additionally, he would need to be provided with appropriate wait times so that he may express himself while avoiding the frustration that may occur due to others’ expectations for rapid responding. Charlie could also engage in tasks or games at home that would allow him to practice fine motor skills. Games such as Pick-Up Sticks or various sorting tasks may be helpful in improving his dexterity. Careful observation of the development of his writing skills would be warranted because this may prove a weakness for Charlie in the future. Should this be the case, accommodations could

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be provided such as early instruction in keyboarding, because typing may better match his thinking speed than printing or cursive handwriting. Voice-activated software might be considered if typing is overly difficult. Charlie presented with generalized weaknesses in his overall academic functioning. Prior to his injury, he was beginning to demonstrate early reading skills. At the time of the assessment, it appeared that Charlie was continuing to develop limited sight vocabulary but was struggling with phonological decoding. It was recommended he continue with a phonics-based instructional approach while monitoring his reading development to determine whether he may be experiencing any difficulties in blending word sounds. Should this be the case, using an analytic phonics-based program that would teach him to recognize letter cluster sounds would be beneficial in reducing blending difficulties and creating a fluent reading style. Utilizing concrete approaches when teaching math skills might aid Charlie to develop basic math concepts. Tangible items, such as an abacus, could help him to understand basic addition and subtraction. Similar strategies might be incorporated when teaching multiplication and division. It would be helpful to monitor his math functioning over time because this could continue to be an area of weakness for Charlie. Should this be the case, it would be helpful to teach math in a straightforward step-by-step manner, such as using recipe cards on which each step is laid out in the appropriate sequence. His mother should continue instructing him in a home-school education program for the remainder of the school year because transitioning to a formal education program might be difficult, given his tendency to fatigue easily. Charlie was struggling with generalized learning and memory weaknesses. However, he was capable of learning when information was repeated for him. It would be important to keep this in mind when presenting new material to him. Using repetition and attempting to relate any new information to that which he had learned previously might allow him to more readily consolidate the information. Attempting to utilize mnemonic strategies, which might include visualization or chunking related material, could help Charlie enhance his initial learning of information. Frequently repeating instructions for him and, when doing so, maintaining his eye contact and speaking in short, simple phrases, pausing between statements, would allow him time to process the material. Asking him to repeat or paraphrase instructions would provide an opportunity to demonstrate his understanding of the task and practice rehearsal strategies. Charlie demonstrated weaknesses with several measures of attention-based functioning, particularly in processing speed. A pharmacotherapy trial might be undertaken to determine what positive impact it may have on his overall functioning. It would be important to carefully observe his adjustment to medication to determine whether it is producing the desired benefit and monitor for any unwanted side effects. Charlie seemed to attend fairly well over short time frames, so breaking down longer tasks into shorter segments may help him focus on one segment at a time. Incorporating breaks between segments and providing him with reinforcement for maintaining his focus might help produce a more efficient working style

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while avoiding frustration secondary to fatigue. Charlie may continue to demonstrate motor restlessness, which may decrease over time with ongoing recovery. For the time being, these behaviors should be ignored, as long as they do not interfere with his education or progress in therapies. Allowing him opportunities to expend excess energy during breaks might also be considered. Charlie tended to engage in impulsive behaviors that could result in increased careless errors. It may be helpful to teach him self-monitoring through verbal mediation or self-talk strategies. Encouraging Charlie to narrate his approaches to problem solving may allow him to catch his mistakes. In addition, using a cardboard screen to cover all but one problem on a worksheet may help to avoid difficulties with distractibility. Testing revealed a pattern of lateralized weaknesses to the left side of the body, a finding that would not normally be anticipated with anoxic encephalopathy. Although the imaging conducted soon after the event was read as revealing no structural damage, it may be beneficial to consider additional neurological assessment to determine whether there is any correlating evidence and possibly to repeat imaging. Charlie was still in the early phases of recovery and likely would continue to make sizable gains at least for the next 6–12 months. Then recovery rates may slow substantially but still continue for the next 2–3 years. Reassessment in 12 months may be beneficial in monitoring the extent of his gains while providing an opportunity to direct other strategies for remediation, based on his level of functioning at that time.

Additional Resources Key Concepts and Terms Analytic Phonics The analysis of whole words to detect phonetic spelling patterns and then to split them into smaller parts to help with reading. Anoxic Brain Injury Brain injury caused by a lack of oxygen in the blood, also known as Anoxic Encephalopathy. Immediate Memory The capacity to retain information upon immediate presentation. Jaundice A condition characterized by yellowness of skin and the whites of eyes caused by excess bilirubin in the blood. Left Neglect The failure to attend to sensory stimuli presented to the left body side. Phonological Decoding Translating the letters or spelling patterns of a written word into speech sounds in order to identify the word and gain access to its meaning. Processing Speed The speed at which sensory information is processed, that is, how quickly one can produce a response after information has been presented.

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References Resources for Clinicians Ball, J., Budrionis, M., & Trott, K. (1987). Neuropsychological effects of cold water neardrowning in an identical twin. International Journal of Clinical Neuropsychology, 9(2), 71–73. Retrieved Tuesday, May 22, 2007 from the PsycINFO database. Christensen, D., & Jansen, P. (1997). Outcome and acute care hospital costs after warm water near drowning in children. Pediatrics, 99(5), 715. Retrieved Tuesday, May 22, 2007 from the Psychology and Behavioral Sciences Collection database. Cruikshank, B., Eliason, M., & Merrifield, B. (1988). Long-term sequelae of cold water neardrowning. Journal of Pediatric Psychology, 13(3), 379–388. Retrieved Tuesday, May 22, 2007 from the PsycINFO database. Johnstone, B., & Bouman, D. (1992). Anoxic encephalopathy: A case study of an eight-year-old male with no residual cognitive deficits. International Journal of Neuroscience, 62(3), 207–213. Retrieved Tuesday, May 22, 2007 from the PsycINFO database. O’Flaherty, J., & Pirie, P. (1997). Prevention of pediatric drowning and near-drowning: A survey of members of the American Academy. Pediatrics, 98(2), 169. Retrieved Tuesday, May 22, 2007 from the Psychology and Behavioral Sciences Collection database. Pearn, J., Bart, R., & Yamoka, R. (1979). Neurologic sequelae after childhood near-drowning: A total population study from Hawaii. Pediatrics, 64(2), 187. Retrieved Tuesday, May 22, 2007 from the Psychology and Behavioral Sciences Collection Database. Quan, L., Wentz, K., Gore, E., & Copass, M. (1990). Outcome and predictors of outcome in pediatric submersion victims receiving prehospital care in King County, Washington. Pediatrics, 86(4), 586. Retrieved Tuesday, May 22, 2007 from the Psychology and Behavioral Sciences Collection Database. Quart, L., Gore, E., Wentz, K., Allen, J., & Novack, A. (1989). Ten-year study of pediatric drownings and near-drownings in King County, Washington: Lessons in injury prevention. Pediatrics, 83(6), 1035. Retrieved Tuesday, May 22, 2007 from Psychology and Behavioral Sciences Collection database. Tipton, M., Eglin, C., Gennser, M., & Golden, F. (1999). Immersion deaths and deterioration in swimming performance in cold water. Lancet, 354(9179), 626–629. Retrieved Tuesday, May 22, 2007 from the Psychology and Behavioral Sciences Collection database. Zelman, K. (1999). Memory, attention, and academic achievement skills in children who Survive near-drowning accidents. Retrieved Tuesday, May 22, 2007 from the PsycINFO database. Zelman, K (2002). Neurodevelopmental outcome for extended cold water drowning: A longitudinal case study. Journal of the International Neuropsychological Society, 8(4), 588–595. Retrieved Tuesday, May 22, 2007 from the PsycINFO database. Zelman, K (2005). Anoxic brain injury following near-drowning in children. Rehabilitation outcome: Three case reports. Brain Injury, 19(13), 1147–1155. Retrieved Tuesday, May 22, 2007 from PsycINFO database.

Resources for Families Bierens, J. J. L. (2005). Handbook on drowning: Prevention, rescue, treatment. Berlin Heidelberg New York: Springer 714. Dworkin, G. M. (1997). The Heimlich controversy in near-drowning resuscitation. Parks and Recreation, 32(11), 16. University of Alaska Sea Grant. (1990). Cold water near‐drowning. Alaska: University of Alaska Sea Grant. Verive, M., Heidemann, S., & Fiore, M. (2007). Near drowning. Emedicine. Retrieved August 27, 2007 from http://emedicine.com/ped/topic2570.

Part II

How It Can All Go Wrong: Developmental Disorders

At the very beginning of her introductory graduate course in child development at the University of Kansas three decades ago, the noted developmental psychologist Frances Dean Horowitz challenged her students with the question, What makes development happen? She was speaking to those of us who would devote our careers to researching normal development or understanding and working to “fix” abnormal behavior and cognition or some combination of these ventures. This question also undergirds what Fletcher and Taylor (1984) termed “developmental neuropsychology” as an approach to the study of developmental disabilities. This class of disorders, which in plain English could be described as “just grew up that way,” is the focus of Part II. In an incisive analysis of several major types of developmental problems that are often assessed by pediatric neuropsychologists, Pennington (1991) proposed that symptoms within any given syndrome can be grouped into an important hierarchy. The most important difficulties – often implied to be causal or even necessary and sufficient for the disorder to occur – that a child is experiencing are conceptualized as primary, whereas associated symptoms that often but not always appear in a certain disorder are considered secondary, correlated, or artifactual. For example, phonological processing deficits are generally considered to be a proximal cause of most cases of word-level reading disability (often used synonymously with developmental dyslexia and learning disability) in basic reading. Debate continues on the degree to which genetic predisposition vs. environmental factors (especially exposure to different instructional approaches and the extent or intensity of such exposure) contributes to reading decoding difficulties in any individual case, although ample research evidence has been replicated to demonstrate the existence of both sources of cause in samples of children and adults with reading disabilities in general. Thus, Pennington (1991) sorted the symptoms of dyslexia as follows (Box 1): Primary: problems with reading and spelling, problem in the phonological coding of written language Secondary: poor reading comprehension, poor math, poor self-esteem, letter reversals, eye movement differences in reading

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Box 1 Pennington’s (1991) symptom catagories Primary: core symptoms, universal, specific, and persistent Correlated: same etiology, affect different brain systems Secondary: consequences of above symptoms Artifactual: appear associated, not causally related

Correlated: problems in language processes—articulation, naming, verbal shortterm memory, verbal long-term memory Artifactual: problems with attention, delinquency, visual-spatial problems Let’s play a game. As you read the stories in Part II, we invite you to pause and try answering Horowitz’s challenge by sorting the story parts into Pennington’s symptom categories. You could get clues by perusing Chaps. 17 and 18 in reference to the dyslexia example provided or by reading Pennington’s excellent book to see how he sorts several other disorders, such as the attention-related problems in Chaps. 15 and 16 and the autistic spectrum disorders in Chaps. 22 and 23. In the end, your own ever-developing knowledge base about the science of child development will shape your response to Horowitz’s query. Have fun!

References Fletcher, J. M., & Taylor, H. G. (1984). Neuropsychological approaches to children: Towards a developmental neuropsychology. Journal of Clinical Neuropsychology, 6, 39–56. Pennington, B. F. (1991). Diagnosing learning disorders: A neuro-psychological framework. New York: Guilford Press.

Chapter 15

The Energizer Bunny Meets Shirley Temple: Attention Deficit Hyperactivity Disorder, Combined Type Michael J. Zaccariello

‘‘She will not sit still and be quiet! She just goes and goes nonstop.’’ Jenna’s exasperated mother moaned during the initial clinical interview. Her voice was tinged with both frustration and guilt over her reactions to her daughter’s behavior that occasionally resulted in abrupt, sharp rebukes. In addition, Jenna herself had begun to realize that something was ‘‘wrong.’’ This previously effervescent and optimistic child began to remark that she was stupid and dumb and was being ridiculed by peers at school. Historically, Jenna was a highly energetic toddler who talked excessively. Although never significantly or consistently oppositional or argumentative, her behavior became increasingly more difficult to control as she grew older. Jenna’s mother noted that she literally played all day long and seemingly never tired. In addition, it was impossible for her to sit down appropriately for longer than a few minutes at a time. She constantly fidgeted, kicked her legs, or touched objects. Cognitively, she had extreme difficulty staying focused and on-task, and needed frequent redirection or a quiet, nonstimulating environment to complete work. She had a tendency to be impulsive, rushing through her school assignments, which resulted in careless mistakes. She had organizational problems in her day-to dayactivities (e.g., frequently losing objects or articles of clothing). Academically, she had always been an average to above average student, but starting in the second grade she began to demonstrate some struggles in mathematics, spelling, and handwriting. Her mother’s perception was that Jenna’s difficulties in these academic areas were not reflective of a skill deficit but, rather, extreme problems with sustained focus and impulsivity. Medically, Jenna was in perfect health, and both language and motor developmental milestones were reached within normal limits. She had no significant problems with vision, hearing, eating, or sleeping. She was prescribed no medication at the time of the evaluation. Socially, Jenna was described as an outgoing and engaging child who enjoyed horseback riding and any outdoor activities. She lived with her parents and an older

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brother who had been diagnosed with Attention Deficit Hyperactivity DisorderPrimarily Inattentive Type (ADHD-I) by a pediatric neuropsychologist. The home environment was described as positive and loving. Jenna’s parents requested a neuropsychological evaluation to identify her pattern of cognitive strengths and weaknesses. However, their prime concern was her increasing restlessness and difficulties with attention. They were also concerned with the emotional toll of social ridicule because of her high energy behavior. Her parents questioned if she met formal criteria for Attention Deficit Hyperactivity Disorder (ADHD) and wished for professional corroboration of their suspicion. Jenna had never undergone a neuropsychological evaluation, and her mother was curious about her overall cognitive skill set. Consequently, a broad approach was taken, with a focus on attention and mental executive function measures. Measures of attention are thought to assess one’s ability to direct and maintain focus on a task. Executive functions are considered a related construct and involve higher-ordered, goal-directed abilities that are organizational or supervisory in nature. This cluster of abilities allows one to engage in abstract reasoning, problem-solving, judgment, cognitive flexibility, planning, and organization. Utilizing feedback to correct responses, inhibit inappropriate behavior, and sustain one’s attention is also considered in this area. Specific domains that were assessed included intellectual ability, fine motor functions, attention, executive functions, language fluency, visual-spatial abilities, and comprehensive academic skills development. Behavioral ratings scales completed by both Jenna’s teacher and parents were included as a critical foundation of the assessment.

Conceptual Diagnostic Background With the introduction of the Diagnostic and Statistical Manual of Mental Disorders, Third Edition, in 1980, delineation was made between the presence and absence of hyperactive symptoms in children with attentional difficulties. Current diagnostics further clarified this distinction into three types: primarily inattentive (ADHD-I), primarily hyperactive-impulsive (ADHD-H), and combined (ADHD-C). However, some recent studies have questioned the diagnostic accuracy of the current categorical subtypes of ADHD and propose more of a dimensional model (Frazier, Youngstrom, & Naugle, 2007). Prevalence studies indicate that ADHD (devoid of a specific subtype) is diagnosed three times as often in boys than girls in community samples. This discrepancy rises to five to nine times more often in clinic-referred groups. It is thought that ADHD-C occurs more often in boys and that ADHD-I tends to be diagnosed more in girls. As children age, hyperactive symptoms typically dissipate, but inattention symptoms can remain prominent. In a sense, one does not typically outgrow ADHD. There has been much debate in the literature concerning the usefulness and relevance of neuropsychological tests in the diagnosis of ADHD. Although a comprehensive review of this issue is beyond the scope of this brief case,

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indications suggest that the most scientific and powerful diagnostic indicator of ADHD is a significant level of inattention or hyperactive symptoms as reported on behavioral rating scales. So, this begs the question, what is the point of a comprehensive neuropsychological evaluation of a child with ADHD features? Although not necessarily diagnostic, a neuropsychological evaluation can provide a snapshot of how significantly inattention or hyperactivity interacts with performance in other cognitive and academic domains. Once such areas have been identified, specific recommendations beyond those typically given for ADHD can be outlined in the report to maximize a child’s learning potential (Tables 15.1–15.3).

Test Results On formal testing, Jenna presented as an absolutely delightful and pleasant young girl. She readily engaged in conversation and expressed an appropriate range of emotion throughout the evaluation. Her social interactions were appropriate, and she spontaneously demonstrated a very kind and helpful attitude (e.g., picking up dropped items). In terms of her approach to testing, there was significant evidence of motor restlessness throughout the evaluation, coupled with moderate levels of distractibility, inattentiveness, and impulsivity. She had extreme difficulty sitting upright and still in her seat. She frequently fidgeted, got out of her chair, rubbed the walls, and looked at herself in an one-way mirror. Rather frequently, she reached for test stimuli before being told to do so and/or blurted out answers before receiving Table 15.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Full Scale IQ General Ability Index Verbal Comprehension Index Similarities Vocabulary Comprehension Perceptual Reasoning Index Block Design Picture Concepts Matrix Reasoning Working Memory Index Digit Span Letter-Number Sequencing Processing Speed Index Coding Symbol Search a Standard score, (scaled score), [T-score], {z-score}.

Scorea 113 111 99 (10) (8) (12) 121 (12) (14) (14) 110 (11) (13) 109 (12) (11)

Percentile 81st 77th 47th

92nd

75th

73rd

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Table 15.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition Broad reading Letter-word identification Passage comprehension Reading fluency Basic reading skills Letter-word identification Word attack Broad math Calculation Applied problems Math fluency Broad written language Spelling Writing samples Writing fluency Academic skills Academic applications Academic fluency

Table 15.3 Other cognitive functions Tests Attention-Deficit Hyperactivity Disorder Rating Scale – Fourth Edition Home Version Total Hyperactivity-impulsivity Inattention

Scorea

Score 108 110 100 108 109 110 106 120 113 126 103 104 103 104 102 110 112 106

Percentile 71st 75th 51st 70th 73rd 75th 66th 91st 80th 96th 59th 60th 58th 60th 55th 75th 78th 65th

Percentile

>99th 97th–98th 98th–99th

School Version Total Hyperactivity-impulsivity Inattention

95th–96th 94th–95th 94th–95th

Achenbach Child Behavior Checklist Parent Form Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior

[63] [50] [53] [50] [58] [80] [55] [60]

90th 50th 62nd 50th 79th >97th 69th 84th

Teacher’s Report Form Anxious/depressed Withdrawn/depressed Somatic complaints

[50] [50] [50]

50th 50th 50th (continued)

15 The Energizer Bunny Meets Shirley Temple Table 15.3 (continued) Tests Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior

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Scorea [54] [50] [71] [59] [60]

Percentile 65th 50th >97th 81st 84th

Gordon Diagnostic System Vigilance task (9 minutes) Correct Commission errors

100 <1

50th <1st

NEPSY Auditory Attention and Response Set Attention Task Response set task

(10) (12) (10)

50th 75th 50th

Wisconsin Card Sorting Test Categories Total errors Perseverative responses Perseverative errors Nonperseverative errors Conceptual level responses Failure to maintain set Learning to learn The Tower of London Behavior Rating Inventory of Executive Function Parent Form Behavioral regulation index Inhibit scale Shift scale Emotional control scale Metacognition index Initiate scale Working memory scale Plan/organize scale Organization of materials scale Monitor scale Global executive composite

72

>16th 39th 45th 45th 30th 50th 2nd–5th 2nd–5th 3rd

[61] [80] [45] [48] [74] [67] [70] [69] [59] [87] [70]

86th 96th 38th 50th 97th 95th 94th 94th 83rd 99th 96th

96 98 98 92 100

instructions. Despite these behaviors, she was relatively easy to redirect but did require constant prompting throughout the test session. Consistent with Jenna’s test session behavior, both her parents and teacher were observing a significant level of inattentive and hyperactive symptoms. Her mother also noted marked problems with impulse control, general organizational skills, and the ability to self-monitor behavior. Her cognitive and behavioral difficulties were leading to mild to moderate compliance issues at home and school.

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A quick glance at Jenna’s neuropsychological profile revealed that most of her scores were average to above average. Overall intellectual ability was high average with a significant strength in nonverbal intellectual ability relative to her solidly average verbal intellectual ability. She had little difficulty on specific tests of language, visual-spatial processing, or fine motor dexterity. Her academic skills development was progressing at the expected rate with no evidence of deficit in any academic domain. A different picture emerged on tests of attention and mental executive function. Her test scores ranged from average to impaired. Jenna had difficulty on most tasks where she had to rely on self-regulation strategies to successfully negotiate the measure and did not have the examiner encouraging her to remain attentive. She was impulsive, which resulted in a high number of mistakes. In addition, she displayed a weakness in being able to change problem solving strategies efficiently.

Formulation and Recommendations On the basis of the responses to behavioral questionnaires and test session behavior, Jenna had significant inattention, distractibility, impulsivity, and motor restlessness, which were consistent with individuals diagnosed with ADHD-C. She could not sit appropriately for longer than 10–15 min at a time and frequently reached for test stimuli or blurted out answers. Jenna was subsequently diagnosed with ADHD-C, which includes a mixture of problems with behavioral inhibition, sustained attention, organization, consistency of performance, and/or goal-directed behavior. ADHD-C is the most common form of ADHD that arises in childhood in 3–7% of the population (American Psychiatric Association, 2000). The symptoms of ADHD do not spontaneously disappear in the majority of cases but can be treated to some extent with behavior management and/ or medication. Jenna’s combination of ADHD-C features included average intellectual ability and average to above average underlying academic skills. Therefore it would be realistic for her to attain average to above average performance in school most of the time. Although not directly assessed, another important finding concerned is the Jenna’s emotional functioning. During the assessment, she acknowledged struggles in school and admitted self-esteem issues related to her attentional problems. Studies have shown that 40% of children with ADHD show signs of either depression and/or anxiety (Spencer, Biederman, & Wilens, 1999). It is vitally important to address the behavioral and cognitive difficulties that children with ADHD typically experience. However, professionals, caretakers, and school personnel should also be acutely aware of the impact and range that ADHD can have on a child’s emotional and social well-being. Her parents were provided with psychotherapy referrals, which they could use if her distress continued or worsened. At least 75% of children with ADHD benefit from stimulant medication. Jenna’s behavioral and cognitive presentation of ADHD symptoms make an evaluation for stimulant medication routinely recommended. If she continues using the medica-

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tion over a long period of time, observation trials off medication for a week at a time approximately twice during each school year would be useful to monitor her ongoing benefit or need for the medication. Standard suggestions for managing attention and organization problems in the classroom included obtaining eye contact with Jenna prior to delivering directions. It may be helpful to place a hand on her shoulder or arm and to be sure that directions are clear, simply stated, and given one at a time. Delivering more complex directions in brief, simple, numbered steps (e.g., ‘‘First, read pages 1–10; second, answer questions 1–5; and third, check answers in the back of the book’’) would also be helpful. If Jenna continues to have difficulty, writing down key instructions and taping them to her desk may help cue her. Presenting material in small, successive units that can be mastered hierarchically would allow Jenna to maximize her attentional capacity, assist in organizing the material to be learned, reduce the feeling of being overwhelmed by the material, and develop greater self-confidence as she progresses through the material. Jenna would need distractions minimized to the greatest extent possible in the classroom (e.g., seating her at the front of the class and increased one-to-one contact with the teacher), as well as regular feedback provided with concrete suggestions for appropriate behaviors. It would also be helpful to provide consistency and structure through daily schedules; standard seating arrangements; clearly defined classroom expectations, rules and consequences; and clear places for necessary materials, such as color-coded subject folders, and reinforcement for using organizers. Jenna needs guided practice in planning how to complete assigned tasks (what is needed, how to break tasks into manageable parts, estimating time needed for each part) and to be assigned tasks or classroom duties that she can successfully complete. It would be helpful to provide other organizational checklists, such as steps to get ready to go home after school, and to remind Jenna at the end of the day about what she needs for home and the next day. Also, Jenna would benefit from pacing her work and changing the pace or task frequently and from opportunities for controlled movement. Given Jenna’s inattentive and hyperactive tendencies, she would not always be expected to perform at top efficiency level on timed tasks. Extra time can be given strategically when needed, and curriculum requirements such as timed math tests might be loosened or modified for her, but not omitted. At home, Jenna needs more frequent attentional cueing. Adults should help her break complex tasks into smaller steps. Commands should be given one or two at a time, and longer sets of commands should be repeated, rehearsed, or written down. More frequent attentional cueing (e.g., ‘‘look at this now’’; ‘‘this is important to pay attention to’’) in everyday life will be needed in the long run, and adults should try to avoid becoming frustrated with this need. She should be given extra instruction in organizational skills such as outlining, diagramming, planning a sequence of steps in complex tasks, and taking strategic breaks during extended study time. Jenna and her family would benefit from ongoing consultation with a psychologist who is knowledgeable about behavior management approaches for children

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with ADHD symptoms. Family psychotherapy could also provide an opportunity to work on family interaction issues, emotional issues, and/or peer interaction issues that often accompany ADHD. Jenna’s difficulties with abstract reasoning and logical problem-solving tasks suggest that she may have difficulty with some complex, novel tasks. Specifically, she may find it difficult to analyze the requirements of a task and apply effective strategies toward a solution. As a result, she may get frustrated with the acquisition of novel cognitive skills such as higher level academics. Although not indicated at this time given her average to above average academic skills development, with a diagnosis of ADHD, Jenna may be eligible to have an Accommodation Plan prepared for her as mandated under Section 504 of the Rehabilitation Act of 1973. This could be particularly important if her academic performance begins to suffer due to her ADHD presentation as she progresses to higher grades with greater requirements for attention and self-control. Many of the specific attention and organization suggestions outlined could be incorporated into an Accommodation Plan.

Additional Resources Key Concepts and Terms Attention The cognitive process of selectively concentrating on one aspect of the environment while ignoring other things. Executive functions Processes that are most involved in giving organization and order to actions and behavior. Executive functions involve (1) strategic thinking and future planning, (2) the ability to inhibit or delay responding, (3) initiating behavior, and (4) shifting between activities flexibly.

References Resources for Clinicians American Psychiatric Association. (2004). Diagnostic and statistical manual of mental disorders (4th ed., Text revision). Washington, DC: American Psychiatric Association. Barkley, R. A. (2006). Attention-deficit hyperactivity disorder. (3rd ed.). New York: Guilford Press. Cutting, L. E., & Denckla, M. B. (2003). Attention: Relationships between attention-deficit hyperactivity disorder and learning disabilities. In H. L. Swanson, K. R. Harris, & S. Graham (2003), Handbook of learning disabilities (pp. 125–139). New York: Guilford Press. Ellison, P. (2005). School neuropsychology of attention-deficit/hyperactivity disorder. In R. C. D’Amato, E. Fletcher-Janzen, & C. R. Reynolds (Eds.), Handbook of school neuropsychology (pp. 460–486). New Jersey: Wiley

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Frazier, T. W., Youngstrom, E. A., & Naugle, R. I. (2007). The latent structure of attention-deficit/ hyperactivity disorder in a clinic-referred sample. Neuropsychology, 21, 45–64. Spencer, T., Biederman, J., & Wilens, T. (1999). Attention-deficit/hyperactivity disorder and comorbidity. Pediatric Clinics of North America, 46, 915–927. U.S. Department of Education, Office of Special Education Programs’ (OSEP), Individuals with Disabilities Education Act (IDEA) web site: http://www.idea.ed.gov/explore/home

Resources for Families A.D.D. Warehouse, www.addwarehouse.com/shopsite_sc/store/html/index.html. Provides general information on Attention Deficit Hyperactivity Disorder and a comprehensive catalog of books, tapes, and training materials. Barkley, R. A. (2000). Taking charge of ADHD: The complete, authoritative guide for parents. (2nd ed.) New York: Guilford Press. Children with Attention Deficit Disorders (CHADD), http://www.chadd.org. Lecture programs, parent support groups, and valuable information for parents and teachers. Dawson, P., & Guare R. (2003). Executive skills in children and adolescents: A practical guide to assessment and intervention. New York: Guilford Press. Flick, G. L. (1996). Power parenting for children with ADD/ADHD: A practical parent’s guide for managing difficult behaviors. Hoboken, New Jersey: Jossey-Bass. Learning Disabilities Association of America, http://www.ldanatl.org National Center for Learning Disabilities, http://www.ncld.org, www.schwablearning.org and http://www.ldonline.org/.

Chapter 16

‘‘He is not Working up to Potential’’: Atypical Attention Deficit Hyperactivity Disorder with Executive Weaknesses Jennifer Niskala Apps and Dawn Pflugradt

Robert was referred for neuropsychological evaluation because of concerns regarding his academic achievement. In fact, he had reportedly demonstrated inconsistent motivation in school for quite some time. In early elementary school, he had not shown any difficulty acquiring basic academic skills. However, he was occasionally distractible or inattentive. In fifth grade, his parents become concerned about his relationship with his teacher. They said Robert clashed with his teacher, developed more negative emotions about school, and struggled with classroom performance. He began therapy with a social worker, which appeared to enhance his coping skills, and he showed some improvement in the classroom, but his academic performances continued to be variable. He would ‘‘freeze’’ on certain tests, and his mother described him as learning faster when things were presented in a song or pattern. As Robert progressed through school, he appeared smart but did not seem to care about school, was nonchalant, and would not work to his potential. He had difficulty attending at times, inconsistently turned in assignments (even if he completed them), and would not complete work even if he was able to do it. In the year before he was referred for an evaluation, Robert completed ninth grade in a new school. The transition to the new school was difficult for him, as he had to make new friends while also adjusting to the high school environment. He did well initially, but his grades declined over the course of the year. As a result, he failed two math classes and was placed in summer school. The summer school teacher quickly indicated that Robert knew all of the mathematical content of the class, and the teacher reported being confused as to why he had not passed the class in the first place. Robert’s mother became increasingly concerned about his academic difficulties. She began to realize how much assistance with structure she gave him in the home environment, including help with planning and lists. She decided that it was time for a professional opinion about his development. When Robert’s mother met with the neuropsychologist, she described him as a good kid who was generally happy but had some difficulty expressing his emotions. While she highlighted his difficulties with organization, she also described him as

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laid back, with no overt anxiety or depression (other than situational and generally related to normal adolescent issues). Robert had never been defiant with his parents or had any problems with his conduct. He also had no sleep problems. Reportedly, he had recently become interested in his physical health, and he was active in exercising and remaining fit in hopes of joining his high school football team. His interest in football had also driven his decision not to use alcohol or other drugs. Robert’s mother described her pregnancy and delivery with him as normal and without complication; however, he was delivered 2 weeks late. He was described as an ‘‘easy-going and funny’’ infant, who developed into a good kid. He met all of his developmental milestones within normal limits. Starting at approximately 4 years of age, he developed chronic ear infections, which were treated with adenoidectomy and tonsillectomy, as well as placement of ear tubes. At this time, he was diagnosed with asthma and prescribed an inhaler to use on an as-needed basis, which he continued to use at the time of evaluation. He also had seasonal and environmental allergies, for which he was prescribed medications. Robert experienced a concussion while playing football last year, following helmet-on-helmet contact. He did not lose consciousness and was able to walk off the field by himself. Following the game, he experienced a severe headache for several days, although no memory loss or vomiting was involved. He was taken to the hospital, and cranial magnetic resonance imaging (MRI) and a computed tomography (CT) scan were normal. He remained out of practice for several weeks, following which he returned with no subsequent complications. His additional medical history consisted of only minor injuries throughout his lifetime. At the time of evaluation, Robert was living with his mother. His parents divorced when he was approximately 3½-years of age. He spent every other weekend with his father. Maternal family history was described as remarkable for mild depressive symptoms in his mother upon the deaths of her parents and learning difficulties in a nephew. Paternal family history was remarkable for alcohol problems and depressive symptoms. Family stressors were significant; when Robert was 2½-years old, the family experienced the loss of his infant brother. Approximately a year later, his parents divorced, and he and his mother moved in with his maternal grandparents. When Robert was around 6½-years of age, he and his mother moved into their own apartment; however, he remained close with his grandparents. At age 13, Robert experienced the death of his grandfather. Over the subsequent year, his grandmother became quite ill, and he and his mother were actively involved in her care. Robert was with her when she died the following summer. Following her death, he and his mother relocated, and he enrolled in his new high school and started ninth grade.

Test Results Given Robert’s past difficulties with distractibility, attention, and possible emotional challenges, these areas were the focus of assessment.

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Robert was presented as a pubescent adolescent male dressed in khakis, a Tshirt, and a baseball cap. His initial reaction to the examiner was appropriate, and rapport was established quickly. He was extremely polite throughout the evaluation day, and had good awareness of social cues. Although he did not initiate spontaneous social conversation, he was able to carry appropriate conversation when the examiner began it. Robert’s sustained attention appeared good, and he was not overly distractible in the one-to-one testing situation. However, his attention and motivation appeared to vary on certain tasks. At times, he would make silly mistakes, answer too quickly, or lose track of an item or thought. However, he was easily reorientated to task, and this did not appear to negatively impact testing. His mood was euthymic, and his affect was appropriate. Overall, Robert was very cooperative with the entire testing process. He appeared self-motivated and wanted to perform well. He worked persistently on items, although he was more inclined to say he did not know an answer when asked verbal questions. On visual items, he worked persistently until given the option to stop. In reviewing Robert’s evaluation results, his intellectual abilities were in the overall average range (Table 16.1). He demonstrated average verbal and nonverbal intellectual abilities. His current level of academic skill acquisition was in the average to above average range (Table 16.2). This was consistent with, or higher than, what would be expected given his overall intellectual abilities. Robert did not appear to be demonstrating any overt indications of a learning disability. However, Robert demonstrated variable attention and executive function skills (Table 16.3). His working memory was in the low average range, which was slightly lower than would be expected given his overall intellectual abilities. He demonstrated a statistically significant difference (p < 0.05) of 16 points between Verbal Comprehension and Working Memory Indices. Additionally, he had greater difficulty on

Table 16.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal Comprehension Index Perceptual Reasoning Index Working Memory Index Processing Speed Index Full Scale IQ Similarities Vocabulary Comprehension Digit Span Letter-Number Block Design Picture Concepts Matrix Reasoning Coding Symbol Search a Standard score, (scaled score), [T-score], {z-score}.

Scorea 104 104 88 97 100 (9) (13) (11) (8) (8) (13) (10) (9) (10) (9)

Percentile 61st 61st 21st 42nd 50th

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Table 16.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition, Form A Broad reading Letter-word Reading fluency Passage comprehension Broad math Calculation Math fluency Applied problems Broad written language Spelling Writing fluency Writing samples

Table 16.3 Other cognitive functions Tests Delis-Kaplan Executive Function System Verbal Fluency Test Letter fluency Category fluency

Score

Percentile

119 106 122 116 104 109 100 102 117 127 98 118

90th 65th 93rd 85th 62nd 72nd 50th 54th 87th 96th 45th 88th

Grade equivalent 13.5 10.6 14.1 18.0 10.5 12.1 8.9 10.3 13.0 16.0 8.4 17.7

Score

(8) (6)

Category switching Total Accuracy

(7) (8)

Design Fluency Test Condition 1 total Condition 2 total Condition 3 total

(12) (10) (11)

Color-Word Interference Test Color naming Inhibition Inhibition/switching

(8) (6) (9)

Tower Test Achievement Move accuracy ratio

(14) (9)

Conners’ Continuous Performance Test Omissions Variability Standard error by block

[53.71] [74.28] [84.84]

both verbal and visual tasks requiring the greatest amount of abstract thought. Further, he demonstrated mild relative weaknesses in executive skills including verbal fluency, the inhibition of behaviors, vigilance, and attention. He demonstrated average visual fluency, overall processing speed, and problem-solving

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skills. This pattern of mild executive dysfunction does not formally fall within the diagnostic category for Attention Deficit Hyperactivity Disorder (ADHD). However, the conceptualization of ADHD as a neuropsychiatric disorder of executive functions leaves open for interpretation how to categorize those children with mild executive difficulties.

Formulation and Recommendations The lack of specific diagnostic categories for various types of executive deficits often results in ADHD becoming the default diagnosis. Often these children exhibit symptoms that meet formal DSM-IV-TR criteria for the disorder, although in Robert’s case, his symptoms met formal criteria only minimally. He was qualified as inattentive because of his difficulties with attention to detail, sustained attention, follow-through, organization, and forgetfulness. However, these symptoms were not clear enough to teachers and caregivers for Robert to appear as ‘‘classically ADHD.’’ In cases like Robert’s, the clinician must help those living and working with the child to understand how relative weaknesses in verbal fluency, impulsive verbal behaviors, variable attention, mild distractibility, and lower relative abstract reasoning skills can be conceptualized as a variant of ADHD and result in impaired functioning. Individuals who experience fundamental weaknesses in executive functioning generally demonstrate a host of difficulties with their behaviors. Executive functions allow a person to solve problems, think abstractly and flexibly, plan and organize behavior, and alter behaviors based on experience and feedback. Problems in these areas can result in difficulties organizing information and efficiently managing large amounts of information. Individuals may not use prompts well and may have trouble thinking flexibly, often becoming stuck responding in inefficient manners. Formal testing offers a window into a person’s executive skills but does not comprehensively measure such a complex construct. Indeed, the introduction of executive function measures is still relatively new in the field of neuropsychology, with some areas having a greater predominance of research available. Measures of fluency, verbal working memory, inhibition, and planning have relatively significant bodies of research contributing to overall validity. However, many of these measures are not available at all ages. Further, our understanding of how all aspects of executive skills develop in children continues to be an area requiring further research. Therefore, while measures in these areas can inform, they are not all inclusive in their explanation of a child’s functioning. Robert appeared to struggle with some of these skills, particularly relative to his overall average intellectual abilities. These relative weaknesses were likely to impact his ability to acquire adequate study skills. However, his additional cognitive strengths were areas from which he could build upon in order to learn to compensate for his relative weaknesses.

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Robert’s emotional development appeared age-appropriate. However, he was struggling to reconcile his known abilities with his apparent weakness in remaining organized and studying successfully. Robert had become stuck in a pattern of expecting himself to somehow try harder. Intervening with Robert and his family to help them understand how to compensate for his weaknesses became an important step in his emotional growth and self-esteem. Robert’s family was informed that he would likely function best in a structured, consistent environment free of relative distractions. Although his executive weaknesses did not appear significant enough to qualify him for special education assistance through an Individualized Educational Program (IEP), his parents were strongly encouraged to help him learn appropriate study skills and environmental controls in order to help him compensate for his difficulties (some environmental compensations in the home environment are listed in Box 16.1). Robert was likely to experience some difficulty organizing materials and keeping track of multiple classes and assignments. More abstract assignments and concepts might be harder for him, and he might have difficulty acquiring effective study strategies, particularly compared with other young men of his age. A tutor or academic mentor was strongly encouraged to provide the structure, guidance, and assistance Robert may need to organize, synthesize, and manage the amount of information involved in high school courses. This tutor should be someone whom he can get along well with, who is less like a parent and more like a coach. The tutor

Box 16.1 Recommendations for the environment l l l

l

l

l l

Keep rules clear and brief, and keep task lists short. Provide organizational structure, including using lists, calendars, and structured routines. Provide a distraction-free environment for the completion of homework or other chores, including turning off the TV, video games, and/or music, closing windows, and reducing noise. Assist Robert in getting started with assignments or projects, if he requires this, by ensuring that he understands all of the instructions and expectations. Build in breaks, planned interspersed times of sustained attention for longer assignments or projects. Establish a regular routine for homework with a specific time, place, and schedule. Monitor and give feedback, while not doing all of the work together, by praising positive effort and hard work.

Box 16.2 Where do I find a tutor? Parents often find tutors in a wide variety of places. A few places to start inquiring might include the following: l l l l l

Family members with a background in education Past and current teachers Local universities and colleges Neighbors Pastors or church members

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or mentor should have experience working with children with mild attention and executive problems (Box 16.2). Robert and the tutor should meet as necessary, probably two to three times a week, and the tutor could provide structured study skills. Such a relationship will also allow for additional presentations of material as needed, guidance in organizing and keeping track of classes and assignments, structure and discipline in study skills, and alternative explanations for more difficult abstract concepts. Further, the tutor would be encouraged to help Robert learn how to recognize hints or cues in assignments that describe what output is expected and could model and help Robert learn how to break larger tasks into logical steps. An overall goal for Robert would be to learn better self-evaluation skills, so that he can more effectively monitor and alter his own behavior and actions academically. Robert’s tutor and his parents were encouraged to work closely with each of his teachers to encourage assistance, such as additional visual study aids or written handouts of any lectures. Robert was likely to have greater difficulty than other students in taking notes in a timely fashion and remaining orientated to longer auditory lectures. Utilizing multimedia formats in the classroom may be helpful, such as tape recording lectures. If Robert’s school were to need a formal document to provide him with these recommendations, his parents were encouraged to share the neuropsychologist’s report with his school’s special education director and to request that Robert be evaluated for a 504 plan to assist him with his academic weaknesses. Section 504 ensures that children with disabilities who do not qualify for formal assistance through the special education department still have access to accommodations that they may require. Robert’s family was advised to continue to monitor his academic and emotional development. Returning to a mental health care professional was recommended should any further emotional or cognitive struggles develop. However, it was anticipated that with the improved understanding both Robert and his family now had about his executive weaknesses, he would be able to develop compensatory strategies and experience increased academic success and improved self-esteem.

Additional Resources Key Concepts and Terms Attention Deficit Hyperactivity Disorder A disorder defined by the Diagnostic and Statistical Manual of Mental Disorders (2000) that encompasses problems with inattention, distractibility, and at times hyperactivity and impulsivity, which reach a level of impairment across environments. Executive Functions A cluster of cognitive abilities including but not limited to working memory, attention, planning, set-shifting, and problem solving associated with prefrontal-subcortical brain systems.

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504 Plan Part of the civil rights act, Rehabilitation Act of 1973, which protects the rights of people with various forms of disabilities to ensure that they are not denied access to any program that receives federal funding.

References Resources for Clinicians American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders (4th ed., text revised). Washington, DC: American Psychiatric Association. Barkley, R. A. (2005). ADHD and the nature of self-control. New York: Guilford Press. Dawson, P., & Guare, R. (2004). Executive skills in children and adolescents: A practical guide to assessment and intervention. New York: Guilford Press. Furman, L. (2005). What is attention-deficit hyperactivity disorder (ADHD)? Journal of Child Neurology, 20, 994–1002. Nigg, J. T. (2006). What causes ADHD: Understanding what goes wrong and why. New York: Guilford Press. Pliszka, S. R. (2003). Neuroscience for the mental health clinician. New York: Guilford Press. Shallice, T., Marzocchi, G. M., Coser, S., Meuter, R. F., & Rumiati, R. (2002). Executive function profile of children with attention deficit hyperactivity disorder. Developmental Neuropsychology, 21, 43–71. Voeller, K. K. S. (2004). Attention-Deficit Hyperactivity Disorder (ADHD). Journal of Child Neurology, 19(10), 798–814.

Resources for Families Children and adults with attention deficit/ Hyperactivity disorder: http://www.chadd.org Edward M. Hallowell. (1994). Driven to distraction: Recognizing and coping with attention deficit disorder from childhood through adulthood. Random House, New York, USA: Pantheon Books. Michael Gordon. (1990). ADHD/Hyperactivity: A consumer’s guide for parents and teachers. New York: GSI Publications. Mountain Plains Regional Resource Center. A parent and educator guide to section 504: Another service option for children with disabilities. http://www.rrfcnetwork.org/images/stories/ MPRRC/Products/Generic/Section504/504parentguide.pdf. National Resource Center on ADHD: http://www.help4adhd.org. Parent Advocacy Coalition for Educational Rights: http://www.pacer.org/index.htm Russell Barkley. (2000). Taking Charge of ADHD: The Complete, Authoritative Guide for Parent. (2nd ed.,) New York, USA: Guilford Press. Russell Barkley. (2005). ADHD and the nature of self-control. NY, USA: Guilford Press. The San Diego ADHD Project: http://www.sandiegoadhd.org.

Chapter 17

Sorting Sounds: Reading Disability with Phonological Awareness Deficit Robert F. Newby

As Angie’s father was reading to her one evening near the end of junior kindergarten, he paused in the story to play a game with her of matching letters to their sounds. Angie had been slow in learning to recognize printed letters earlier that year, and he thought it would be good to practice some more advanced alphabet skills now. He was dismayed to discover during the game that she was bafflingly inconsistent at recognizing even the /a/ sound that began her own name. Had Angie’s teacher not commented recently that the class was working on this for the past several weeks? After bedtime, Angie’s father called his sister, who was a reading specialist in the next town. ‘‘Classic sign,’’ she burst out immediately, ‘‘particularly since Angie has talked up a storm since the first words came out of her mouth!’’ Classic sign of what? How could Angie’s aunt detect something with such clarity after her dad’s brief phone description? In fact, her aunt was right: Angie turned out to have a classic case of emerging word reading disability, and catching it early was a good thing. On her aunt’s suggestion, Angie’s parents next requested testing by an Individualized Education Program (IEP) team at school, who documented her strong general intelligence, with robust verbal comprehension skills in particular. Angie also was whizzing ahead in early math development, drew with artistic flair, and wrote numbers with amazing precision, but she meandered through most attempts to write letters as if she were drawing abstract expressionism. She simply could not do the oral language tests that asked her to put together sounds like n-e-t into one word (even though she was an avid fisherwoman) or to take out a single sound like the /c/ from the word cart to come up with the word for her favorite subject at school. She also was very slow in saying the names of simple color spots on a page, even though she already was choosing paint for her room with names like fuchsia. The IEP team concluded that Angie was at risk for the emergence of a reading disability and offered to pull her out of some class time during senior kindergarten

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the next fall for extra training in phonemic awareness (like the test tasks just illustrated), beginning phonics skills (the association of sounds and printed letters), and early literacy conventions (such as scanning from left to right and handling basic punctuation like periods). Angie’s parents were stunned. Reading disability in such a bright girl? What were they to do? They decided to invite Angie’s aunt for a coffee conference the next day to try to put the puzzle together. At coffee, Angie’s mom added an interesting twist to the story: ‘‘Ever since we decided to pursue this testing, I have had this sinking de´ja` vu that I couldn’t quite pinpoint, but it just came to me this morning. Remember when I spent that summer after first grade at the college department of education clinic, being a guinea pig for those graduate students practicing their teaching methods? And how hard reading was for me all the way through grade school? Well, it is flashback time now: Angie is me all over again.’’ Angie’s mom needed some support from both family members and professionals to get around her fear that Angie was doomed to struggle throughout her school career. At this point, Angie’s aunt felt that it would be best for the family to have some additional expert consultation outside of school, so she referred Angie for a neuropsychological evaluation to clarify the apparent emerging reading disability and to add other relevant recommendations for intervention.

Test Results The evaluators considered all Angie’s results from the school’s testing valid and reliable, because her effort, cooperation, attention, stamina, self-control, mood, and social response were positive throughout all sessions. She was aged 5 years and 6 months at the time. Angie’s overall intelligence was in the high average range, with no significant difference between verbal and nonverbal reasoning abilities, including some strongly above average subskills in each domain. Although none of the academic skills tested at school were below average, indicators of alphabet knowledge, beginning visual cue word recognition, and letter-sound awareness were in the lower half of the average range. Graphomotor (paper and pencil) skills were average, and math reasoning was high average to above average. In contrast, both phonological awareness and rapid naming were mildly impaired and significantly discrepant from verbal reasoning (Tables 17.1–17.3). Some children have had enough relevant testing prior to referral for pediatric neuropsychological evaluation, so that little or no further testing is necessary. In these cases, the pediatric neuropsychologist assumes the role of a consultant, explaining results coherently to parents and helping to flesh out an optimal treatment plan. This is what happened with Angie. Several other domains of neuropsychological functioning could have been assessed for thoroughness, including manual dexterity, other basic sensory and

17 Sorting Sounds: Reading Disability with Phonological Awareness Deficit Table 17.1 Intellectual Differential Abilities Scale Scorea Verbal cluster 112 Nonverbal cluster 107 General cognitive ability 110 Verbal comprehension [49] Naming vocabulary [65] Picture similarities [68] Pattern construction [43] Copying [51] Early number concepts [55] a Standard score, (scaled score), [T-score], {z-score}.

Table 17.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition, Form A Letter-word Passage comprehension Word attack Spelling Writing samples Applied problems

161

Percentile 79th 68th 75th 46th 93rd 96th 24th 54th 69th

Score

Percentile

Grade Equivalent

93 101 92 98 95 119

33rd 53rd 30th 45th 38th 90th

K.1 K.8
Table 17.3 Other cognitive functions Tests Comprehensive Test of Phonological Processing Phonological awareness Phonological memory Rapid naming Elision Blending words Sound matching Memory for digits Nonword repetition Rapid color naming Rapid object naming Beery-Buktenica Developmental Test of Visual-Motor Integration

Score

Percentile

76 91 76 (6) (6) (7) (9) (8) (5) (7) 106

5th 27th 5th

66th

motor skills, memory, receptive and expressive language, attention, executive mental functions, and other categories of academic skill included in the federal guidelines for learning disability in the Individuals with Disabilities in Education Improvement Act (Table 17.4). In addition, from a psychometric perspective, zero

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Table 17.4 Categories of learning disability in federal special education law l

Oral expression

l

Listening comprehension

l

Written expression

l

Basic reading skill

l

Reading fluency

l

Reading comprehension

l

Mathematics calculation

l

Mathematics problem solving

or very low raw scores on several subtests raised questions about whether those skills were being sampled with adequate range of item difficulty. The decision was made against further testing in this case for three main reasons: First, neither Angie’s history nor the testing already completed raised concerns in any of these other domains. Second, there is something to be said for pragmatic focus to conserve health care and rehabilitation resources. For instance, what if Angie’s parents wanted to consider marshalling family funds to buy out-of-school tutoring services more than exhaustive testing? Third, the psychometric considerations were outweighed by the consistency of the pattern in data from all available resources.

Formulation and Recommendations In this relatively basic neuropsychological profile, a clear pattern of solid, roughly equivalent core verbal reasoning and visual-spatial problem-solving is seen, contrasting subtly but not convincingly with some low average early academic skills. Clinicians commonly observe that formal academic achievement test scores obtained at kindergarten or early elementary age often do not capture the emerging struggles of many students who eventually have learning disabilities. Early testing also often fails to find a large aptitude-achievement discrepancy that comprises a cornerstone of the traditional and legal definitions of LD, which have been seriously questioned in recent research and scholarly discussion. Keeping these caveats in mind, the most salient aspects of Angie’s profile consisted of her difficulties in phonological awareness and rapid naming. These red flags triggered the recommendations by the IEP team outlined earlier. Without formally classifying Angie into special education services, the team was advising a trial to determine her response to intervention as a prestep to special education. Angie was considered at risk for word reading disability or developmental dyslexia, which is the most common type of learning disability. The pattern can range from mild to severe, so even highly qualified professionals sometimes disagree about when to apply the diagnosis. The main learning difficulty in word

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reading disability involves recognizing or decoding words in reading, but secondary difficulties in spelling, written expression, reading comprehension, memorizing math facts, or math word problems often accompany the key word recognition deficit. Early problems in learning the alphabet, writing letters and numbers, associating letters with their sounds, or rhyming can place a child at risk for dyslexia, but many children outgrow these problems and become normal readers. For this reason, reading disability is often not diagnosed until at least second grade and often not until the child has been exposed to an appropriate form of extra help in reading beyond the regular classroom. The underlying cognitive processing cause in almost all cases is a core deficit in phonological awareness, including segmenting words into component sounds, blending sounds together, and associating letters and letter clusters with their appropriate sounds. Some children with reading disability also show reduced speed in reading symbols (letters and numbers) and/or naming commonly recognized items (colors and pictures of objects); this type of rapid naming deficit often is associated with slow reading fluency or speed, as discussed more thoroughly in the Chaps. 19 and 20. Because a subtle difficulty in specific aspects of language processing in the brain is implied in a diagnosis of reading disability, it is important to rule out environmental causes such as inappropriate instruction when making the diagnosis. Although a growing body of research is beginning to clarify what this brain processing problem may be, no reliable medical tests have been developed yet to establish the diagnosis. Many children with reading disability have immediate or extended family members with similar problems, and there is strong scientific evidence that the phonological processing problems underlying this pattern can be genetic. Myths about dyslexia can mislead families who are trying to get appropriate help for their children, especially the idea that the learning problem is caused by a detectable basic vision difficulty, such as eye tracking. In light of an ample body of outcome research in reading interventions, built on the knowledge base outlined in the previous paragraph, the following recommendations were made for specific instructional techniques for Angie. Children with phonological awareness deficits are at risk for reading disabilities and usually need extra training in segmenting and relating sounds in oral speech. This is called phonemic awareness training. Phonemic awareness is a more basic process than traditional phonics training, which focuses on the correspondence between written letters and sounds in speech. Recent research suggests that most children in kindergarten or first grade can learn these prephonics skills through regular exercises in which they carefully listen for and articulate phonemes in oral speech, break words into sound components or phonemes, match similar sounds in words, and detect or produce rhyming words. Older children who already have diagnosed reading disabilities also may benefit from this type of training, but it takes a lot more time to achieve the benefit. Work on traditional phonics skills should be emphasized in Angie’s reading program. The most effective phonological development components in reading

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research at this time involve systematic review of basic phonics elements, such as letter sounds when needed; practice in segmenting short words in print; blending methods, such as orally stretching out the sounds in words; contextual application of analytic phonics methods, such as word families; and an integration of reading and writing activities. Reading decoding work should focus on reading material that is at Angie’s instructional level for word recognition at any given time. Angie probably will need extra instruction in some aspects of written language, particularly spelling. It is often most helpful to have children focus on spelling words that are integrated with their reading curriculum and/or words that are grouped together in phonologically related word families. The use of a ‘‘word wall’’ would be helpful for Angie. In classroom application, this consists of a list of words on the wall, grouped according to their beginning letters alphabetically, to which children can refer when doing written work. Children are expected to spell correctly all words on the word wall in their written work, and words are gradually added as they are taught in the curriculum. Easily confusable similar words (e.g. what, when) are printed on different colored paper and cut out with the outline shape closely following the configuration of the letters in the word. This type of system can be individualized with a chart on the child’s desk if the child is working at a lower level than the overall class or if this type of system is not usable in the whole class format. In the long run, if Angie’s spelling development does not fully normalize, the goal may be for Angie to spell reliably a circumscribed list of commonly used words. Parents and school staff should monitor Angie’s progress closely in the related areas of reading fluency, reading comprehension, sentence construction, paragraph structure, and organization of longer written products, in case extra instruction in these areas becomes necessary.

Update The end of Angie’s story? Taking advantage of the extra instruction available at school during kindergarten and first grade, her aunt’s expertise in after-school tutoring during weekends and summers, a semester in the small-group reading program at the very same college clinic her mother had attended, and the wonderful additional knowledge they gained by reading Dr. Sally Shaywitz’s book for parents and teachers, Overcoming Dyslexia, Angie and the adults working with her accomplished just what the title of Shaywitz’s book says. Now in second grade, Angie reads just above the average range in all three of the key areas of word decoding, fluency or speed, and comprehension. Her spelling is still a little below average, but in her free time she is already composing clever plays for her friends to act out. Researchers in reading intervention now estimate that the majority of cases (with numbers ranging from 75% to as high as a perhaps overoptimistic 95%) of ongoing reading disabilities essentially could be prevented by the type of early identification and intervention that Angie received. Three other case examples, which illustrate variations that deserve the keen eye of the sophisticated clinician, will be considered in the next chapters to further

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elaborate on Angie’s case, which represents a prototype of a well-assessed and properly treated positive outcome in a young child with the most common kind of phonological processing deficit.

Additional Resources Key Concepts and Terms Dyslexia The International Dyslexia Association IDA Board of Directors, on November 12, 2002, adopted the following definition, which is also used by the National Institute of Child Health and Human Development (NICHD): Dyslexia is a specific learning disability that is neurological in origin. It is characterized by difficulties with accurate and/or fluent word recognition and by poor spelling and decoding abilities. These difficulties typically result from a deficit in the phonological component of language that is often unexpected in relation to other cognitive abilities and the provision of effective classroom instruction. Secondary consequences may include problems in reading comprehension and reduced reading experience that can impede growth of vocabulary and background knowledge.

Individualized Education Program (IEP) Defined by federal law, an IEP means a written statement for each child with a disability that is developed, reviewed, and revised in a meeting including school staff and parents. Each IEP must include statements of the child’s present levels of academic achievement and functional performance, measurable annual goals, how the child’s progress toward meeting the annual goals will be measured, the special education and related services and supplementary aids and services to be provided, and any individually appropriate accommodations necessary to measure the academic achievement and functional performance of the child on state and district-wide assessments. Phonological Awareness An individual’s awareness of and access to the sound structure of his or her oral language. Overlaps with or is sometimes used synonymously with phonemic awareness.

References Resources for Clinicians Adams, M. J., Foorman, B. R., Lundberg, I., & Beeler, T. (1998). Phonemic awareness in young children: A classroom curriculum. Baltimore, MD: P. H. Brooks Publishing. Blachman, B. A., Ball, E. W., Black, R., & Tangel, D. M. (2000). Road to the code: A phonological awareness program for young children. Baltimore, MD: P. H. Brooks Publishing. Fletcher, J. M., Lyon, G. R., Fuchs, L. S., & Barnes, M. A. (2007). Learning disabilities: From identification to intervention. New York: Guilford Press.

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Harris, K. H., & Graham, S. (2005). Handbook of learning disabilities. The Guilford Press. Pennington, B. F. (1991). Diagnosing learning disorders: A neuropsychological framework. New York: Guilford Press. Spear-Swerling, L., & Sternberg, R. J. (1994). The road not taken: An integrative theoretical model of reading disability. Journal of Learning Disabilities, 27(2), 91–103, 122. United States Department of Education, Office of Special Education Programs. IDEA – Building the legacy of IDEA 2004. Retrieved November 21, 2007, from http://idea.ed.gov/explore/home Wagner, R. K., Torgesen, J. K., & Rashotte, C. A. (1999). Comprehensive test of phonological processing (CTOPP). Austin, TX: Pro-Ed.

Resources for Families International Dyslexia Association, http://www.interdys.org LD OnLine, http://www.ldonline.org/ Shaywitz, S. E. (2003). Overcoming dyslexia: A new and complete science-based program for reading problems at any level. New York: A. A. Knopf.

Chapter 18

Well Compensated But Never Quite Solved: Lingering Dyslexia Robert F. Newby

Tim, whose family owned a bookstore, first came for evaluation in third grade to explain and provide recommendations for his academic struggles, particularly in reading, spelling, written language, and memorization of math facts. He then came for two separate evaluations during middle and high school. The longitudinal information thus available about the progression of his reading problems illustrates a common case of partially remediated word reading disability. At the time of Tim’s first evaluation, his math reasoning, penmanship, and attention in school were inconsistent, but his written expression was remarkably strong. Tim’s second and third evaluations were conducted to update his progress and determine whether ongoing accommodations and/or additional interventions were needed in his high school or college work. His effort and cooperation at all three evaluations were quite sufficient to ensure that the reported results were valid and reliable. Tim’s medical history was basically noncontributory over the years. He was prescribed glasses for nearsightedness since late elementary school, with regular checkups. His social and emotional functioning were positive. Throughout his school career, he attended parochial schools with good learning support services, received extra reading and writing instruction both at and outside of school, took advantage of curriculum accommodations such as oral testing and books on tape, put in the extra academic effort that students with learning disabilities usually need, and used his good social skills to both maintain a satisfying nonacademic life and advocate for himself with teachers at school.

Third Grade Test Results Tim’s first evaluation included intellectual, academic, and selected information processing skills that could be related to his learning struggles (Tables 18.1–18.3). Tim showed average verbal comprehension and high average perceptual organization, two key factors that have appeared recurrently in factor analysis of the J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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Table 18.1 Intellectual Wechsler Intelligence Scale for Children – Revised Full Scale IQ Verbal IQ Information Similarities Arithmetic Vocabulary Comprehension Digit Span Performance IQ Picture Completion Picture Arrangement Block Design Object Assembly Coding a Standard score, (scaled score), [T-score], {z-score}. Table 18.2 Academic achievement Tests Woodcock Reading Mastery Test – Revised Word Attack Letter-Word Identification Passage Comprehension Wide Range Achievement Test – Revised Spelling Woodcock Johnson Psychoeducational Battery – Revised Calculation Applied Problems

Scorea 96

Percentile 39th

(10) (9) (5) (10) (9) (9)

50th 37th 5th 50th 37th 37th

(9) (13) (14) (11) (6)

37th 84th 90th 63rd 10th

Score

Percentile

Grade equivalent

67 70 74

1st 2nd 4th

1.3 2.1 2.1

67

1st

1E

72 98

3rd 43rd

2.2 3.6

Qualitative Reading Inventory Grade Type Words per minute Primer Narrative 52 1 Narrative 36 a Instructional level >90% oral reading accuracy. b Instructional level >70% comprehension.

Accuracya 89% 78%

Comprehensionb 75% 50%

Wechsler scales, in this case the Wechsler Intelligence Scale for Children (Revised) (WISC-R). Math reasoning skills and memory were also average. The deficits in this profile could be grouped into four main areas that illustrated a classic presentation of reading disability or developmental dyslexia, accompanied by some common comorbidities, most of which were fortunately mild or marginal in Tim’s case. First, Tim was very impaired in phonics analysis (word attack or reading of nonwords) and reading word identification. In Pennington’s (1991) scheme, these were primary or core symptoms of his reading disability (Table 18.4). Second, Tim’s associated marked deficits in spelling and reading comprehension were considered as secondary symptoms. Written expression was not assessed in

18 Well Compensated But Never Quite Solved: Lingering Dyslexia Table 18.3 Other cognitive functions Tests Raw score Gordon Diagnostic System Vigilance task Correct/45 38 Commission errors 1 Beery-Buktenica Developmental Test of Visual-Motor Integration Wide Range Assessment of Memory and Learning Story memory Design memory

Score

Percentile

169

Age equivalent

Interpretation

Borderline Normal 86

18th

(8) (12)

25th 75th

7–2

Table 18.4 Tim’s symptoms in Pennington’s (1991) scheme l

Primary: Phonics analysis and reading word identification

l

Secondary: Spelling and reading comprehension

l

Correlated: Math calculations

l

Artifactual: Graphomotor and attention

this set of testing but was reported to be quite satisfactory in his everyday school performance, setting aside factors such as penmanship and spelling. Third, Tim showed correlated symptoms in math calculations, with relatively preserved math reasoning at that time. A number of hypotheses about the possible information processing links between reading disability and difficulties memorizing math facts have been presented in the professional literature, ranging from working memory to processing of symbols, but consensus on a single explanation has not yet emerged. Fourth, Tim was low average or borderline impaired in two areas that could be considered artifactual symptoms, meaning that they appear at an increased prevalence in children with reading disability compared with base rates in the general population but that they do not have compelling direct links with the cognitive systems or underlying neural systems, which cause the primary deficits in reading disability. Mildly reduced graphomotor (paper and pencil) skills were evident in both his copying geometric drawings and his low efficiency in a clerical copying task. He also showed modest attention issues in the latter finding, in his number of items correct on a computerized continuous performance task and in his mental math performance.

Third Grade Formulation and Recommendations Recommendations included a strong emphasis on remediation of core and secondary deficits, along with compensatory approaches to reduce the ongoing impact of these deficits in the broad school curriculum. The main recommendations were

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the same as for Angie in the previous case description, plus the following two strategies. Tim’s reading speed, or automaticity, could be enhanced with repeated reading and/or a set of recently developed instruction methods called retrieval, automaticity, vocabulary elaboration, orthography (RAVE-O). In repeated reading, Tim is asked to read some books, chapters, or stories repeatedly (3–5 times) within a several-day period. He should not be asked to do this with all reading materials—which would turn off anyone to reading! This method should gradually help Tim enter more words into his automatic sight-vocabulary. A variation of repeated reading is to have a parent or peer read orally along with Tim, either with the assisting reader reading all the words at the same pace that Tim reads or with the assisting reader providing only those words that Tim cannot decode immediately himself, to help smooth out the flow of reading. These methods should be done with reading material at Tim’s comfortable instructional level for reading connected text. Tim should make use of audio-taped books in school. This will allow him to listen to textbooks while reading and following along, which can enhance his opportunities to use and remember information in science, social studies, and literature.

Eighth Grade Test Results, Formulation, and Recommendations Only partial reevaluation was needed at the end of middle school, in order to help Tim qualify for continued learning support services in high school (Tables 18.5 and 18.6). Assessment procedures were chosen to update previous areas of concern and to document current academic impairments and associated information processing deficits. Tim showed three types of improvement at this time. The first type, probably representing actual reduction in primary symptoms in response to cumulative effects of treatment, included an increase from around the first percentile to around the 15th percentile in his primary symptoms of phonics analysis and reading word identification. Additional test evidence about the ‘‘layer of language under phonics’’ was available at this time, that is, the Rosner, which was a forebear of the Elision subtest from the Comprehensive Test of Phonological Processing (CTOPP), suggesting that Tim’s phonological awareness was at a similar level as phonics analysis. Reading speed, however, remained slow. In secondary symptoms, on the one hand, Tim demonstrated continuing poor spelling, which comprised most of his errors on the Dictation subtest from the Woodcock-Johnson-R. On the other hand, he was showing some strong compensation in several more cognitively complex areas, also probably in response to treatment. Specifically, his reading comprehension was average or near average on different measures, which was significantly better than his underlying reading decoding skills. He appeared to be using both his verbal reasoning and memory abilities as well as reading strategies that were explicitly taught in tutoring to bootstrap this important end product of the global reading process to a higher

18 Well Compensated But Never Quite Solved: Lingering Dyslexia Table 18.5 Academic achievement Tests

Score

Woodcock Johnson Psychoeducational Battery – Revised Broad reading 90 Letter-word identification 83 Passage comprehension 99 Calculation 75 Applied problems 66 Broad written language 90 Dictation 72 Writing samples 123 Supplemental battery Word attack 85 Qualitative Reading Inventory Grade Type Words per minute 6 Expository 86 7/8 Expository 75 a Instructional level >90% oral reading accuracy. b Instructional level >70% comprehension. Table 18.6 Other cognitive functions Tests Raw score Gordon Diagnostic System Vigilance task Correct/45 42 Commission errors 2 Rosner Test of Auditory Awareness Skills Beery-Buktenica Developmental Test of Visual-Motor Integration Wide Range Assessment of Memory and Learning Story memory Design memory

Score

171

Percentile

Grade equivalent

25th 13th 48th 5th 1st 24th 3rd 94th

5.5 4.3 8.1 4.4 4.0 5.4 3.5 14.9

16th

3.3

Accuracya 95% 93%

Percentile

Comprehensionb 75% 60%

Age equivalent

Interpretation

Normal Normal 87

19th

105

66th

(11) (13)

63rd 84th

14–15

level than would have been predicted on the basis of his elemental word reading capacity at that time. Although written expression had not been assessed in the earlier testing, precluding a direct comparison across time in this area, he demonstrated well above the average skill, at least at the sentence composition level in a testing format that did not penalize for errors in more basic areas such as spelling. Tim’s third type of improvement probably represented fluctuation in artifactual symptoms over time, rather than true gain in underlying skills: Testing of both graphomotor skills and attention was in the average range. As an aside to his primary reading disability, Tim showed very little growth in math reasoning since his third grade evaluation. On close examination, two factors

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seemed responsible for this disappointment. His errors in applied math problems during the testing were almost always due to calculation mistakes, illustrating his continuing delay in memorizing math facts. In addition, his school had not accommodated his reading difficulties in the math curriculum, for instance, by providing oral presentation of math word problems, which artificially limited his cumulative learning and practice in the latter area. This error was corrected with remedial applied instruction in high school. The most important recommendation for reading and written language during Tim’s high school years was that Tim should continue to make use of audio-taped books in school.

Eleventh Grade Test Results Tim’s final evaluation (Tables 18.7 and 18.8) had the sole purpose of providing documentation for similar services and accommodations at college as he had received earlier. Although some organizations, such as college entrance examination services, specify more extensive domains to be tested for documentation of learning disability (usually including at least intelligence, academic skills, and relevant information processing areas), Tim had scored well enough for admission to his first-choice college on his initial ACT under standard testing conditions, so some of the more detailed testing that usually would have been required for this particular purpose turned out to be not necessary, and the college itself was flexible in considering the history of his previous evaluations with a brief update.

Table 18.7 Academic achievement Woodcock Johnson Psychoeducational Battery – Revised Broad reading Letter-word identification Passage comprehension Broad written language Dictation Writing samples Supplemental battery Word attack

Score

Percentile

87 87 97 94 82 121

19th 19th 41st 34th 11th 92nd

79

8th

Table 18.8 Other cognitive functions Wide Range Assessment of Memory and Learning Story memory

Grade equivalent 5.4 10.1 8.6 6.0 16.9 4.3

Score (11)

Percentile 63rd

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Eleventh Grade Formulation and Recommendations Tim’s level and pattern of performance were very similar to the eighth grade evaluation, indicating stability of both deficits and compensations over time. The following strategies were emphasized at this time. Tim should continue receiving extra remediation outside the regular classroom for his learning struggles, particularly advanced reading decoding and reading fluency. Although earlier efforts focused most heavily on reading and language, some more recent attention to math skills may need to be added during his senior year as he finishes high school requirements for graduation. He should continue using compensatory technologies such as a calculator in math (although some further drilling of math facts is needed rather than 100% reliance on a calculator) and a word processor for written language (with particular emphasis on use of a spelling checker). In addition to required reading for school and practice reading for his tutoring outside of school, Tim should read regularly for pleasure, including rereading materials to which he is particularly attracted, to increase reading speed or fluency. Tim should continue to make use of audio-taped books in school. Spelling instruction at this age should be limited to very-high-frequency words actually to build a basic repertoire rather than to attempt to spread Tim too thin with a wide repertoire of known spelling words. Tim probably will require extra time to complete academic requirements in classes with a heavy load of reading or writing. This could be facilitated by arranging for him to take a reduced course load, both during the remainder of high school and at the college level. Shortened assignments at school would be relevant in many subject areas, particularly when extensive reading or writing is required. If Tim is required to take further standardized, group-administered testing in the future, it should be conducted with extended time limits and with auditory presentation (e.g., audiotape and headphones) to accompany all reading material in content areas that are not designed specifically to assess reading comprehension (e.g., social studies). This type of accommodation also would be justified in testing for classes at school, if requested. On testing in which written answers are required, errors in spelling, punctuation, capitalization, and word usage should not be penalized, and oral answers should be allowed or encouraged so Tim can elaborate on his written answers. Tim’s mother appropriately wants to support his self-confidence and self-esteem by helping him understand his dyslexia as fully as possible. Some discussion of this issue was conducted in a follow-up feedback interview on the day of testing with Tim and his parents. Further counseling or information in this area should be made available if he requests.

Update Tim began high school intending to take advantage of a strong instrumental music program there and then to pursue a career in music teaching. Along the way, however, he discovered a love of American literature, in which he majored at a

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small local college, from where he recently graduated. He will take his place as the front desk manager in his family’s bookselling business, having worked various other part-time jobs in their stores throughout his high school and college years. Imagine – a student with dyslexia running a bookstore!

Additional Resources Key Concepts and Terms Phonological Awareness An individual’s awareness of and access to the sound structure of his or her oral language. Overlaps with or is sometimes used synonymously with phonemic awareness.

References Resources for Clinicians Adams, M. J., Foorman, B. R., Lundberg, I., & Beeler, T. (1998). Phonemic awareness in young children: A classroom curriculum. Baltimore, MD: P. H. Brooks Publishing. Blachman, B. A., Ball, E. W., Black, R., & Tangel, D. M. (2000). Road to the code: A phonological awareness program for young children. Baltimore, MD: P. H. Brooks Publishing. Fletcher, J. M., Lyon, G. R., Fuchs, L. S., & Barnes, M. A. (2007). Learning disabilities: From identification to intervention. New York: Guilford Press. Harris, K. H., & Graham, S. (2005). Handbook of learning disabilities. New York: Guilford Press. Pennington, B. F. (1991). Diagnosing learning disorders: A neuropsychological framework. New York: Guilford Press. Spear-Swerling, L., & Sternberg, R. J. (1994). The road not taken: An integrative theoretical model of reading disability. Journal of Learning Disabilities, 27(2), 91–103, 122. The Center for Reading and Language Research, Tufts University. The RAVE-O program: A comprehensive, fluency-based reading intervention program. Retrieved November 21, 2007, from http://www.ase.tufts.edu/crlr/raveo.html. United States Department of Education, Office of Special Education Programs. IDEA – building the legacy of IDEA 2004. Retrieved November 21, 2007, from http://idea.ed.gov/explore/ home. Wagner, R. K., Torgesen, J. K., & Rashotte, C. A. (1999). Comprehensive test of phonological processing (CTOPP). Austin, TX: Pro-Ed.

Resources for Families International Dyslexia Association, http://www.interdys.org. LD OnLine, http://www.ldonline.org/. Recording for the Blind & Dyslexic, http://www.rfbd.org/. Shaywitz, S. E. (2003). Overcoming dyslexia: A new and complete science-based program for reading problems at any level. New York: A. A. Knopf.

Chapter 19

Emily Confronts Her Fiercest Bear: Word Reading Disorder with Naming Speed and Phonological Deficits Molly Darke Shiffler

The winding road leading through the forest grew narrower and darker until it tapered into a two-track lane where gravel and horseshoe prints dotted the slippery surface of compacted snow. The reading specialist was feeling almost lost in that dark subzero landscape when the lights of a small enclave shone through the thick woodland. Set in a ring encircling an oval-shaped forest clearing, the windows of the hand-built fieldstone homes glowed with the serenity of an illustration from Van Allsburg’s Polar Express. Centered in the clearing stood a snow-patched barn, a maze of hound dog runs, sleigh-ride facilities, and a shed reserved for bear hunting gear—mainstays of Emily’s family’s lifestyle for three generations. That December night, thankfully, the local bears did not make one of their routine visits. The reading teacher was visiting Emily’s home because her slow reading pace, labored decoding, and sharp contrast between listening comprehension and reading comprehension echoed the reading patterns of an older generation living in those softly lit houses who had been students in the school district’s first Title I elementary reading program 30 years ago. Informal assessment at school indicating slow reading rates (16 words per min) and weak phonological segmenting skills had suggested that Emily was at risk for double-deficit dyslexia, a form of reading disorder as formidable as any bear. Her parents described her as an inquisitive, bright child who struggled with reading, math facts, and writing, especially noting reversed words in reading and letters in writing. Her birth and developmental history were not significant for illnesses, injuries, or serious medical conditions. Milestones included speaking single words at 8–9 months, composing sentences at 1 year, and walking at 10 months. Family history included dyslexia for Emily’s father and his two brothers, although her father felt that his reading difficulty resulted from lack of effort and his own father’s habit of withdrawing his sons from school to help in the woods and fields. Kindergarten screening with Emily had shown difficulty copying basic shapes, printing her name, and articulating a few speech sounds—/t/, /sh/, /l/, and /ch/. Her kindergarten teacher reported that, although Emily could recognize and

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produce rhyming words, the retention and recall of letter names and sounds were difficult for her, and that Emily was not using sound-symbol correspondence in her developmental writing at the end of kindergarten. Emily was placed in the kindergarten and first grade Title I programs, received extra help from her teachers at lunch time, and participated in a literacy mentoring program staffed by community volunteers. It became evident that Emily’s extra literacy support was not enough to ensure proficient reading. Neither her sight vocabulary nor phonic skills were automatic, although she was somewhat faster at sight words than at pseudowords. In the middle of first grade, Emily qualified for learning disabilities programming. Although her standard achievement scores appeared to be in an average range, the placement was based on the discrepancy between achievement scores and her overall intellectual functioning in a superior range. Emily began receiving reading and writing intervention approximately 8 hours per week in a resource room setting that included a phonological analysis and blending program and a structured phonological encoding program with writing exercises. She was felt to have good long-term memory but tended to retrieve information slowly. She tended to be fidgety and was apt to play with items in her hands or mouth. Connors’ Rating Scales-Revised, completed by parents and teachers, indicated scores in the risk-significant range (T-scores above 70) on inattention, hyperactivity, and cognitive indexes. Emily was placed on methylphenidate for attention deficit hyperactivity disorder (ADHD). Despite this combination of treatments, her reading progressed very slowly One promising approach for students like Emily who have not responded to phonological interventions derives from a growing body of research based on double-deficit hypothesis. As currently conceptualized, double-deficit dyslexia encompasses both phonological deficits and naming-speed deficits, which impede rapid recognition of visually presented verbal stimuli. The proposed double-deficit hypothesis represents a substantial paradigm shift in understanding severe reading disabilities. At the present time, there is fairly widespread agreement that phonological deficits (difficulty with linguistic operations such as blending, identification, segmenting, retrieval, and memory on the basis of the sounds of speech) play a major causative role in reading disabilities. Wolf, Bowers, and Biddle (2000), however, have proposed that phonological deficits alone do not account adequately for all reading disabilities and that a separate naming-speed deficit (in the processes underlying the rapid recognition and retrieval of visually presented verbal stimuli) represent a separate core deficit in readers with specific reading disabilities. Hence, the term double-deficit hypothesis refers to phonological deficits and naming-speed deficits as independent but not exclusive processes impacting the reading of students with word reading disorders. In other words, struggling readers might exhibit a phonological deficit, a naming-speed deficit, or a double-deficit composed of both phonological and naming-speed deficiencies. The impact of naming-speed deficits on reading disabilities can remain pervasive even into the adult years; in fact, several studies measuring factors associated

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with word reading disorders found naming-speed deficit to be the single variable differentiating adults with reading disabilities from control groups. Reading practitioners often encounter high school students referred for diagnostic reading testing with reading rates of 20–40 words per min below standard fluency norms, below average scores on comprehension measures, and history of difficulty learning letter names and sight words.

Test Results Both processing deficits and achievement deficits were measured, and more than one test measured each construct to sustain construct validity (Fig. 19.1). Since the intervention program in Emily’s district was designed to address phonological, naming speed, or both deficits, a core group of assessments was specified to determine severity of deficit both at processing and single word application levels. The assessment measures and criteria for determining placement in phonological and naming-speed subtype groups are diagrammed in Fig. 19.2, which shows that multiple measures were also used to assess naming-speed processing. On the WISC-III administered at age 6 years and 11 months by the district’s elementary psychologist, Emily obtained a Full Scale IQ of 121, which placed her intellectual functioning in the Superior range. Comparison of Verbal (VIQ 114) vs. Performance (PIQ 123) scores indicated that Emily did not differ significantly on tasks requiring verbal ability and nonverbal ability (Table 19.1). Emily’s ability to rapidly name the series of alphanumeric symbols (numbers and letters) on the CTOPP rapid naming subtests and the single words on both timed TOWRE subtests was extremely compromised; these scores were significantly disparate from her WISC-III scores in the Superior classification (Table 19.2). In addition, her reading rate on a familiar grade 2 narrative passage on the QRI-3

Assessment Construct Clusters

Phonological Processing/ Application Measures

Naming-Speed Processing/ Application Measures

Word Reading Measures

Decoding Measures

Comprehension Measures

CTOPP1 Elision CTOPP Blending TOWRE3 PDE WRMT-R WA

CTOPP RDN CTOPP RLN RAN/RAS2 TOWRE SWE

WRMT-R WI TOWRE SWE WRAT-3 Rdg.

WRMT-R WA TOWRE PDE WRAT-3 Splg. % GFE's

WRMT-R Passage Comp. QRI-3 Rdg. & Listening Comp.

Fig. 19.1 Testing grouped by assessment construct clusters

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Word Recognition Deficit Determination Tests and Criteria

Phonological Processing Measures

Naming-Speed Processing Measures

CTOPP 1 Elision <25%ile CTOPP Blending <25%ile TOWRE 2 Phonics Decoding Efficiency <85SS WRMT-R Word Attack <85SS

CTOPP Rapid Digit Naming <25%ile CTOPP Rapid Letter Naming <25%ile RAN/RAS3 Rapid Automatized Naming <85SS TOWRE Sight Word Efficiency <85SS

Fig. 19.2 Processing and achievement tests and criteria used for deficit determination

Table 19.1 Intellectual Wechsler Intelligence Scale for Children – Third Edition Full scale IQ Verbal IQ Information Similarities Arithmetic Vocabulary Comprehension Digit span Performance IQ Picture completion Picture arrangement Block design Object assembly Symbol search Mazes a Standard score, (scaled score), [T-score], {z-score}.

Scorea 121 114 (12) (10) (13) (14) (13) (10) 123 (14) (18) (11) (10) (14) (12)

Percentile 92nd 75th 50th 84th 90th 84th 50th 90th 99th 63rd 50th 90th 75th

was at 16 words per min correct—well within frustration level (Table 19.3). According to miscue analysis on the QRI-3, Emily had begun to develop some basic phonological decoding skills, including consonant-vowel-consonant patterns and consonant digraphs (-sh, -ch), although, even on two and three-phoneme words, she blended words very slowly. She made neither long and short vowel generalizations nor could she consistently elicit both sounds when reading a two-letter consonant blend (pl-); even as a second grader, she still had difficulty correctly decoding final consonants on three-letter words.

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Table 19.2 Other cognitive functions Tests Comprehensive Test of Phonological Processing Phonological awareness composite Elision Blending Rapid naming composite Rapid digit naming Rapid letter naming Test of Word Reading Efficiency Sight word efficiency Phonological decoding efficiency Table 19.3 Academic achievement Tests Woodcock Reading Mastery Test – Revised Word identification Word attack Passage comprehension Wide Range Achievement Test – Third Revision Spelling Reading Qualitative Reading Inventory Reading comprehension Listening comprehension Grade 2 passage Test of Written Language – Third Edition

Score

Percentile 42nd 25th 63rd 5th 9th 9th

81 82

Raw score

Score Percentile 84 90 91

14th 25th 27th

100 82

50th 12th

100

50th

16 words per minute correct 1/8 correct 8/8 correct 52/70

When formally measured on the WRMT-R Word Attack subtest with its lenient 5-s-per-word time allowance, Emily’s decoding skills appeared to be in the average range (although significantly disparate from her IQ); however, when a timing component was added to a nonsense-word reading task, her deficits in phonological decoding were much more apparent. Measures of word reading were more depressed than the decoding testing cited. In fact, standard scores achieved on the three formal word reading measures were within 3 points of each other; there was no difference between the TOWRE’s stringently timed Sight Word Efficiency and Phonemic Decoding Efficiency subtests. The WRMT-Passage Comprehension subtest consists of phrases, sentences, and paragraphs in which one word is missing; the student is to read the connected text and supply the missing word. Once again, although Emily’s standard score of 91 appeared to be in the average range, comparison with her Full Scale IQ of 121 revealed a discrepancy of 2 standard deviations. The QRI-3 gave a profile of Emily’s functional reading achievement that closely resembled the pattern her classroom teachers described. On a second-grade familiar narrative passage, Emily was able to read at only 16 words per min (frustration level) and had an

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accuracy rate of 74% (frustration level). Comparing Emily’s listening and reading comprehension levels using the familiar narrative grade 2 passages from the QRI-3 demonstrated the sharp contrast between her cognitive and word recognition abilities. On the QRI-3 passage, which Emily read aloud, she answered one of eight questions correctly. In contrast, when the examiner read a passage of corresponding length and difficulty to her, she was able to obtain a perfect score by answering all eight questions correctly.

Formulation and Recommendations Emily’s decoding, word identification, and alphanumeric naming automaticity were strikingly disparate from her superior general cognitive skills. Although her phonological processing and untimed decoding achievement scores appeared to be in an average range, they were, in fact, significantly discrepant when juxtaposed with her intellectual functioning that was in the superior range. Since Emily had met regression-based aptitude/achievement discrepancy criteria when she entered the district’s learning disability program prior to Double Support project testing, it was not deemed necessary to determine whether she qualified as reading disabled at the time of the specialized additional assessment described in the previous section. At first viewing, Emily’s CTOPP Phonological Awareness composite quotient at 97 and 42nd percentile appeared too high to place her in the double-deficit subtype group. After consultation, however, the reading specialist determined that a double-deficit placement was justified when the following factors were considered: (a) the discrepancy between CTOPP Elision score at the 25th percentile, which met program criteria, and Blending score at the 63rd percentile had been obscured when the two subtests were averaged to form the Phonological Composite score (therefore, Emily qualified as phonologically-deficit according to program criteria of 25th percentile or lower on either a single CTOPP subtest or composite score); (b) the disparity, exceeding two standard deviations, between Emily’s TOWRE Phonemic Decoding Efficiency score (82SS) and her WISC-III Full Scale IQ at 121; (c) the 31 point discrepancy between her WRMT-R Word Attack at 90SS and her FSIQ; and (d) comments from kindergarten and first grade teachers noting Emily’s marked difficulty with phoneme-grapheme correspondence, phonological decoding and encoding, and reading pseudowords. Returning to double deficit criteria as displayed in Fig. 19.2, school and clinical staff determined that Emily indeed had both phonological and naming speed deficits because she met the following criteria: (a) achievement scores, as represented by her WRMT-R Word Identification and Word Attack standard scores, at least one standard deviation discrepant from her Superior range WISC-III scores; (b) her WISC-III Full Scale score was well above 90SS indicating Superior intellectual abilities; (c) a phonological deficit as indicated by CTOPP Elision

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and/or Blending scores at or below the 25th percentile; and (d) a naming-speed deficit as indicated by either RAN/RAS or CTOPP Rapid Digit Naming and/or Rapid Letter Naming scores at or below the 25th percentile. According to these data, combined with comments from her teachers noting marked difficulty on phonological tasks, Emily qualified for the Double Support intervention as having both naming speed and phonological deficits. As is typical of individuals with the proposed double-deficit, Emily’s primary learning difficulty in word reading disability involved recognizing or decoding words fluently in reading but secondary difficulties, aggregated by decreased print exposure, include a. Spelling because the orthographic/phonemic consolidation develops more slowly b. Reading comprehension because of attentional resources directed to decoding and more difficulty storing information acquired at a slower rate c. Written expression because of reduced exposure to orthography patterns and print syntax. Consistently, individuals with naming-speed and double deficit reading disabilities show reduced speed in reading symbols (letters and numbers) and/or in naming commonly recognized items (colors and pictures of objects) and slowed reading speed. The rapid naming subtests of the CTOPP are often used to determine reduced naming speed, while the alphanumeric subtests do not include such easily confused orthographic symbols as b and d or p. For that reason, if a child appears to have other naming characteristics but the CTOPP rapid naming subtests are not markedly depressed, it may be advisable to administer the Rapid Automatized Naming/Rapid Alternating Stimuli (RAN/RAS), which does contain frequently miscued alphanumeric symbols, as a second measure. When considering a possible naming speed deficit, it is always important to rule out any environmental factors that may have impacted naming fluency such as limited opportunity for reading practice, chronic inappropriate (usually too difficult) texts, linguistic or dialectic code-switching, more pervasive cognitive conditions, or limited visual acuity. Research has not shown a link between naming speed and popular dyslexia explanations such as ‘‘eye muscle convergence,’’ ‘‘eye tracking,’’ color-sensitive filters, or readjusting visual perspective. Characteristic reading patterns of phonological, naming speed, and double deficit word recognition disorders observed by clinicians or literacy professionals vary in several aspects as displayed on Table 19.4. Reading practitioners note that students with phonological but not naming speed deficits, a literacy profile discussed in previous chapters, tend to be relatively fast but inaccurate readers. Unable to depend on decoding skills, they manage to use well-developed context-based word recognition strategies to get the overall meaning of their reading; they often surprise others with adequate comprehension in spite of numerous errors. On the one hand, semantic substitutions, such as father for dad, that have no phonetic resemblance

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Table 19.4 Patterns of skills of deficit groups assessed in grade 3 Naming speed deficit Phoneme aware deficit Double deficit Word identification and attack Word identification and attack Word identification and relatively poor attack very poor okay but less than nodeficit group Slower reading Faster reading Slowest reading Poor spelling, especially Poor spelling, both dictation Very poor spelling, both spelling recognition and recognition dictation and recognition Poor orthographic [accuracy] Better or similarly poor Poor orthographic skill skill orthographic skill % [of students] below 25th % [of students] below 25th % [of students] below 25th percentile on word percentile on word percentile on word identification: 90% identification: 20–30% identification: 20–30% From Dyslexia, Fluency, and the Brain (p. 44) by M. Wolf (Ed.), 2001, Timonium, MD: York Press. Copyright 2001 by York Press.

but have similar meaning, frequently punctuate the oral reading of individuals with phonological deficits. On the other hand, readers with naming speed deficits appear to be consistently accurate but slow readers, dependent on sound-by-sound decoding well-beyond the usual early elementary years; comprehension is sometimes compromised. If the reader also has attentional issues, the rate may not be as slow but numerous tracking errors are often evident. In the case of students with the bifurcated deficit pattern, labored or absent decoding attempts, very slow rates, and poor comprehension are all generally present. For students like Emily who have double reading deficits, both phonological and naming-speed intervention should be addressed in any recommendations. The naming-speed reading model proposes that processing speed may be inadequate at one or more sequential levels (visual features of individual symbols; grapheme recognition; grapheme/phoneme assignment; visual, auditory, and semantic lexical access; syntactic integration; comprehension; and articulation). At the present time, only the RAVE-O intervention is specifically designed to address each of the levels in the proposed naming speed sequence. This early elementary level (grades 2–3) reading intervention connects phonemic analysis and blending skills with fluency training at orthographic pattern, word, and sentence level incorporating adequate exposure to frequently used sublexical units (examples, re-, sh-, -ip, -ake) for increased word recognition fluency. RAVE-O addresses three equivalent goals: a. Increased achievement in overt word identification, decoding, and comprehension b. Automaticity in underlying phonological, orthographic, semantic development, sublexical, and lexical processes c. Improved self-efficacy through metacognitive use of decoding and retrieval strategies The program consists of about 30-min sessions for 16 weeks, 4-days per week. A program for older students is being developed but not yet available.

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Specifically addressing Emily’s core phonological and naming-speed deficits, school and clinical staff recommended Emily’s participation in the district’s Double Support project, which included a naming speed intervention and phonological intervention, the basic curriculum in her learning disability pull-out program. In addition, a paraprofessional guided her through a computer-based intense automatic high frequency word recognition program.

Recommendations Targeting Specific Layers of Naming Speed Sublexical/Word Part Level Automaticity: According to naming speed constructs, the automatic recognition of word parts increases students’ reading rate. Therefore, both analytic and synthetic phonic instruction (see previous chapter for discussion) are recommended to enhance reading proficiency. The parallel phonological intervention, however, is the primary source for synthetic word recognition instruction. In either approach, the metacognitive strategy of constructing and identifying word parts must be stressed. In younger readers, sound-by-sound accuracy surpasses any emphasis on rate, so it is appropriate to encourage single phoneme decoding as a word recognition strategy. However, since an overdependence on sound-by-sound word attack is typical of older naming speed readers, practitioners should proactively coach students who are accurate but slow to use decoding-by-analogy, a metacognitive strategy for recognizing words by comparing them to known words. For, example, when pronouncing the word O/bama by syllables, a student might say, ‘‘If g-o is go, then o must be /o¯/; if m-a-m-a is mama, then b-a-m-a must be bama.’’ Commonly, synthetic phonics instruction stressing sublexical units includes explicit instruction with visual mnemonic cues for common vowel/syllable patterns, building and blending words sound-by-sound then part-by-part with letter tiles, and making word activities stressing changing orthographic patterns. Analytic phonics, by definition, emphasizes automatic identification of common phonograms or word families (examples: -and, -ack, -ump, -ight) within words being read through explicit instruction, wall charts (or personal folders), or phonogram towers, manipulations of common multiletter graphemes, and word sorts. Sublexical/Word Part Level Automaticity: Recent research by Blachman (see References) suggests that word pattern review cards may also increase the automatic recognition of sublexical units. These are simply note cards with previously taught patterns on one side, such as the vowel diphthong _ai_; the underlines indicate that the pattern is in the middle of a word, and a clue word or picture on the reverse side, for example, _ai_, rain and a picture of raindrops. The verbal cue is ‘‘What do a and i say together in the middle of a word or syllable?’’ Until the pattern is secure, the clued ‘‘teaching side’’ is flashed;

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once the student recognizes the pattern, the pattern-only side is used for review and automaticity. Review cards could be used in recommendations for parents, classroom, or supplementary intervention. Sublexical automaticity through analytic phonics and phonograms was a foundational component of Emily’s RAVE-O naming speed intervention, while synthetic phonics was the instructional core of her parallel phonological intervention.

Word and Sentence Level Fluency: Students with naming speed deficits appear to have more difficulty learning the irregular orthography of many high frequency words. High frequency word lists like those by Sitton, Frye, or Dolch rank such words in order of their occurrence in print. A multistep learning technique to improve lexical retention and retrieval for each word might include visual presentation, briefly discussing any phonetically regular or distinctive orthographic or semantic features, spelling by chant, writing while verbalizing the writing strokes, visualizing, and recording on an index card in a memorable personal sentence. A high frequency review box with categories for words at automatic (within 1 s) recognition, approaching automatic, and introductory levels may also be used in multiple settings. Emily received intense high frequency word reinforcement through a computer or paper high-frequency word program administered by a paraprofessional.

Word and Sentence Level Fluency/Semantic Development: Easily the most recommended form of sentence level fluency intervention, repeated reading may be done in a variety of formats depending, somewhat, on the age and reading level of the student. One of the most intense interventions, Repeated Oral Assisted Reading (ROAR) developed by Jane Flynn Anderson (unpublished), consists of the following steps as the student and assisting reader sit side by side: a. The assisting reader reads a sentence or two while running his finger across the top of the print as the student runs her finger under the print (the finger tracking continues through all steps) b. Both readers read the same sentence or two together with the assisting reader establishing the rate and prosody (expression, intonation, phrasing) c. The student reads the same sentence(s) on his own d. At the end of a paragraph and page, the student reads those sections independently Text at the high end of the student’s instructional range (between 3 and 10 miscues per 100 words) should be used; four 15-min sessions per week are ideal, less time has minimal impact and more time gets very tedious. Often this method works well for class texts. To ensure Emily had fluency practice at word and sentence level, a high school student came to do ROAR with Emily three times a week for 20 min. ROAR also expands the student’s exposure to high frequency

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words, builds oral vocabulary for faster lexical retrieval, expands syntax, and serves as strong modeled fluency reading, an important component in fluency instruction.

Word and Sentence Fluency/Semantic Development: Listening to tapes while reading along has also been shown to increase the fluency of struggling readers even through adulthood. This serves several purposes, including accessing recreational content material at any level without energy being diverted to word recognition; markedly expanding the semantic exposure at vocabulary, syntactic, and schema levels; and actually increasing the silent and oral reading fluency of the listener. In Emily’s case, the school had tape recordings of all literature books used in the curriculum available for student checkout. Tapes or CDs also make it possible for all students to be familiar with grade level trade books being discussed in class. Many schools and public libraries have a collection of tapes or CDs for class texts and recreational reading, respectively.

Word and Sentence Fluency/Semantic Development: No matter what intervention is used for students with naming speed word recognition deficits, 10–30 min per day of independent level reading, in addition to school texts and remediation programs, is essential for building fluency. The recommended minutes and method may vary depending on the age of the student. The high school student who did repeated readings with Emily also guided her selection and oral reading of independent level self-chosen books.

Semantic Development: Oral vocabulary is a critical link to both decoding and comprehension. Readers recognize words eight times more quickly when they know the word’s meaning; likewise, one of the key determinants in comprehension proficiency is readers’ receptive knowledge of print vocabulary, or degree of semantic lexical access. The RAVE-O design incorporates semantic elaboration because of its importance in the naming speed process, while most commercial or even teacher-designed fluency interventions do not. For this reason, at least one recommendation should address lexical access. Again, options vary by student situation but recommendations should include development of ‘‘word consciousness’’ through collection of M & M (multiple meaning) words, adults reading aloud texts about 4 years ahead of grade level, vocabulary expansion programs, as well as a supporting framework of interactive conversations and broad experiences. Sometimes expanding semantic resources is most effectively accomplished in a group situation.

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Additional Resources Key Concepts and Terms Double-Deficit Dyslexia A specific reading disability composed of phonological and naming-speed deficits. Dyslexia/Reading Disabilities A specific learning disability that is neurological in origin. It is characterized by difficulties with accurate and/or fluent word recognition and by poor spelling and decoding abilities. These difficulties typically result from a deficit in the phonological component of language that is often unexpected in relation to other cognitive abilities and the provision of effective classroom instruction. Secondary consequences may include problems in reading comprehension and reduced reading experience that can impede growth of vocabulary and background knowledge. Adopted by the International Dyslexia Association IDA Board of Directors, Nov. 12, 2002. This Definition is also used by the National Institute of Child Health and Human Development (NICHD). Fluency Wolf’s inclusive working definition (Wolf and Katzir-Cohen, 2001, see themed issue of Scientific Study of Reading in resources) frames fluency as the efficient acquisition of the naming speed processes stressed in RAVE-O and decidedly essential to proficient reading: In its beginnings, reading fluency is the product of the initial development of accuracy and the subsequent development of automaticity in underlying sublexical processes, lexical processes, and their integration in single-word reading and connected text. These include perceptual, phonological, orthographic, and morphological processes at the letter, letter pattern, and word levels, as well as semantic and syntactic processes at the word level and connected-text level. After it is fully developed, reading fluency refers to a level of accuracy and rate where decoding is relatively effortless; where oral reading is smooth and accurate with correct prosody; and where attention can be allocated to comprehension. (p. 219)

Grapheme The minimal unit in a writing system making a difference (usually a letter). Naming-Speed Deficits Deficits in the processes underlying the rapid recognition and retrieval of visually presented verbal stimuli. Phoneme The smallest unit of sound that can make a difference in a word. Phonemic awareness is awareness and manipulation of phonemes (individual sounds) in print and speech. Phonemic segmentation is segmentation of spoken or written word into phonemes. Phonological Awareness The ability to identify and manipulate spoken sound units. Phonological Deficits Difficulty with linguistic operations such as blending, identification, segmenting, retrieval, and memory that are based on the sounds of speech.

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Prosody Differences in pitch, loudness, expression, tempo, phrasing, and rhythm in oral reading. Sublexical Lexical units are word-level units of meaning; the lexicon of a language is its vocabulary. Sublexical refers to units such as rimes and affixes that are smaller than a full word.

References Resources for Clinicians and Families Both the Journal of Learning Disabilities (July/August 2000, Volume 33, Number 4) and Scientific Studies of Reading (2001, Volume 5, Number 5) published theme issues addressing doubledeficit hypothesis, naming-speed, and related fluency issues. Blachman, B. A., Schatschneider, C., Fletcher, J. M., Francis, D. J., Clonan, S. M., Shaywitz, S., et al. (2004). Effects of intensive reading remediation for second and third graders and a 1-year follow-up. Journal of Educational Psychology, 96, 444–461. Fletcher, J. M., Lyon, G. R., Fuchs, L. S., & Barnes, M. A. (2007). Learning disabilities: From identification to intervention. New York: Guilford Press. Morris, R., Lovett, M., & Wolf, M. (In press). Two upcoming articles reporting results of large population study on phonological and naming speed interventions. Journal of Educational Psychology (in press). Recording for the Blind & Dyslexic. Retrieved November 21, 2007, from http://www.rfbd.org/. The Center for Reading and Language Research, Tufts University. The RAVE-O program: A comprehensive, fluency-based reading intervention program. Retrieved November 21, 2007, from http://www.ase.tufts.edu/crlr/raveo.html. Winn, B. D., Skinner, C. H., Oliver, R., Hale, A. D., & Ziegler, M. (2006). The effects of listening while reading and repeated reading on the reading fluency of adult learners. Journal of Adolescent and Adult Literacy, 50(3), 196–205. Wolf, M. (2007). Proust and the squid: The story and science of the reading brain. New York: HarperCollins. Wolf, M., & Denckla, M. (2005). Rapid Automatized Naming and Rapid Alternating Stimulus Tests. Austin, TX: Pro-Ed. Wolf, M., & Katzir-Cohen, T. (2001). Reading fluency and its intervention. Scientific Studies of Reading, 5(3), 211–238. Wolf, M., Bowers, P. G., & Biddle, K. (2000). Naming-speed processes, timing, and reading: A conceptual review. Journal of Learning Disabilities, 33(4), 387–407.

Naming Speed/Fluency and Phonological Interventions Although RAVE-O is the only comprehensive intervention targeting all levels of the naming speed process currently available, this chapter’s resource list also describes published fluency and phonological interventions that address namingspeed, double deficit, and phonological word reading disorders. Fluency programs listed generally address automaticity and accuracy at word and sentence level but not at word part or semantic levels, which should be considered for students with

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naming speed deficits. Because of cost and training required, some programs are limited to school district, university literacy center, and professional tutoring services (Fluency: RAVE-O, Read-It; Phonological Decoding: Wilson, Spell Read/ P.A.T., Language!). Others are more appropriate for less intense individual or small group intervention situations (Fluency: Read Naturally, Great Leaps, QuickReads; Phonological Decoding: REWARDS). Most programs contain some overlap between phonological and fluency remediation elements.

Benchmark Word Identification/Vocabulary and Word Detective Programs: Elementary intervention stressing building words from orthographic patterns for students with basic phonemic knowledge (http://www.benchmarkschool.org). Decoding by analogy, an analytic metacognitive strategy, taught with numerous common spelling patterns forms the core of the Benchmark Word Detective program by Irene Gaskin developed for students with severe word recognition disorders. Although time consuming, Benchmark offers a very thorough analytical approach to sublexical automaticity.

Systematic Sequential Phonics They Use (Cunningham): Presents making word activities introducing word patterns that become progressively more difficult at a logical, consistent pace for striving readers. Also appropriate for a private tutor or in most home situations. Edmark Reading Program, http://www.riverdeep.net/pls/portal/url/page/RVDP_PO. Computerbased, intense automatic high frequency word recognition program. Great Leaps Reading, http://www.greatleaps.com. Kindergarten through high school fluency intervention. Language! A Literacy Intervention Curriculum, http://www.language-usa.net. Comprehensive program for struggling readers grades 4 and above QuickReads, http://www.pearsonatschool.com. Fluency program with CD for grades 2–5. RAVE-O, http://www.ase.tufts.edu/crlr/rave-o.html. Multilayered fluency intervention program for early elementary grades addressing all levels of naming-speed model. Reading Mastery (Science Research Associates), https://www.sraonline.com. Basic curriculum in a learning disability pull-out program. ReadIt, http://www.reading-assistant.com. Electronic fluency program developed by Marilyn Adams for grades 2–5. Read Naturally, http://www.readnaturally.com. Self-monitored CD fluency program for grades 1–7. REWARDS, http://www.sopriswest.com. Word attack and rate development program for elementary grades and above. Spell Read P.A.T. (Phonemic Analysis Training, www.spellread.com Phonological/decoding/ spelling intervention for ages 5 years to adult. Text Talk http://teacher.scholastic.com/products/texttalk/. Isabelle Beck’s outstanding vocabulary building program embeds both lexical and syntactic development in age-appropriate literature conversations. Students with low to below average semantic knowledge scores in one university’s urban literacy center responded enthusiastically to Text Talk, and it was incorporated

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into both the after-school program many of the students attended as well as the kindergarten classrooms in low income schools that other students attended. Wilson Reading System, http://www.wilsonlanguage.com. Elementary to adult phonological/ decoding/spelling intervention based on Orton Gillingham.

Resources for Families Hall, S., & Moats, L. (1998). Straight talk about reading. New York: NTC Press. National Institute of Child Health and Human Development, http://www.nichd.nih.gov. Includes summaries of neurological studies. National Joint Committee on Learning Disabilities, Finding Common Ground, http://www.ld.org/ advocacy/Ldroundtable.cfm Words Their Way (4th Ed.) and the related series of reading stage-appropriate sort books by Bear, Invernizzi, Johnston, and Templeton builds general analytic phonic skills and would be valuable as a home or supplementary program component. See also Resources for Families in previous chapter.

Chapter 20

A Tale of Two Assessments: Reading Fluency Kara Lindstedt and Michael J. Zaccariello

It seemed Ethan had it all: he was popular among his peers, renowned for his athletic prowess, and the son of educated and well-established parents residing in the upper echelon of a Midwestern community. Further, he was an 11th grader at an esteemed private school known for its academic rigor and for producing future doctors, lawyers, and philosophers. Yet, there was much more to Ethan than met the eye. Academically, he struggled to keep up with class assignments and to meet the expectations of his high-achieving parents. He had to jump many hurdles to meet the lofty goals that were the norm in his everyday world, and this weighed heavily on him. Ethan had difficulty with reading since grade school. He found reading extremely challenging and laborious, and he was unable to keep up with the volume of reading that was expected in his classes. Not only did the demands of in-class tasks produce obstacles for him, but homework was particularly frustrating—at times he would give up. Ethan had begun to develop what psychologists call ‘‘learned helplessness.’’ He would not attempt assignments, feeling as if no matter how well or hard he worked, he would inevitably fail. In contrast, he tended to function with ease in courses that had less reading. Beyond Ethan’s difficulties with reading, he found it difficult to sustain his focus and concentration. He also felt that it took him longer than his friends to plan or organize various school assignments. Finally, he felt that he had problems recalling things he read. As an upperclassman with hopes of attending a university, Ethan, like his peers, took the college entrance exams. Though he achieved a score of at least 30 on the math section of the ACT, he attained only a 19 in reading. In spite of his reading struggles, Ethan had managed to achieve average to above average grades in all classes. Ethan’s family was aware of his stress and frustration at school. Fireworks ignited whenever his parents initiated conversations about his schoolwork. He had become increasingly reclusive and evaded conversations with family members. Was Ethan a typical adolescent, ‘‘too cool’’ for time with family, or a troubled teenager tired of having to fight to achieve the things that came easily to his friends? Or, was he a teenager not just stressed by high academic, athletic, and social demands, but also a frustrated young man trying to distance himself from fiery verbal altercations in the home that intensified preexisting stressors and demands in other areas of his life?

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Ethan would tell you that his story was that of an adolescent in conflict with his parents, especially his father. It was the story of a teen feeling badgered by parents who did not listen to him, did not ‘‘get’’ him, and did not trust him. Ethan’s story was that of a young man who, at times, wished he would simply disappear and not have to deal with his problems anymore. He was struggling with symptoms of depression and anxiety exacerbated by family stressors and the effects of a reading disability. Ethan attempted to cope by relying on friends, mentors, physical activity, and/or his religious beliefs; however, at times it got to be simply too much. Ethan underwent two separate neuropsychological evaluations, the first as he entered fourth grade and the second as he completed 11th grade. Consequently, these data outline the nature and progression of Ethan’s reading difficulties as he advanced in age and grade. His first evaluation, at age 9 years and 6 months was to outline and ascertain the extent of his reading comprehension problems. The second neuropsychological assessment, conducted when Ethan was 17-years old, was to update his cognitive profile and to determine whether additional accommodations or recommendations were necessary for him to negotiate his rigorous high school curriculum.

Initial Test Results Ethan’s initial neuropsychological evaluation comprised intellectual ability, phonological awareness, overall academic skills development, and an informal reading inventory (Tables 20.1–20.3). His effort and cooperation during testing were adequate to ensure that the results attained were valid and reliable. Ethan showed mild anxiety at times, in the form of modest concern when he was not able to answer a question. He was sociable, fun to work with, and asked intriguing questions about a variety of topics. In general, Ethan was not distractible, impulsive, or restless, but he needed reminders to keep his attention focused at times. Ethan demonstrated superior overall intellectual ability as assessed by the Wechsler Intelligence Scale for Children—Third Edition (WISC-III) with superior verbal comprehension ability and high average nonverbal intellectual ability. The vast majority of Ethan’s cognitive and academic profile was solidly average to well above average. However, there existed one glaring and significant weakness that was the most likely culprit for his reading difficulties. That weakness was in the realm of phonological processing.

Initial Formulation and Recommendations Phonological processing is the method by which sound structure in language (reading, writing, listening, and speaking) is processed (Wagner and Torgesen, 1987). According to Wagner et al. (1999), phonological processing is composed

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Table 20.1 Intelligence – first evaluation Wechsler Intelligence Scale for Children – Third Edition Verbal Comprehension Information Similarities Vocabulary Comprehension Freedom from Distractibility Arithmetic Digit Span Full Scale IQ Perceptual Organization Picture Completion Picture Arrangement Block Design Object Assembly Processing Speed Coding Symbol Search Verbal IQ Performance IQ a Standard score, (scaled score), [T-score], {z-score}.

Scorea 124 (16) (12) (15) (14) 121 (16) (11) 122 116 (14) (11) (15) (10) 111 (8) (16) 127 111

Table 20.2 Academic achievement – first evaluation Tests Score Woodcock Johnson Psychoeducational Battery – Revised Broad reading 115 Letter-word identification 104 Passage comprehension 125 Broad written language 105 Dictation 107 Writing samples 100 Punctuation/capitalization 112 Spelling 104 Usage 121 Broad mathematics 139 Calculation 125 Applied problems 138 Supplemental battery Basic reading skills 103 Word attack 103 Basic writing skills 111 Proofing 113 Qualitative Reading Inventory Grade Type Words per minute 5 Expository 68 6 Expository 43 7/8 Expository 58

Accuracy (%) 96 96 94

Percentile 95th

92nd

93rd 86th

77th

96th 77th

Percentile 84th 60th 95th 64th 69th 50th 78th 61st 92nd 99th 95th 99th 57th 57th 77th 81st

Comprehension (%) 87 75 50

194 Table 20.3 Other cognitive functions – first evaluation Comprehensive Test of Phonological Processing Phonological awareness Elision Blending words Rapid naming Rapid digit naming Rapid letter naming Phonological memory Memory for digits Nonword repetition Alternate rapid naming Rapid color naming Rapid object naming

K. Lindstedt, M.J. Zaccariello

Score 106 (13) (9) 76 (6) (6) 88 (8) (6) 61 (3) (4)

Percentile 65th

5th

21st

7th

of three distinct yet interrelated concepts. First, phonological awareness is an individual’s awareness of and access to sound structure in language. Second, phonological memory is the ability to temporarily hold phonological information in mind while manipulating it. Third, rapid naming assesses the ability to quickly and efficiently retrieve phonological information. During feedback with families, we refer to phonological awareness as the basic tools of reading. Phonological memory is the ability to briefly hold and manipulate the tools, and rapid naming is how quickly one can accurately use the tools. Ethan’s rapid naming skill was markedly poorer when compared with his other basic phonological processing skills and general neuropsychological profile. Although Ethan’s phonological toolkit was stocked and he could use and manipulate phonological information, he was extremely effortful and inefficient in using the tool box, which rather exquisitely explained his reading difficulties. Dysfluent reading, even if accurate, can significantly affect reading comprehension. Significant time and resources must be expended on decoding individual words, thereby impeding the integration of the material necessary to understand the overall meaning of the passage. The mental energy expended on reading quickly impedes the integration of aspects of the reading passage, often resulting in frustration. Basically, Ethan was a slow reader. On the basis of the evaluation, recommendations included enhancing Ethan’s reading speed or automaticity with a combination of repeated reading and instruction methods called Retrieval, Automaticity, Vocabulary Elaboration, Orthography (RAVE-O) (http://ase.tufts.edu/crlr/raveo.html). In repeated reading, Ethan is asked to read books, chapters, or stories repeatedly (3–5 times) within a severalday period. He should not be asked to do this with all reading materials. This method should help expand Ethan’s automatic sight-vocabulary. A variation of repeated reading is to have a parent or peer read orally along with Ethan. The assisting reader can read all the words at the same pace as Ethan reads, or the assisting reader can provide only those words that Ethan cannot decode immediately himself.

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All children with reading problems need to read regularly at home, for instance 15 min per day for elementary schoolchildren and up to 30 min per day for older children. Ethan’s parents should try to make this fun or practical by using incentives, allowing school-assigned reading to count in the daily reading period, encouraging a mixture of silent and oral reading, alternating reading paragraphs/ sentences with him, and giving a day off from time to time. Above all, home reading should involve high-interest material at Ethan’s current reading level that he helps choose, with a wide range of reading including short books, favorite magazines, newspaper articles, videogame instruction manuals, or chapter books. Ethan’s second evaluation, approximately 8 years later, was to document any changes in his reading fluency. His parents wanted an update of his neuropsychological profile to determine whether intervention or accommodation was necessary. Ethan was resistant to public accommodation or intervention at school and had not received any significant intervention since his initial assessment. The prior test battery was essentially duplicated to provide a comparison of Ethan’s performance relative to his peer group (i.e., norm-referenced comparison) and to evaluate any changes relative to his initial profile (i.e., more of an ipsative comparison).

Second Test Results Ethan’s effort and cooperation throughout testing were once again adequate to ensure that the results attained were a valid and reliable interpretation of his everyday functioning. He demonstrated social behavior suitable for the testing context. He made appropriate eye contact and both initiated and engaged in spontaneous conversation about a myriad of topics. Ethan’s mood appeared reasonably positive. He displayed no overt evidence of significant inattention, distractibility, or impulsivity. Overall, Ethan was polite and his demeanor was pleasant, even on tasks where he had difficulty. Ethan’s neuropsychological profile was for the most part consistent with that seen when he was in fourth grade. A direct comparison across time was not possible because of revisions of some tests and the administration of others that were appropriate for his age (Tables 20.4–20.6).

Second Formulation and Recommendations Ethan’s primary deficit continued to be in rapid naming and reading fluency for which he would qualify for a specific learning disability in Reading Fluency. Scores that were initially mildly to moderately impaired fell to the severely impaired range during testing. The discrepancy in rapid naming scores was not necessarily indicative of a regression in skills. Rather, Ethan was making slow progress in reading fluently. It was as if he were driving in a NASCAR race in first gear while everyone

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Table 20.4 Intelligence – second evaluation Wechsler Adult Intelligence Scale – Third Edition Verbal Comprehension Information Similarities Vocabulary Working Memory Letter-Number Sequencing Arithmetic Digit Span Full Scale IQ Perceptual Organization Picture Completion Block Design Matrix Reasoning Processing Speed Coding Symbol Search Verbal IQ Performance IQ

Score 110 (14) (9) (13) 104 (10) (14) (8) 116 138 (18) (15) (14) 96 (9) (10) 110 124

Table 20.5 Academic achievement – second evaluation Tests Woodcock Johnson Tests of Achievement – Third Edition Broad reading Letter-word identification Passage comprehension Reading fluency Basic reading skills Letter-word identification Word attack Nelson-Denny Reading Test Reading rate Accuracy Gray Oral Reading Test – Fourth Edition Rate Accuracy Fluency Comprehension Oral reading quotient

Percentile 75th

61st

86th 99th

39th

75th 95th

Score

Percentile

95 106 113 85 110 106 113

37th 66th 80th 16th 75th 66th 81st

71–72 62–67

3rd 1st

(6) (5) (2) (9) 73

4th

else was cruising along in fifth gear. Ethan’s poor reading rate was magnified when reading higher ordered, connected text or passages typically encountered in both high school and college. He was extremely dysfluent in his reading, which had a mild to moderate adverse impact on his reading comprehension abilities.

20 A Tale of Two Assessments: Reading Fluency Table 20.6 Other cognitive functions – second evaluation Comprehensive Test of Phonological Processing Phonological awareness Elision Blending words Rapid naming Rapid digit naming Rapid letter naming Phonological memory Memory for digits Nonword repetition

197

Score 106 (10) (12) 52 (2) (2) 97 (10) (9)

Percentile 65th

<1st

42nd

Individuals most benefit from remediation of reading disorders in elementary school. Some respected researchers have even opined that kindergarteners who are at risk for reading disorders can benefit from intervention (Fletcher, 2007). We continued to propose those recommendations outlined during his first evaluation. However, the focus shifted to more accommodation rather than remediation of Ethan’s reading fluency issues. The following suggestions were added. Ethan’s profound reading fluency impairments suggest that it will take him an extraordinary amount of time to complete tests, assignments, or homework that relies heavily on reading. Consequently, he should not be expected to perform at top efficiency level on timed tasks. This does not mean that he should be released from this type of task, but extra time can be given strategically when needed, or homework assignments may be shortened. In addition, curriculum requirements such as timed reading tests might be loosened or modified for him. Shortened assignments at school or a reduced course load would be relevant and beneficial in many subject areas, particularly when extensive reading is involved. Ethan’s parents should apply to a program for visually impaired and dyslexic citizens that can provide access to a library of tapes that includes many of the books he will use in school. This will allow Ethan to listen to textbooks while reading and following along, which will enhance his reading comprehension in all subjects. Given his level of reported psychological distress, a recommendation was also offered for individual psychotherapy, coupled with a strong family dynamic component, to address his behavioral and emotional issues as well as what appear to be mild to moderate family interaction issues. Counseling could provide Ethan an environment to explore his feelings and learn effective coping strategies to deal with his distress and anxiety. Given his level of distress, psychopharmacologic intervention may also be warranted and should be discussed with his psychotherapist. In addition, psychotherapy could allow Ethan’s family a structured venue to explore, discuss, and address ongoing family stressors.

Discussion An important aspect of this assessment was ‘‘text level reading’’ or ‘‘connected text reading’’ The passages included in such instruments are longer and more complex than those completed on most tests of academic achievement. They resemble those

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typically encountered by students at various levels of schooling from preschool to postsecondary education. In essence, they are arguably an ecologically valid way of assessing how a deficient building block of reading can impact higher-ordered processes. Text level tests are broadly broken down into two categories: informal (e.g., Qualitative Reading Inventory-3; QRI) and formal (e.g., Gray Oral Reading Test-4th Edition; GORT-4, or Nelson-Denny Reading Test). The major distinction is that informal reading inventories often lack normative data, such as standard scores and percentiles, which their formal counterparts have. For the second evaluation, only formal reading inventories were used because such tests are commonly accepted by the entities that regulate college entrance exams. The GORT-4 was chosen because it allowed multiple, formal, psychometric levels of evaluation to include comprehension and, what was most important in Ethan’s case, reading rate and fluency. A caveat of using a formal reading inventory such as the GORT-4 is that it relies on having the individual read the passage aloud, quite an uncommon practice for students after the fifth grade. The Nelson-Denny Reading Test was used because it is generally thought to have longer passages and more difficult comprehension questions than the GORT-4. The individual is also allowed to read the passages silently, so it is particularly suited to adolescents or young adults in high school or university settings. The Nelson-Denny is not amenable to some of the fine-grained test scores that the GORT-4 provides. The integration of data from both measures was used to provide a double check of Ethan’s reading fluency impairment, as observed on the test of phonological processing, and to ascertain as validly as possible the extent of his reading problems.

Additional Resources Key Concepts and Terms Rapid naming A component of phonological processing. Refers to how efficiently individuals can retrieve phonological information. Retrieval, Automaticity, Vocabulary Elaboration, and Orthography (RAVE-O) A fluency-based reading intervention program designed to increase reading rate and efficiency with three basic goals: (1) development and enhancement, or fluency, in word identification, work attack, and reading comprehension; (2) reinforcing sublexical (e.g., scanning, pattern recognition, faster initial, and final phoneme recognition) and lexical (e.g., associations) knowledge; (3) practice and accumulation of metacognitive strategies aimed at decoding and retrieving words (Wolf et al. 2000). Phonological Awareness An individual’s awareness of oral language. Phonological Memory Coding information phonologically for temporary storage in working memory. Phonological Processing The method by which sound structure in language (reading, writing, listening, and speaking) is processed.

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References Resources for Clinicians Bowers, P. G., & Ishaik, G. (2003). RAN’s contribution to understanding reading disabilities. In H. L. Swanson, K. R. Harris, & S. Graham (Eds.), Handbook of learning disabilities (pp. 345– 363). New York: Guilford. Chard, D. J., Vaughn, S., & Tyler, B. J. (2002). Synthesis of research on effective interventions for building reading fluency with elementary students with learning disabilities. Journal of Learning Disabilities, 35, 5, 386–406. The Center for Reading and Language Research (n.d.). The RAVE-O program. A competency, fluency-based reading intervention program. Retrieved June 11, 2007 at http://ase.tufts.edu/ crlr/raveo.html Fletcher, J. M. (2007, February). Identifying and treating learning disabilities: The importance of response to intervention. Seminar presented at the meeting of the International Neuropsychological Society, Portland, OR. Fletcher, J. M., Lyon, G. R., Fuchs, L. S., & Barnes, M. A. (2007). Learning disabilities: From Identification to Intervention. (pp. 164–183). New York: The Guilford Press. Joseph, L. M. (2005). Understanding and implementing neuropsychologically based literacy interventions. In R. C. D’Amato, E. Fletcher-Janzen, & C. R. Reynolds (Eds.), Handbook of school neuropsychology (pp. 738–757). Hoboken, NJ: Wiley. Wagner, R. K., & Torgesen, J. K. (1987). The nature of phonological processing and its causal role in the acquisition of reading skills. Psychological Bulletin, 101, 192–212. Wagner, R. K., Torgesen, J. K., & Rashotte, C. A. (1999). Comprehensive Test of Phonological Processing. Austin, TX: Pro-Ed. Wolf, M., Miller, L., & Donnelly, K. (2000). Retrieval, automaticity, vocabulary elaboration, orthography (RAVE-O): A comprehensive, fluency-based reading intervention program. Journal of Learning Disabilities, 33, 4, 375–386.

Resources for Families Council for Learning Disabilities (n.d.). CLD infosheets: Secondary students with learning disabilities in reading: Developing reading fluency, http://www.cldinternational.org/ Infosheets/fluency.asp Hall, S., & Moats, L. (1998). Straight talk about reading. New York: NTC Press. Mather, N., & Goldstein, S. (2001). Reading fluency, http://www.ldonline.org/article/6354 National Institute for Literacy (n.d.). Put reading first, http://www.nifl.gov/partnershipforreading/ publications/reading_first1fluency.html Recordings for the Blind and Dyslexic, http://www.rfbd.org Spear-Swerling, L., & Sternberg, R. J. (1998). Off track: When poor readers become ‘‘learning disabled’’. Boulder, CO: Westview Press. Spear-Swerling, L. (2006, May). Preventing and remediating difficulties with reading fluency, http://www.ldonline.org/spearswerling/8811 Torgesen, J. K. (1998). Catch them before they fall: Identification and assessment to prevent reading failure in young children, http://www.ldonline.org/article/225

Chapter 21

Lost in Space: Nonverbal Learning Disability Richard J. Clark

Though Catherine had always been somewhat intense and anxious in her style and approach to people and events, her recent experiences of extreme anxiety, moodiness, and outbursts of frustration seemed out of character and much more than ‘‘adolescent angst.’’ She was unable to sleep and experienced tachycardia at night, which fueled acute panic attacks and a chronic cycle of fears about her health. She was exceedingly irritable and difficult to reason with or to help calm down. She constantly complained about her ‘‘friends’’ not being friends at all; they thought she was mean or nosey when she was only trying to be helpful and did not share her interests or listen to her ideas. She became particularly focused on certain kids who irritated her and would obsess about their behavior even when it did not affect her directly. Though she had learning difficulties, she had always been a conscientious student, utilizing the support available from school and getting along okay, but now everything seemed too difficult and she was tired of doing the same thing over and over, especially in math. At the time Catherine was referred, she was just beginning her eighth grade year. Her periods of acute anxiety had subsided, and the summer break had been somewhat helpful in reducing her overall stress level, but she remained chronically irritable, edgy, and frustrated with almost any demands, whether they be academic, interpersonal, or routine expectations. Her attitude toward school was especially negative, feeling that she was not learning anything, kids were all mean, and so on. Within individual therapy sessions, efforts to develop relaxation and self-monitoring skills yielded limited results, because her rigidity in interpreting social cues and situations made it very difficult for her to step back and reflect on her options. Work in a therapy group focusing on social interaction and problem solving was somewhat helpful, but her core frustration and tension remained at a high level. After reviewing the progress and challenges in her case, a full psychological evaluation was recommended to help better understand her cognitive and emotional development and hopefully generate new directions for her treatment. A review of Catherine’s developmental history yielded few significant issues in her early years. The final 3 months of the pregnancy were particularly stressful for her mother, as Catherine’s oldest brother died of complications from a brain tumor 5 weeks prior to her birth. Early developmental milestones were within typical

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ranges, though she was slightly delayed in walking and was described as somewhat clumsy or uncoordinated and being rather passive in exploring her environment as a toddler. She had numerous ear infections in her toddler and preschool years and a bout of chicken pox at age 5, which involved prolonged high fever. Catherine was initially diagnosed with Attention Deficit Hyperactivity Disorder (ADHD) in second grade. Dextroamphetamine sulfate, and then dextroamphetamine extendedrelease, had been somewhat helpful in improving focus and work completion at school. Near the beginning of seventh grade, however, she was switched to sertraline HCl because of diminished effectiveness of the dextroamphetamine sulfate and increased stress around her adjustment to middle school. The sertraline HCl was discontinued while her tachycardia was being investigated but was restarted after no medical reason could be established. At about this time, she was referred for therapy to address her anxiety and irritability issues. Catherine’s academic difficulties had begun to emerge in prekindergarten. Although she spoke well and recognized letters and words fairly quickly, she seemed lost when it came to number recognition, one-to-one correspondence, and math reasoning. Though friendly and kind to others, and despite her noted creativity, she frequently played alone and seemed to have trouble integrating her ideas with others. An initial screening suggested some pragmatic language delays and early math deficits, but no services were provided. Near the end of her kindergarten year, however, her difficulties with language processing, receptive understanding, and inference led to initiation of Speech and Language services at school. By the middle of her first grade year, learning disability services were added to support her ongoing struggles with math. Her early writing was impacted by poor fine motor skills (second percentile on a Beery-Buktenica Developmental Test of VisualMotor Integration in first grade) but legibility improved as she practiced, though she would sometimes take a long time to get things ‘‘just right.’’ In reading she could decode words okay but initially had difficulty tracking and pointing to a word within a sentence and was unaware of the reason for spaces between words. At the time of initial referral, she received services through the Learning Disability program at her middle school, with direct math instruction as well as support for other classes. The Wechsler Intelligence Scale for Children – Third Edition had been administered in February 1997 and again in November 1997 as part of the process for determination of special educational need. The initial assessment found borderline deficient ability overall (Verbal 75, Performance 78, Full Scale 75). Although she was consistently in the borderline range on the Performance subtests, she showed a great deal of variability within the Verbal scale, ranging from average on Information and Similarities to very deficient on Arithmetic and deficient on Comprehension (scale scores 1 and 4, respectively). The 11/97 assessment (at age 6 years and 11 months) yielded higher estimates in all areas, with a Verbal Score of 94, Performance Score of 86 and Full Scale score of 90. Catherine’s history of math difficulties, pattern of abilities in early cognitive assessments, and functional social/emotional weaknesses raised the question of whether she had a Nonverbal Learning Disability (NLD). NLD is a construct

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that has been summarized by Rourke (1989) as a pattern of neuropsychological strengths and weaknesses, which have impact on both academic and socioemotional development. More specifically, individuals with NLD usually show relative strengths in language processing, rote verbal memory, reading recognition, and spelling skills. Conversely, they typically show relative weaknesses in visual-motor coordination, nonverbal problem solving, abstract reasoning, tactile perception, arithmetic calculation and reasoning, and social interaction skills. One of the general initial indices for NLD has historically been a significant split between Verbal and Performance (nonverbal) scale scores, in favor of the former, on the Wechsler Intelligence Scales for Children, Revised and Third Edition. The new revision of the Wechsler scales, however, represented a fairly significant change, expanding to four scales instead of two, as well as adding and dropping some subtests. The changes in what had previously been called the Performance scale, now the Perceptual Reasoning scale, were most marked, with only one subtest remaining from the previous edition. The new Working Memory and Processing Speed scales included some of the key subtests from the previous measure, along with new ones. A new, expanded assessment (Wechsler Intelligence Scales for Children, Fourth Edition, Integrated) was also now available, which provided additional measures within each domain as well as some alternative administration options and break down of measures into different components. While attempting to understand Catherine’s capacities and needs, this assessment would also provide a look at how an apparent NLD profile ‘‘from history’’ would appear on this new measure.

Test Results Catherine was pleasant and cooperative throughout the three evaluation sessions. While clearly anxious at various points and with particular activities (especially math related), her familiarity with the examiner seemed to help her to continue to make a good effort. She showed relatively good verbal communication skills but sometimes got stuck on certain ideas and did not readily adjust to the feedback she was receiving. She frequently had difficulty expanding on her initial answer to clarify or show more depth of understanding. Although she commented on many activities that she perceived as difficult (‘‘these are getting hard!’’), she tended to be overly positive in appraisal of her actual performance, with little critical thinking or reflection of awareness or frustration in less successful completion of the tasks, especially those involving visual detail, construction, or mental manipulation. She was generally more fluid in responding to verbal items while being less efficient and organized in attempting tasks that involved more visual complexity, perceptual speed, and processing skills. On the basis of her effort and attitude throughout, these appeared to be valid measures of her current levels of cognitive and academic functioning (Tables 21.1–21.3).

204 Table 21.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition, Integrated Verbal Comprehension Index Perceptual Reasoning Index Working Memory Index Processing Speed Index Full Scale IQ Verbal comprehension Similarities Vocabulary Comprehension (Information) (Word Reasoning) Perceptual Reasoning Block Design Picture Concepts Matrix Reasoning (Picture Completion) Perceptual Domain Supplemental Block design no time bonus Block design multiple choice Block design multiple choice no time bonus Block design process approach Elithorn Mazes Elithorn Mazes no time bonus Working memory Digit span Letter-number sequencing (Arithmetic) Working memory domain supplemental – registration Digit span forward Visual digit span Spatial span forward Letter span nonrhyming Letter span rhyming Working memory domain supplemental – manipulation Digit span backward Spatial span backward Letter-number sequencing process approach Arithmetic with time bonus Arithmetic process approach part A Arithmetic process approach part A time bonus Arithmetic PA Part B Written arithmetic Processing Speed Coding Symbol Search (Cancellation)

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Scorea 98 61 59 78 70

Percentile 45th 0.5th 0.3rd 7th 2nd

(10) (10) (9) (6) (8)

50th 50th 37th 9th 25th

(2) (3) (6) (4)

0.8th 1st 9th 2nd

(1) (4) (6) (1) (6) (6)

0.3rd 2nd 9th 0.3rd 9th 9th

(5) (1) (3)

5th 0.3rd 1st

(8) (11) (5) (9) (12)

25th 63rd 5th 37th 75th

(3) (2) (3) (6) (4) (5) (3) (2)

1st 0.8th 1st 9th 2nd 5th 1st 0.8th

(7) (5) (8)

16th 5th 25th (continued)

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Table 21.1 (continued) Wechsler Intelligence Scale for Children – Fourth Edition, Integrated Processing Speed Domain Supplemental Cancellation Random Cancellation Structured Coding Copy Similarities Vocabulary Comprehension Information Word Reasoning Block Design Picture Concepts Matrix Reasoning Picture Completion Digit Span Letter-Number Sequence Arithmetic Coding Symbol Search Cancellation a Standard score, (scaled score), [T-score], {z-score}. Table 21.2 Other cognitive functions Tests Beery-Buktenica Developmental Test of Visual-Motor Integration California Verbal Learning Test – Children’s Version List A total trials 1–5 List A trial 1 List A trial 5 List B trial 1 List A short delay List A short-delay cued recall List A long delay List A long-delay cued recall Percent recall – primary Percent recall – middle Percent recall – recency Percent recall consistency List A recognition The Tower of London Wisconsin Card Sorting Test Conceptual level responses Categories correct Failures to maintain set Perseverative responses

Raw Score

Scorea

Percentile

(7) (11) (5) (10) (10) (9) (6) (8) (2) (3) (6) (4) (5) (1) (3) (7) (5) (8)

17th 63rd 5th

Score 65

Percentile 1st

[49] [50] [50] [45] [45] [40] [50] [45] [55] [45] [55] [50] [50] 77

47th 50th 50th 32nd 32nd 16th 50th 32nd 68th 32nd 68th 50th 50th

58 <1st >16th 0 (continued)

206 Table 21.2 (continued) Tests Perseverative errors Intermediate Booklet Category Test Dominant hand (left) Grip strength (kilograms) Finger Tapping Test (per 10 S) Grooved Pegboard Test Trial 1 seconds Trial 1 drops Trial 2 seconds Trial 2 drops Nondominant hand Grip strength kilograms Finger Tapping Test (per 10 S) Grooved Pegboard Test Trial 1 seconds Trial 1 drops Trial 2 seconds Trial 2 drops Dominant hand (left) (errors per 20 trials) Finger localization Fingertip X/O perception Fingertip number perception Nondominant hand Finger localization errors/20 Fingertip X/O perception Fingertip number perception Achenbach Child Behavior Checklist Parent form Withdrawn/depressed Anxious/depressed Somatic complaints Social problems Thought problems Attention problems Rule breaking behavior Aggressive behavior Teacher’s report form Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule breaking behavior Aggressive behavior

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Raw Score 0

Score

Percentile

33

<1st

8 29

37 53

125 2 83 0

0 63

16 28

69 61

123 0 102 0

0 40

4 0 14 14 0 13

76 70 68 69 72 83 51 70

99th 98th 96th 97th 99th >98th 54th 98th

65 56 50 57 65 54 50 50

93rd 73rd <50th 76th 93rd 65th <50th <50th

21 Lost in Space: Nonverbal Learning Disability Table 21.3 Academic achievement Wechsler Individual Achievement Test – Second Edition Reading composite Word reading Reading comprehension Pseudoword decoding Math composite Numerical operations Math reasoning Written language composite Spelling Written expression

207

Score 99 104 100 102 45 55 51 97 99 98

Percentile 47th 61st 50th 55th 0.02nd 0.3rd 0.08th 42nd 47th 45th

Grade equivalent 9.8 9.1

3 2.8 8.5 8.5

Catherine’s overall cognitive ability was at the cusp of the borderline deficient to deficient range, though it is her pattern of functioning across the various scales and supplemental measures that helps to tell the story of her cognitive capacities. She was generally consistent in scoring in the average range on the subtests of the Verbal Comprehension scale, resulting in a standard score of 98. She demonstrated average vocabulary knowledge, language development, crystallized abilities, and verbal reasoning. Catherine was generally able to relate her ideas and knowledge effectively and understand verbal input in order to access this knowledge. She provided acceptable responses on many tasks requiring reasoning and integration of knowledge, though she sometimes had trouble providing additional detail or clarification of meaning. Catherine’s performance on the Perceptual Reasoning and Working Memory scales was in marked contrast to these global verbal strengths, with scores in the deficient and very deficient ranges, respectively. Within the global fluid reasoning and simultaneous processing difficulties measured by the Perceptual Reasoning cluster, she showed marked weaknesses in visual perception and organization, abstract categorical thinking and reasoning, concept formation, and separation of visual figure/ground. Her best performance in this area (though still at the cusp of borderline deficient) was on the Matrix Reasoning subtest, on which she showed slightly better ability to recognize a pattern and choose from options to complete it. In attempting the Block Design items, Catherine showed extreme weakness in orienting the blocks individually or in relation to each other. The supplemental Block Design Multiple Choice (BDMC) and Process Approach (BDPA) measures further underscored her visual perception and organization difficulties. In the former, the Block Design task was presented as a multiple choice task (the child is shown a design and four possible block arrangements, with the blocks within each separated by about half an inch, then asked to choose the one that ‘‘when put together in your mind’’ matches the design). The multiple choice format eliminates the motor manipulation of the blocks to form the design and is more dependent on being able to visualize the block patterns, as well as requiring vigilance and impulse control. Under this format, she was unable to get any of the items correct. Within the Process Approach, she failed to correctly reproduce any of the six new designs

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and then did not benefit at all from a second opportunity, which included the use of an overlay to differentiate the individual blocks within each design. It was striking to note the differences in her performance on the Similarities and Picture Concepts subtests. These both involve the ability to apply fluid reasoning abilities to establish increasingly abstract associations between objects and concepts. Their primary difference lies in input modality, with verbal presentation and response on Similarities vs. the visual presentation and selection of response (either verbally or by pointing) from groups of pictured objects on Picture Concepts. Catherine scored in the average range when the items were presented verbally but in the deficient range when she had to view the options and choose. Her poor performance in the Picture Completion subtest also underscored her difficulty accessing and organizing visual gestalts and differentiating essential details within them. Her ability to recognize, access, and retrieve information through the visual input channel appeared to be significantly compromised. Catherine showed marked contrasts in performance across the Working Memory subtests and supplementary measures. She was consistently in the average range on measures of immediate rote recall for letters and numbers, thus demonstrating adequate ability, through both the auditory and visual input modalities, to register and promptly recall various nonmeaningful items in correct sequence. In contrast, she did poorly on all of the mental manipulation tasks, which require registering input, organizing it in some way, and then responding. She was also weak on the Spatial Span subtest, which requires ability to maintain a spatial pattern in memory, then copy it exactly or in reverse. This pattern of functioning suggested that Catherine’s basic encoding of information (other than more spatially oriented) was intact but that the transfer to the working memory centers was inefficient. This would likely contribute to difficulties relating new information to previous knowledge, recognizing larger implications of information, maintaining understanding of concepts while also attempting to copy from the board or take notes, and responding to test items that use distracters or foils. Catherine’s scores on the Processing Speed measures were also significantly lower than her verbal abilities, though somewhat better than her perceptual organization and working memory abilities. She had most difficulty on a task requiring her to scan a row and find items matching to a stimulus. Her low average score in Coding was likely effected most by her slower visual motor speed, as was seen in her borderline score in the Coding Copy supplemental subtest. On the Cancellation subtest, she benefited significantly from the added organizational structure in the second trial (difference of 4 scale points). Previous measures of Catherine’s visual motor integration had shown this to be an area of weakness. The current assessment validated this, as her VMI score was significantly low. Other observations of her graphomotor output via writing samples and drawings also suggested delays: she made dark, heavy lines, was accurate but somewhat slow in her letter formation and writing, and showed a high degree of pencil pressure, and her drawings were quite immature and ill formed for her age. Additional assessment of Catherine’s memory was accomplished via the California Verbal Learning Test for Children (CVLT-C). On the CVLT-C, Catherine

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was relatively consistent in her performance, showing average total recall over the five trials, immediate recall upon initial presentation of the list, and cumulative recall by the fifth trial. She maintained adequate recall after the interference list was read and after a 20-min delay, and was able to recognize words from the list adequately when read to her. An area of weakness was seen in her failure to benefit from—and in fact the detrimental effect of—the organizational cues provided in the short and long-term recall trials. This suggested that although Catherine was able to store input adequately, she did not easily relate this information to previous knowledge and use such categorical concepts for more efficient recall or to develop or understand higher level relationships. Assessments of tactile perception and aspects of executive functioning were also completed. Marked disparity in fine manual dexterity was evident on the Grooved Pegboard Test, as she showed moderate impairment in her dominant hand (left) with severe impairment in her nondominant hand. Other measures of tactile perception showed generally poor but variable performance, with severe impairment in the dominant hand on finger tapping and grip strength vs. moderate impairment in the nondominant hand, but much worse impairment on finger localization for the nondominant hand. She also performed much more poorly bilaterally on complex (single digit numbers) vs. simple (X or O) form recognition activities. Her planning and sequential problem solving were in the borderline deficient range on the Tower of London. Set maintenance and flexibility in shifting sets were severely impaired on the Wisconsin Card Sorting Test, as were her abstract reasoning and concept formation on the Booklet Category Test. Catherine’s academic achievement also showed significant variability. Her reading and writing skills were adequate, but she had very significant deficiencies in mathematic reasoning and calculation. Within the reading assessment, Catherine showed average ability to recognize words and comprehend written material. A closer look at her comprehension suggested good ability to answer factual information questions, but more limited ability to draw inferences, tie together concepts or recognize more subtle meanings, especially in more lengthy passages vs. within a single sentence or two. Her written output was also average, with adequate sentence structure, punctuation, and grammar, though again she tended to be somewhat simplistic and concrete in expressing her ideas on paper during the essay portion of the assessment. In contrast to these average abilities, Catherine showed marked and pervasive deficiencies in almost all aspects of her mathematical knowledge. Although she knew the steps of basic calculation, including adding fractions, she made frequent errors in following these steps. Many of her calculation errors reflected her significant visual-spatial deficiencies, as she failed to notice changes in sign (added instead of subtracted), varied the directionality on subtracting (top from bottom), and had trouble aligning the numbers. She was variable in demonstrating skills, missing a simple item and then getting another similar or slightly more difficult one right, though she was consistently unable to do even the simplest multiplication or division problems (8  5; 12  6). She always used her fingers for adding and subtracting (but still made errors, i.e., 6  3 ¼ 2). There was literally no automaticity

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in her sense of math concepts or relationships. Of even greater concern than these calculation errors was her marked inability to reason and think mathematically. She was unable to translate mathematic language to solve very simple word problems, understand or utilize visual representations of information (charts, graphs, icons), or recognize errors or approximations of correct responses—even when presented with choices. The results of the Achenbach checklists completed by Catherine’s parents and teachers were consistent with the pattern of functioning that led to her initial referral (Table 21.2). The teacher checklist yielded mostly nonsignificant levels with the exception of borderline clinical ratings on the Anxious/Depressed and Thought Problems scales. The teacher’s written concerns focused on Catherine’s being overly concerned about things she could not control, being distracted and ‘‘stuck’’ on frustrations with others, and difficulty focusing and responding to ongoing demands for attention. Ratings from Catherine’s parents suggested significant clinical concerns with withdrawal, anxiety, depression, and somatization, which all contributed to a very significant elevation on the Internalizing scale. In addition, concerns of social problems, attention, thought problems, and aggressive behaviors were indicated. They reported that she ‘‘held it together’’ during school but at home became easily agitated and loud, even if the simplest request was made of her. She obsessed about certain topics, either very specific interests (pictures of famous people, TV shows, Poke´mon) or frustrations with school or peers, and was unwilling or unable to engage in much discussion of solutions or interactions around anything else.

Formulation and Recommendations On the basis of observations of her behaviors as well as the data available for the present assessment, Catherine demonstrated a pattern of strengths and weaknesses highly consistent with NLD, a neuropsychological construct hallmarked by a significant discrepancy between relatively intact verbal capacities and rote auditory memory skills as contrasted to significant weaknesses in visual constructive skills, visual processing, tactile perception, and spatial/temporal reasoning (Table 21.4). Most children with NLD have attention problems, struggle academically with higher order thinking, comprehension, and mathematics in general, but develop adequate word reading and spelling skills, have social difficulties due to poor pragmatic language and failure to adjust to social/situational demands, and are at significant risk for anxiety and mood disorders. Most also meet DSM-IV diagnostic criteria for Asperger’s Syndrome. It is interesting to look at how the newer version of the Wechsler scales contributes to the understanding of the NLD profile. Although there is not a formal procedure for diagnosing NLD, and it is not currently included in any classification system, Rourke and his colleagues have proposed guidelines, which are under investigation. While some of the stated guidelines are test specific, and not all of

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Table 21.4 Classification principals for diagnosing NLD (ages 9–15 years) 1. Simple tactile-perceptual skills are superior to complex tactile-perceptual skills. 2. Single word reading is superior to mechanical arithmetic. 3. Straightforward and/or rote verbal skill are superior to those involving more complex processing (e.g., comprehension). 4. Complex visual-spatial-organizational skills and speeded eye-hand coordination are impaired. 5. Memory for visual sequences is impaired. 6. Simple motor skills are superior to those involving complex eye-hand coordination, especially under speeded conditions. 7. Complex tactile-perceptual and problem-solving skills under novel conditions are impaired. 8. Verbal skills are superior to visual-spatial-organizational skills. First 5 features: Definite NLD 7 or 8 features: Definite NLD 5 or 6 features: Probable NLD 3 or 4 features: Questionable NLD 1 or 2 features: Low probability of NLD Used with permission from http://www.nld-bprourke.ca.

the tests were administered in this assessment, the underlying principles for the rules can be looked at relative to the present data, particularly concerning the WISC-IV. In the present assessment, a significant difference was shown between verbal and visual-spatial skills, as evident in the 31 point difference in Verbal Comprehension and Perceptual Reasoning scores. The evidence regarding rote vs. more complex verbal skills can also be seen within the Working Memory scale, especially with the additional measures of the Integrated version, as immediate recall was fine, but she struggled with more complex processing (Letter Number Sequencing, Arithmetic), memory for location in space (Spatial Span), and organizing and manipulating information (Digit Span Backwards, Spatial Span Backwards). Speeded eye-hand coordination was also shown to be a weakness on the core measures of the Processing Speed scale as well as the Coding Copy supplemental measure. In addition to helping flesh out these variables, the WISC-IV integrated information could assist in programming for Catherine. For example, the breakdown of the Cancellation task showed that she did much better when the visual information was more structured. Conversely, her core deficits in processing and performing mental math were not improving by providing paper and pencil or written equations. The depth of her perceptual organization difficulties seen on Block Design was underscored with the supplemental multiple choice and Process approach administrations. At the same time, her slightly better performance on Matrix Reasoning and the supplemental Elithorn Mazes gives some direction to task modifications that may help her utilize her visual processing system more efficiently. Once Catherine’s NLD was identified, the first intervention was to work on educating her teachers and parents regarding the implications of this profile on her academic and interpersonal functioning. Her school system was very receptive to revisiting her Individualized Education Program (IEP) and making curricular

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accommodations as necessary. Individual therapeutic intervention continued, with an emphasis on increasing her awareness of her own strengths and weaknesses and building a toolbox of coping strategies. The following recommendations represented some of the key concepts that were emphasized to all those involved in her programming. Catherine’s significant weaknesses in visual/spatial organization and reasoning will impact her learning in multiple ways. She will struggle more with tasks and materials that are inherently visual or spatial in their nature. More subtly, she will not be able to rely on visualization strategies that are typically used to increase efficiency in learning and recalling information and, most critically, that are often assumed to be operating in giving instruction. Both in learning and interpersonally, she will tend to rely exclusively and literally on verbal input and knowledge and will ignore other important nonverbal strategies and sources of information. Although it is important to play to her strengths, it is also important to continually emphasize visual and other nonverbal aspects of materials and situations and not give up on the visual channel but know that it has to be spelled out and attended to much more specifically. Developing a verbal script or dialogue helps tie together the ideas, steps, and so on that are being taught, especially when there are strong visual components (science activities, maps, charts, physical skills). Catherine tends to learn fragmented facts rather than linking together broader concepts, so utilizing some of her verbal strengths will help build bridges between facts and ideas. This is especially critical in math, where Catherine has failed to develop even a basic sense of arithmetic relationships and the language of them. A literal dictionary of specific math terms and a verbal script for how they are applied should be developed with Catherine. These should be grouped by function (e.g., and, together, in total all involve the adding function) with a phrase for use of each. Catherine’s direct involvement, along with some creativity and humor, will be important in helping this to become functional for her. Once the core terms and associated phrases are established, examples and templates for when these terms might appear should be generated, with an emphasis on real-life experience and need. Providing Catherine with verbal and/or visual ‘‘templates’’ to organize key information, emphasize priority in relation to other concepts, and establish essential vs. less essential elements of the information would be of benefit, as would tapping into Catherine’s solid verbal knowledge and vocabulary to mediate tasks that have more spatial and motor output demands. She would benefit from describing the activity in detail and building a verbal model for the physical characteristics and actions of the task. It is not recommended that she rely on simply modeling (‘‘do it like this’’) but rather verbalize the steps clearly and succinctly. On the CVLT-C, Catherine did not benefit from cues about categories for recalling a word list. This suggests an inefficiency in organizing and relating new input to previous knowledge, which then affects recall on demand. It will be important to provide her with conceptual cues, key terms, and ideas, being especially conscious during the teaching process about the types of cues and triggers, which might be used to assess her understanding at a later point. It would be good to

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engage her actively in this process by asking what she knows about a topic and how this is similar to other material, for example. Catherine continues to show a high degree of emotional reactivity, anxiety, and poor regulation of affect, though she exhibits this mostly at home. It is difficult for her to slow her own reaction in stressful situations, which exacerbates her already poor ‘‘reading’’ of those situations and her ability to generate alternative behavioral responses. At school, it appears that she will internalize this rather than express it outwardly, though it is clearly impacting her concentration and contributes to lower frustration tolerance and meltdowns at home. It will be important for significant figures around her to model emotional reactions, awareness, and functioning. Adults tend not to verbalize the problem solving process, but Catherine needs to hear such things as feelings, content of the situation, and actions. For example, ‘‘I was so upset! I could not understand why that lady cut in front of me in the grocery line. I took a deep breath and decided to just let it go.’’ It is important to work to identify Catherine’s emotional state at the beginning of a conflict cycle and to use words, tone, and expressions to let her know that she is understood to be angry or disappointed, for example. She often does not recognize that others understand her feelings and needs or that others have needs that can also be accounted for in striving for her own agenda (poor ‘‘theory of mind’’). Establishing that there is a shared recognition of her emotional state can, over time, help to reduce her intensity and open the door for a problem solving dialogue. Being patient and not immediately moving to restate the demand or give a solution allows her to expand on or explain her emotional state before moving on. Understanding nonverbal social signals (facial expression, body posture, tone of voice) and integrating them into the rapid pace of reciprocal interactions can be very challenging for Catherine. Group therapy and direct instruction of social interaction skills will be important components of her treatment. For interpersonal and social concepts, which are by nature more abstract, use of Social Stories (a la Carol Gray) may be helpful. This is not simply a list of rules but should incorporate the present task demands, perspectives, and feelings of others as well as Catherine, and how people will feel after successfully negotiating a problematic situation.

Update Three years after the initiation of the evaluation process, Catherine has entered the 11th grade at a large suburban school, working hard to deal with the rigors of high school with a positive attitude. A creative and dynamic team constantly monitors the curriculum to make appropriate accommodations while maintaining high expectations for her learning. Emotional meltdowns and obsessive thinking are greatly reduced at home. Challenges continue and many remain, but Catherine is moving on a positive path and is feeling good doing so!

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Additional Resources Key Concepts and Terms Nonverbal Learning Disabilities Proposed International Classification of Diseases (ICD) definition: The syndrome of Nonverbal Learning Disabilities (NLD) is characterized by significant primary deficits in some dimensions of tactile perception, visual perception, and complex psychomotor skills, and in dealing with novel circumstances. These primary deficits lead to secondary deficits in tactile and visual attention and to significant limitations in exploratory behavior. In turn, there are tertiary deficits in tactile and visual memory and in concept-formation, problem-solving, and hypothesis-testing skills. Finally, these deficits lead to significant difficulties in the content (meaning) and function (pragmatics) dimensions of language. In contrast, neuropsychological assets are evident in most areas of auditory perception, auditory attention, and auditory memory, especially for verbal material. Simple motor skills are most often well developed, as are rote verbal memory, language form, amount of verbal associations and language output. This mix of neuropsychological assets and deficits eventuates in some formal learning (e.g., academic) assets, such as single-word reading and spelling. It also increases the likelihood of significant difficulties in other aspects of formal learning (e.g., arithmetic and science) and informal learning (e.g., as transpires during play and other social situations). Psychosocial deficits, primarily of the externalized variety, often are evident early in development; psychosocial disturbances, primarily of the internalized variety, are usually evident by late childhood and adolescence and into adulthood (from http://www.nld-bprourke.ca).

Pragmatics Everything about communication that is not the words. Meaning that is conveyed via facial expression, utterances, tone of voice (prosody), gesture, posture, proximity, and even clothing!

References Resources for Clinicians Drummond, C.R., Ahmad, S.A., & Rourke, B.P. (2005). Rules for the classification of younger children with Nonverbal Learning Disabilities and Basic Processing Disabilities. Archives of Clinical Neuropsychology, 20, 171–182. Pelletier, P.M., Ahmad, S.A., & Rourke, B.P. (2001). Classification rules for Basic Phonological Processing Disabilities and Nonverbal Learning Disabilities: Formulation and external validity. Child Neuropsychology, 7, 84–98. Rourke, B.P. (1989). Nonverbal learning disabilities: The syndrome and the model. New York: Guilford Press. Rourke, B.P. (Ed.). (1995). Syndrome of nonverbal learning disabilities: Neurodevelopmental manifestations. New York: Guilford Press. Byron Rourke’s Web site, http://www.nld-bprourke.ca

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Resources for Families Duke, M. P., Nowicki, S. & Martin, E. S. (1996). Teaching your child the language of social success. Atlanta: Peachtree. NLDLine (Sue Thompson’s support site), http://www.nldline.com/ NLD on the Web, http://www.nldontheweb.org Stewart, K. (2002). Helping a child with nonverbal learning disorder or Asperger’s Syndrome. Oakland, CA: New Harbinger. Thompson, S. (1997). The source for nonverbal learning disorders. East Moline, IL: LinguiSystems. Whitney, R.V. (2002). Bridging the gap: Raising a child with nonverbal learning disorder. New York: Perigee.

Chapter 22

Beyond Diagnosis: Applied Behavior Analysis Treatment of Moderate Autism Spectrum Disorder Christopher P. Wiebusch

Sam’s case, like many cases of Autism Spectrum Disorders (ASD), can challenge the usual approach to neuropsychological assessment. It illustrates one application of Applied Behavior Analysis (ABA) treatment and the incorporation of an assessment model different from the typical rigorous use of norm-referenced instruments. The criterion-referenced measure does not produce standard scores or age equivalents but, rather, identifies more fine-tuned, skill-specific targets for treatment as well as progress toward treatment goals through regular reevaluation. Such an approach can be a highly practical resource that is directly applicable to treatment, steering intervention by providing information not available from normreferenced measures of broader ability areas. Sam was the first child born to a loving family, who watched him respond to noises and speech as an infant and develop cooing, babbling, and jargon prior to age 2. His language development stopped, and he became less verbal and generally appeared less happy at the age of 19 months. He no longer laughed and giggled as he had previously, and he would engage in prolonged periods of staring or rolling toy trains or cars back and forth. He did not respond when called. His parents wondered whether these changes related to the coincident birth of his sister, with whom he would not interact at the time. Speech and language evaluation at age 2 years and 3 months noted extremely limited expressive (9–12 month age-range) and receptive (6–9 month age-range) language. Screening at that time indicated normal hearing. When Sam was aged 2 years and 6 months, speech therapy observations described the following as significant progress: he would leave the couch, allow a therapist to touch him, respond to his name by turning his head when called, and use one to two words. He preferred isolation. Although these observations were intended to communicate progress, they also reflected the severity of Sam’s troubling symptoms. Although the difficulties were considered communication-related at the time, the implication that he had not been responding when called by name was a meaningful clue of his impending broader ASD diagnosis. An Early Childhood educational evaluation conducted in Sam’s home when he was aged

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2 years and 10 months indicated that he seemed aware of actions around him but that he had poor eye contact and spoke mainly with jargon and screeches. Particular delay in social and emotional skills was noted. Sam’s parents, attuned to developmental concerns, attempted biological interventions often advocated for children with his profile, including a casein (gluten) free diet and vitamin supplements, without obvious benefit. Sam’s mother, reflecting the reality of the family’s experience that is often missing from the typical clinical summaries, wrote, ‘‘No one thinks it might happen to them: to have a disabled child. . . to face the fact that our sweet little boy might never be able to have an education, hold a job, or have a family of his own.’’

Test Results At age 3 years and 3 months, after Sam had been attending speech and language therapy for some time, he underwent another speech and language evaluation. At that point records began to note that clinicians had suggested the possibility of Pervasive Developmental Disorder, or Autism Spectrum, symptoms. Verbalizations continued to consist primarily of jargon, rarely with communicative intent. He lacked eye contact, cooperative play, and age-appropriate imitation ability. His expressive and receptive language delays continued, and diagnostic consultation to consider an ASD was recommended. At age 3 years and 5 months, Sam was taken to a pediatric neuropsychologist involved in Autism treatment. At that time, he had limited social interaction, no gestural communication, and no peer interaction. His mother wrote, ‘‘He only spoke a few words. He did not say ‘mama’ or ‘dada’ or even ‘yes’ or ‘no.’ He could not follow simple directions or even respond routinely to his own name. He would spend hours flipping through the pages of an address book or phone book. He put up a barrier that was impossible for us to break through and we felt helpless and frustrated.’’ However, he also had improved modestly in responsiveness to others, was smiling more frequently, and, importantly, was demonstrating some possible affective reciprocity, such as smiling in response to facial expressions. His language continued to be characterized by much jargon and included stereotyped and scripted phrases, such as ‘‘mmm cake’’ in any context related to food or hunger. He lacked communicative intent, only infrequently communicating his desires as he typically attempted to fulfill them independently. He did not ask questions. Although he aligned objects frequently, his earlier agitation if the alignment was changed was subsiding as he developed greater ability to tolerate change. He sometimes walked in circles or spun his body. Sam’s case, like so many early childhood ASD cases, raised the question of the utility of traditional neuropsychological assessment. His ability to respond to assessment sufficiently to produce a valid and useful profile, the cost/benefit ratio of evaluation particularly considering the impending time and financial cost of treatment, and administrative requirements in obtaining public funding assistance

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suggested against a neuropsychological battery. Rather, he was evaluated diagnostically through observations, careful clinical interview with his mother, and developmental ratings. These steps suggested that his diagnosis was clear, and the evaluation turned primarily toward the goal of initial treatment planning. Therefore, although standardized diagnostic assessment tools specific to ASD are available, they were not components of Sam’s assessment at the time. Had there been diagnostic doubt, additional standardized observational and symptom rating instruments would have been employed. Results of the Child Development Inventory indicated significant delays in general development, including particular weaknesses in self-help skills, social behaviors, expressive language, and language comprehension, all falling in the 14 to 16-month age-equivalent range. On the Scales of Independent Behavior— Revised, serious delays were documented in social communication, personal living skills, and community skills, all characteristic of a 1-year-old. An ASD was diagnosed on the basis of communication impairment, including primarily atypical and scripted phrases and jargon and very limited purposeful communication; social impairment, including poor joint attention and affective reciprocity, although interaction was not entirely absent; and some self-stimulatory, stereotyped behaviors. Unlike many children with ASD, Sam had the strength of the ability to tolerate changes in his environment without great difficulty.

Formulation and Recommendations Sam’s clinical presentation left little doubt about an ASD diagnosis. Sam was considered a strong candidate for intensive treatment utilizing an Applied Behavior Analysis (ABA) model. His emerging social responses and verbalizations, along with his responses to initial teaching attempts, qualitatively suggested his ability to further develop these and other seriously impaired skill domains. Clarification of the nature of ABA treatment is critical. Although many of those who are primarily engaged in assessment and diagnosis routinely refer to ABA therapies, relatively few neuropsychologists provide ABA or are intimately familiar with the methods. First, it is critical to understand that parents of a child newly diagnosed with ASD face a crushing volume of information about an overwhelming multitude of treatment options. (An Internet search for information on ‘‘Autism treatment’’ yields 1.5 million Web sites.) Although a review of the variety of treatments is well beyond the scope of the current discussion, it is important to note that a parent in this situation is faced with having to choose between behavioral, relational, educational, sensory, biological, dietary, medical, and other interventions, many of which contradict one another and all of which require time and money. The diagnosing professional is in a position to provide guidance and direction at a time when parents are emotionally and practically overwhelmed. In providing direction, the professional benefits from the knowledge that ABA therapies are broadly considered to have the most research support as effective in

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addressing deficits experienced by individuals with ASD (e.g., Cohen, AmerineDickens, & Smith, 2006; Maurice, 1996; Sallows & Graupner, 2005). Because of his pioneering and tremendous contributions to this field, the first name typically associated with the application of ABA in Autism is Dr. O. Ivar Lovaas. However, it is important to note that the Lovaas model of ABA therapy is not synonymous with all ABA therapy. ABA is a broad field that utilizes techniques and data from a number of models and contributors. What ABA programs have in common is that they utilize scientific principles of behavior to build socially useful repertoires and often to reduce problematic behaviors (Cooper, Heron, & Heward, 1989). ABA programs teach skills, or teachable subcomponents of larger skills, through systematic analysis of the desired skills, intensity in terms of time and repetition, and careful positive reinforcement of desired behaviors and elimination of reinforcement of inappropriate behaviors. Data are collected continuously and systematically to guide treatment and ensure efficacy. The ideal goal is to develop a repertoire of functional, not simply scripted or ‘‘robotic,’’ skills that a child can generalize to use in multiple environments. Prior to beginning ABA treatment, Sam’s parents faced the difficult but common experience of receiving conflicting professional advice. Although ABA was recommended by some involved professionals, at least one professional advised against it. This advice against ABA was reportedly given by a professional who was providing a therapy in which Sam was not obtaining significant benefit. The critique was based not upon specific clinical objections to ABA techniques or goals, but on a general negative feeling about ABA. It is not uncommon in clinical practice to encounter families who have been warned that ABA will simply teach repetitive or robotic responses. Properly applied, ABA seeks to teach functional skills and to generalize them to assist clients in using their new skills in a variety of settings, with different people, and in response to varied demands. For example, if a child learns to label a picture of an apple, it is critical that he be able to use the word apple not only as a scripted response to the question, ‘‘What is it?’’ presented at a table by a therapist. He must also be able to ask for an apple, name other pictures of apples, name actual apples, tell his mother he sees an apple, understand that an apple is a fruit, and so on. For many children with ASD, all of these varying uses of the word must be taught, and different clients achieve different degrees of success in the ultimate complexity and fluidity of the skills they develop. Another critique of ABA that is often shared with families seeking treatment is that it seeks to teach skills but does not cure the fundamental disorder of ASD. This is true. Although some clients reach a level of skill development and symptom improvement at which they no longer demonstrate a diagnosable ASD, this does not hold true for most clients, and ABA providers typically do not claim to cure the disorder. What ABA offers is not magic but roll-up-your-sleeves hard work to address skill deficits and behavioral concerns. Sam’s parents opted to pursue ABA, and Sam entered his intensive ABA program prior to his fourth birthday. His treatment heavily included assessment and intervention methods from the field of Applied Verbal Behavior (AVB), an area of ABA that builds on Skinner’s (1992) analysis of verbal behavior to address

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communication deficits. Skinner’s analysis behaviorally defines functionally different types of language (Table 22.1). In Sam’s program, teaching procedures were utilized to teach specifically to each functional category of language. For example, if he had learned through repetition to label a cup, he may not generalize this word to functional use when he wished to request (rather than label) a cup. It was necessary to teach the word as a request as well. This emphasis on teaching to specific functions of language, going well beyond simple memorized labels for therapy materials, was critical to Sam’s treatment and is a hallmark of the AVB approach. Early in Sam’s treatment, his profile of abilities was mapped using the Assessment of Basic Language and Learning Skills (ABLLS) (Partington & Sundberg, 1998). (This instrument has been revised to form the Assessment of Basic Language and Learning Skills—Revised; Partington, 2006). The ABLLS provides very detailed assessment of a child’s mastery level in 25 important areas of functioning, including cooperation, visual performance, receptive language, expressive communication, imitation, play, social interaction, group participation, academic and school functioning, generalization, motor skills, and self-help (Table 22.2). Unlike standard neuropsychological assessment tools, the ABLLS does not provide norm-referenced standard scores but, rather, provides a grid on which the presence or absence of specific criterion-referenced skills within each domain are plotted. If a child possesses a particular skill, the small box for that skill is filled on the grid, color-coded to reflect the date of the assessment. Each item has multiple boxes that can be filled from left to right across the row to indicate the degree of Table 22.1 Behavioral classification of language Receptive Comprehending and following the instructions/requests of others. The tendency to ‘‘touch the red cup’’ when asked to do so. Echoic Repeating what is heard. The tendency to say ‘‘cup’’ after another person does. Imitation Copying the motor movements of another person. The tendency to sign ‘‘drink’’ after another person does. Tact Naming or labeling. The tendency to say ‘‘cup’’ because you see a cup. Mand Asking for desired items. The tendency to say ‘‘cup’’ because you want a cup. RFFC Receptive by Feature, Function, or Class: Receptively identifying items when the feature, function, or class is described. The tendency to point to the cup when asked ‘‘Which one do you drink milk from?.’’ TFFC Tact by Feature, Function, or Class: Expressively labeling items when the feature, function, or class is described and the item is present. The tendency to say ‘‘cup’’ when looking at objects including a cup and asked, ‘‘Which one do you drink milk from?’’ (Added in application after publication of source materials.) Intraverbal Answering questions or giving conversational responses when the response is determined by the words of another. The tendency to say ‘‘milk’’ when asked, ‘‘What do you like to drink?’’ when the milk is not in sight. Textual Reading written words. The tendency to say the word ‘‘cup’’ because you see the written word ‘‘cup.’’ Writing Writing words when they are spoken to you. A tendency to write ‘‘cup’’ when you hear it spoken. Adapted from Sundberg & Partington, 1998 and Skinner, 1957, 1992.

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Table 22.2 Skill areas assessed by the ABLLS (Partington & Sundberg, 1998) Scale Title/skill area A Cooperation and reinforcer effectiveness B Visual performance C Receptive language D Imitation E Vocal imitation F Requests G Labeling H Intraverbals I Spontaneous vocalizations J Syntax and grammar K Play and leisure L Social interaction M Group instruction N Classroom routines P Generalized responding Q Reading R Math S Writing T Spelling U Dressing V Eating W Grooming X Toileting Y Gross motor Z Fine motor

skill mastery or number of items acquired for that skill. Ratings can take many hours and involve parents, teachers, therapists, and others familiar with the child’s abilities. This information can guide initial treatment programming by identifying skills in need of development and assist in school Individualized Education Program (IEP) development by identifying target goals. Subsequently, repeat administrations are completed and plotted on the grid to demonstrate skill acquisition and continuing areas of need. Intervention techniques were individually developed to address each of Sam’s areas of skill deficit, both those identified by the ABLLS and others as they arose. The assessment was updated at intervals of approximately 6 months and the grid completed in different colors corresponding to administration dates to illustrate progress in each area over time (Tables 22.3–22.7). After 6 months of treatment, Sam’s mother wrote, ‘‘It has only been 6 months but the results are amazing. [Sam] now uses over 400 words and some short phrases. He sings songs and has begun to take a real interest in the world around him. These are things we never thought he might be able to do, but now we see small ‘miracles’ each day.’’

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Table 22.3 Steven’s skill development on receptive language C52 C51 C50 C49 C48 C47 C46 C45 C44 C43 C42 C41 C40 C39 C38 C37 C36 C35 C34 C33 C32 C31 C30 C29 C28 C27 C26 C25 C24 C23 C22 C21 C20 C19 C18 C17 C16 C15 C14 C13 C12 C11 C10 C9 C8 C7 C6 C5 C4 C3 C2 C1

Baseline

C52 C51 C50 C49 C48 C47 C46 C45 C44 C43 C42 C41 C40 C39 C38 C37 C36 C35 C34 C33 C32 C31 C30 C29 C28 C27 C26 C25 C24 C23 C22 C21 C20 C19 C18 C17 C16 C15 C14 C13 C12 C11 C10 C9 C8 C7 C6 C5 C4 C3 C2 C1

6 month progress

Previously Mastered Skill

C52 C51 C50 C49 C48 C47 C46 C45 C44 C43 C42 C41 C40 C39 C38 C37 C36 C35 C34 C33 C32 C31 C30 C29 C28 C27 C26 C25 C24 C23 C22 C21 C20 C19 C18 C17 C16 C15 C14 C13 C12 C11 C10 C9 C8 C7 C6 C5 C4 C3 C2 C1

12 month progress

C52 C51 C50 C49 C48 C47 C46 C45 C44 C43 C42 C41 C40 C39 C38 C37 C36 C35 C34 C33 C32 C31 C30 C29 C28 C27 C26 C25 C24 C23 C22 C21 C20 C19 C18 C17 C16 C15 C14 C13 C12 C11 C10 C9 C8 C7 C6 C5 C4 C3 C2 C1

18 month progress

C52 C51 C50 C49 C48 C47 C46 C45 C44 C43 C42 C41 C40 C39 C38 C37 C36 C35 C34 C33 C32 C31 C30 C29 C28 C27 C26 C25 C24 C23 C22 C21 C20 C19 C18 C17 C16 C15 C14 C13 C12 C11 C10 C9 C8 C7 C6 C5 C4 C3 C2 C1

2 year progress

Newly Acquired Skill

Throughout treatment, his skills continued to progress, with growth spurts in specific skills occurring at various times. In Receptive Language, Sam could initially understand and follow receptive instructions to point to items such as body parts and clothing. He made great progress during his first 6 months, obtaining receptive understanding of items such as identifying objects when given a function, feature, or class. He also quickly advanced in more advanced receptive skills, such as following instructions to go to a person, walk to a person and get something, go to a person and perform an action, identify common environmental sounds, and select items that go with other associated items.

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Table 22.4 Steven’s skill development on vocal imitation E9 E8 E7 E6 E5 E4 E3 E2 E1

Baseline

E9 E8 E7 E6 E5 E4 E3 E2 E1

6 month progress

E9 E8 E7 E6 E5 E4 E3 E2 E1

E9 E8 E7 E6 E5 E4 E3 E2 E1

12 month progress

18 month progress

E9 E8 E7 E6 E5 E4 E3 E2 E1

2 year progress

Newly Acquired Skill

Previously Mastered Skill

Table 22.5 Steven’s skill development on requests F27 F26 F25 F24 F23 F22 F21 F20 F19 F18 F17 F16 F15 F14 F13 F12 F11 F10 F9 F8 F7 F6 F5 F4 F3 F2 F1

Baseline

F27 F26 F25 F24 F23 F22 F21 F20 F19 F18 F17 F16 F15 F14 F13 F12 F11 F10 F9 F8 F7 F6 F5 F4 F3 F2 F1

6 month progress Previously Mastered Skill

F27 F26 F25 F24 F23 F22 F21 F20 F19 F18 F17 F16 F15 F14 F13 F12 F11 F10 F9 F8 F7 F6 F5 F4 F3 F2 F1

F27 F26 F25 F24 F23 F22 F21 F20 F19 F18 F17 F16 F15 F14 F13 F12 F11 F10 F9 F8 F7 F6 F5 F4 F3 F2 F1

12 month progress

18 month progress

F27 F26 F25 F24 F23 F22 F21 F20 F19 F18 F17 F16 F15 F14 F13 F12 F11 F10 F9 F8 F7 F6 F5 F4 F3 F2 F1

2 year progress

Newly Acquired Skill

While his motor imitation skills were strong at the outset of treatment, his vocal imitation skills advanced significantly during the first 6 months of treatment. A specific skill absent from his repertoire at the outset of therapy that he developed during treatment included imitating the prosody of words and phrases. His use of communication to make requests grew greatly throughout therapy. For example, between approximately the second and sixth months of therapy, the frequency of

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Table 22.6 Steven’s skill development on labeling G42 G41 G40 G39 G38 G37 G36 G35 G34 G33 G32 G31 G30 G29 G28 G27 G26 G25 G24 G23 G22 G21 G20 G19 G18 G17 G16 G15 G14 G13 G12 G11 G10 G9 G8 G7 G6 G5 G4 G3 G2 G1

Baseline

G42 G41 G40 G39 G38 G37 G36 G35 G34 G33 G32 G31 G30 G29 G28 G27 G26 G25 G24 G23 G22 G21 G20 G19 G18 G17 G16 G15 G14 G13 G12 G11 G10 G9 G8 G7 G6 G5 G4 G3 G2 G1

6 month progress Previously Mastered Skill

G42 G41 G40 G39 G38 G37 G36 G35 G34 G33 G32 G31 G30 G29 G28 G27 G26 G25 G24 G23 G22 G21 G20 G19 G18 G17 G16 G15 G14 G13 G12 G11 G10 G9 G8 G7 G6 G5 G4 G3 G2 G1

G42 G41 G40 G39 G38 G37 G36 G35 G34 G33 G32 G31 G30 G29 G28 G27 G26 G25 G24 G23 G22 G21 G20 G19 G18 G17 G16 G15 G14 G13 G12 G11 G10 G9 G8 G7 G6 G5 G4 G3 G2 G1

12 month progress

18 month progress

G42 G41 G40 G39 G38 G37 G36 G35 G34 G33 G32 G31 G30 G29 G28 G27 G26 G25 G24 G23 G22 G21 G20 G19 G18 G17 G16 G15 G14 G13 G12 G11 G10 G9 G8 G7 G6 G5 G4 G3 G2 G1

2 year progress

Newly Acquired Skill

his spontaneous verbal requests increased from approximately 15 to a range of 50– 85 during a 30-min period of activities intended to elicit requests. At baseline, he was able to request only when prompted and only for a few items. Over time, he developed abilities such as requesting using sentences, requesting that others stop activities or remove items, and requesting information (asking Wh- questions). Ultimately, he was making novel requests that had not been intensively taught and was requesting spontaneously. He advanced significantly in labeling skill as well, from primarily labeling nouns to labeling features of objects, adjectives, noun–verb combinations, and emotions. Socially, he demonstrated development in skills including showing interest in the behavior of others, eye contact, returning and initiating greetings, looking at others to start an interaction, and conversing. There was considerable variety in the areas addressed by Sam’s therapy, with the underlying goal of teaching skills that he could apply functionally in his life. After 3 years of intensive therapy, he had mastered or advanced in the ability to follow

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Table 22.7 Steven’s skill development on social interaction L22 L21 L20 L19 L18 L17 L16 L15 L14 L13 L12 L11 L10 L9 L8 L7 L6 L5 L4 L3 L2 L1

Baseline

L22 L21 L20 L19 L18 L17 L16 L15 L14 L13 L12 L11 L10 L9 L8 L7 L6 L5 L4 L3 L2 L1

6 month progress Previously Mastered Skill

L22 L21 L20 L19 L18 L17 L16 L15 L14 L13 L12 L11 L10 L9 L8 L7 L6 L5 L4 L3 L2 L1

L22 L21 L20 L19 L18 L17 L16 L15 L14 L13 L12 L11 L10 L9 L8 L7 L6 L5 L4 L3 L2 L1

12 month progress

18 month progress

L22 L21 L20 L19 L18 L17 L16 L15 L14 L13 L12 L11 L10 L9 L8 L7 L6 L5 L4 L3 L2 L1

2 year progress

Newly Acquired Skill

receptive instructions, participation and engagement skills necessary for ‘‘Circle Time’’ at school, the ability to identify helpful individuals in the community, the ability to request attention and seek approval, the skill of requesting information (i.e., asking questions), many conversational skills, some early preposition and pronoun skills, academic skills including advancement in phonics and reading, writing skills, game-playing skills, the ability to tell time, answering yes vs. no, improved ability to answer the question ‘‘what is wrong,’’ the ability to describe what he saw, and many self-help skills. Within the behavioral classification of language utilized in AVB, Sam advanced from being a child with little communicative language to demonstrating mastery of many hundreds of requests/mands, labels/ tacts, and 240 documented mastered intraverbals. He fluently requested what he wanted and was able to understand and functionally use directions. In addition, he was demonstrating improved connection to others, wanting to be included in activities and pictures and even negotiating about topics such as bedtime. Along with these substantial gains, he did continue to demonstrate particular difficulty sustaining his attention to activities as well as ASD symptoms including continued limitation in social interaction and spontaneous conversation along with the use of scripted language. Although recommendations and target goals specific to Sam during his intensive treatment are too numerous to list exhaustively, there are general recommendations that were shared with his parents and can be useful for most families with children involved in intensive ABA (Box 22.1). These recommendations can also be beneficial in ensuring the efficacy of most intervention models.

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Box 22.1 General Recommendations to Maximize the Efficacy of Intervention: l

l

l

l

l

Generalize skills to parents and other involved individuals. Parents should be instructed in the intervention techniques and should carry them over into daily life outside of therapy to maximize a child’s functional generalization of abilities. For problematic behavior, assess the function of the behavior in order to formulate an effective intervention. Determining what reinforces or supports behavior will lead to more effective strategies for changing it. For example, if aggression serves the function of seeking attention, responding to it may reinforce the aggression whereas ignoring it (when possible) may be a more effective response. Coordination between therapy providers is essential. A child with ASD typically receives a multitude of educational, behavior, speech, occupational, and other therapies. Be sure that any conflicting advice is resolved and that various individuals in the child’s life are responding consistently. Whenever possible, make only one change in a child’s intervention at a time. It is common to see a ‘‘shotgun’’ approach in which dietary, medication, behavior, and other strategies are all initiated simultaneously, making it impossible to determine which intervention(s) are having the observed impact. Collect data on goals to ensure that benefit is derived from intervention rather than relying on subjective reports or feelings to determine progress.

Sam’s family faced another harsh, but common, reality: intensive ABA therapy is, due to the large number of hours, expensive. During his intensive treatment, Sam typically received between 20 and 30 hours of weekly therapy. Despite research suggesting the long-run cost-effectiveness of intensive ABA (Jacobson, Mulick, & Green, 1998), public funding is limited. Sam’s therapy hours were reduced after 1–1½ years due to governmental budget cuts, and his eligibility for funding of intensive services ended after 3 years.

Update Sam’s gains have been maintained, and he continues to receive a small number of hours of therapy and consultation. His outcome and ongoing maintenance of gains are very positively influenced by the active involvement of his family in utilizing ABA techniques to sustain and advance his skills. In the most recent contact with Sam’s family, 5 years after beginning ABA therapy and nearly 2 years after the intensive treatment ended, his mother indicated that he is more communicative than ever, plays with his sister, and attends school. However, Sam continues to have an ASD and to require aide support in school, illustrating that although intensive ABA dramatically improved his skills, it does not provide a cure. Despite tremendous gains in making Sam able to communicate functionally, his language, like that of many verbal individuals with ASD, is not age-typical. Speech and language evaluation indicated language functioning more typical of a 3-yearold at the chronological age of 7 years and 3 months. Similarly, despite mastery of

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many skills that Sam may not have learned without intervention, his adaptive skills remain impaired for his chronological age. This combination of hope and loss is evident in the words of Sam’s mother: ‘‘Autism is a disorder that steals a child’s personality and ability to relate to the world around him. This type of therapy is the only real hope for these children and their families. It is the only thing that has let us look forward to a brighter future. ’’

Additional Resources Key Concepts and Terms Applied Behavior Analysis (ABA) A field utilizing the principles of behavior to build socially useful repertoires and often to reduce inappropriate ones. Applied Verbal Behavior (AVB) A program or model of ABA utilizing Skinner’s analysis of verbal behavior and highly effective teaching procedures from ABA to teach verbal behavior. Assessment of Basic Language and Learning Skills (ABLLS) A criterion-referenced assessment of important skills in 25 domains that provides detailed information regarding the skills a child possesses or lacks, allowing treatment providers to more precisely target needed skills and to document treatment progress through regular readministration (Partington & Sundberg, 1998). The updated version is the ABLLS-Revised (Partington, 2006), and the Imitation (motor) and Vocal Imitation scales have been expanded to include substantially more skills. Autism Spectrum Disorder (ASD) Disorders falling under the category of Pervasive Developmental Disorders intended to communicate that such disorders vary along a spectrum of symptom presentation and severity.

References Resources for Clinicians Cohen, H., Amerine-Dickens, M., & Smith, T. (2006). Early intensive behavioral treatment: Replication of the UCLA model in a community setting. Journal of Developmental and Behavioral Pediatrics, 27, 145–155. Cooper, J. O., Heron, T., & Heward, W. (1989). Applied behavior analysis. Columbus, OH: Merrill. Jacobson, J. W., Mulick, J. A., & Green, G. (1998). Cost-benefit estimates for early intensive behavioral intervention for young children with autism—General model and single state case. Behavioral Interventions, 13, 201–226.

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Partington, J. W. (2006). The assessment of basic language and learning skills—revised. Pleasant Hill, CA: Behavior Analysts. Partington, J. W., & Sundberg, M. L. (1998). The assessment of basic language and learning skills. Pleasant Hill, CA: Behavior Analysts. Sallows, G. O., & Graupner, T. D. (2005). Intensive behavioral treatment for children with autism: Four-year outcome and predictors. American Journal on Mental Retardation, 110, 417–438. Skinner, B. F. (1992). Verbal behavior. Acton, MA: Copley. (original work published 1957) Sundberg, M. L., & Partington, J. W. (1998). Teaching language to children with autism or other developmental disabilities. Pleasant Hill, CA: Behavior Analysts.

Resources for Families Autism Society of America, http://www.autism-society.org Carbone, V. J.; Workshop schedule, http://www.drcarbone.net/schedule.aspx Different Roads to Learning, http://www.difflearn.com. Catalog of teaching materials. Maurice, C. (Ed.). (1996). Behavioral intervention for young children with autism: A manual for parents and professionals. Austin: Pro-Ed. Verbal Behavior Teaching Materials Resource, vbteachingtools.com

Chapter 23

When Quirks and Quick Learning Create a Quandary: Mild Autism Elizabeth N. Adams

There was nothing Molly enjoyed more than a day at the zoo with her mother. Immediately after entering the gates, she would run to the ‘‘Big Cats’’ house, dragging her mother along. Molly was just like the other Big Cat loving kids, because she relished watching the powerful animals prowl among the rocks and hearing them make those big gut-busting growls just before lunchtime. But, shortly after her third birthday, Molly’s very observant mother noticed that she was different from the other Big Cat loving kids in some ways as well. When the other kids saw the leopards, their wide-eyed facial expressions would turn quickly to their parents’ faces and then back and forth between their faces and the cats. The children’s tiny index fingers would automatically extend, as if instinctually pointing to show these intriguing animals, to share the moment with their parents. Although she felt excited inside just like the other kids, Molly’s external response was very different. She did not attempt to share the experience with her mother by looking or pointing; rather, she stared intently at the animals and sometimes tapped two of her fingers together or made a clicking noise with her mouth. Molly’s mother observed that she seemed like she was in her own world during these times, ‘‘as if her wheels were turning constantly.’’ Her mother soon learned at least part of why those wheels were turning. One day Molly approached the exhibit and was able to accurately label the species name and country of origin of each cat, from memory! At 3 years of age, she had developed quite an extensive knowledge within this area of interest that seemed somewhat precocious. Interestingly, Molly demonstrated advanced knowledge and understanding in other areas as well. Her preschool teacher observed that she seemed to have an uncanny knack for learning and remembering the alphabet. As an illustration, one day while Molly was eating breakfast, she said (with her mouth full of cereal), ‘‘e-l-e-p-h-a-n-t spells elephant!’’ In the context of these vigorous interests and abilities, Molly’s parents had become concerned about her around 2 years of age, when she did not seem to be interested in playing with other children. They observed that she was ‘‘a little

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different’’ and that she seemed to be ‘‘in her own world’’ most of the time. She seemed content to engage in her own play while playing beside other children, but she did not engage in cooperative or interactive play. She did not initiate or maintain consistent eye contact with other children or with her parents. As they became more concerned about her lack of social engagement, Molly’s parents thought back and realized that from an early age, she had rarely expressed a desire for anything. She had never used pointing to show them objects of interest or to request treats. Her parents recalled that they had initially seen her as being laid back or independent but that they were more concerned now that her lack of interest or initiative seemed to be impairing most age-appropriate activities. Because of their concerns, Molly’s parents enrolled her in several communitybased activities that would provide her with opportunities for social interaction. However, despite their attempts to expose her to new experiences and encourage her participation in social settings, her social difficulties persisted. At that point, Molly’s parents consulted with her pediatrician to find more formal intervention for her issues. The pediatrician recommended that she should undergo a neuropsychological evaluation, which would assess Molly’s thinking and learning abilities as well as her social and behavioral functioning to assign an appropriate diagnosis and provide recommendations for intervention. At the time of the evaluation, Molly was aged 3 years and 11 months. The neuropsychologist conducted an extensive clinical interview with her parents to gather more specific information about their concerns. The following information was presented to the neuropsychologist and was later used to formulate a diagnosis and develop a plan for intervention. Molly was an 8-pound and 8-ounce baby following an uncomplicated pregnancy. Delivery occurred 1 week after the due date; labor was induced with oxytocin. There were no complications during the delivery. She was a healthy infant, and had no difficulties with feeding or sleeping. Her parents reported no chronic or acute medical problems. She was not prescribed any medications at the time of the evaluation. All motor milestones were attained at a normal rate; she was toilettrained during the day and night at age 3 years and 6 months. Although early developmental milestones for language were attained at a normal rate (i.e., ‘‘mama’’ and ‘‘dada’’ at 10 months, single words at 12 months), by 24 months Molly’s vocabulary consisted of only 10 words. Because of concerns about this delay, she underwent an evaluation with a speech and language therapist. She attended six sessions of speech and language therapy and made some (though minimal) progress. Then, suddenly during a family vacation her vocabulary increased from 10 words to over 100 in the span of several days, and since that time, her vocabulary development had been, in fact, quite precocious. However, Molly’s parents did report some oddities in her use of language, most notably that she tended to ‘‘parrot’’ a lot of what was said to her. Often, when given a verbal instruction, she would repeat the last phrase of the instruction rather than follow it. Thus, her language development was considered atypical, and she continued to have some difficulties using her adequate vocabulary for the purpose of communicating with others.

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Molly’s parents described her play as very structured and almost obsessive, and they stated that she did not engage in spontaneous pretend or make-believe play. For example, she went through a phase during which she enjoyed playing with dolls, but she did not create stories or pretend to have her dolls interact but rather lined the dolls up very carefully and became upset if one of the dolls was moved from its position. Also, her parents bought her a kitchen center, in hopes of encouraging her to engage in pretend play with a lifelike activity, but she did not pretend to use the kitchen to cook anything and instead repetitively organized the dishes. Molly’s parents reported that she thrived on routine and became very upset when the routine she had intended was changed. They were especially baffled by this, because they had intentionally exposed her to a wide variety of environments, people, and activities during her early childhood in hopes of encouraging flexibility. They did not follow a strict routine themselves; rather, Molly seemed to impose routine on her own. When under stress or apparently concentrating very hard, Molly often made odd movements with her fingers. She would hold three fingers up and look at them for several seconds, and she would tap two fingers together on both hands simultaneously. Also, she sometimes made a ‘‘ticka-ticka’’ noise repetitively until asked to stop. Molly had not successfully demonstrated use of a computer mouse but seemed to be obsessed with rolling the track ball back and forth. Her parents also expressed concern about her tendency to eat nonedible items such as worms, bugs, sand, and sidewalk salt. They felt that she seemed to eat these items intentionally. In addition to Molly’s previously described proclivity for knowledge about animals and words, she also demonstrated an impressive ability to memorize sections from stories and movies but did not seem to understand the overall plot. For example, she could recite sections from the Lion King but did not understand who was good and evil. Also, her favorite book was Where the Wild Things Are, and she could anticipate and recite specific sentences from the book, but she did not seem to understand what happened to the boy in the book. Molly’s parents were concerned about her inconsistent following of instructions. They noted that she seemed to hear what was said and would respond appropriately sometimes, but she often behaved as if she was not aware that someone had given an instruction. They observed that she tended to respond more consistently when in a one-to-one situation, where she received multiple prompts to follow through with the instruction. Molly’s parents enrolled her in a 3K classroom, hoping that consistent participation with same-age peers in a structured setting would help to facilitate development of social skills. They noted that they were not concerned about her academic skills, because they perceived that she had demonstrated age-appropriate achievement in that area. However, per parent and teacher report, Molly had not, thus far, succeeded socially in her classroom and continued to have problems with compliance most days. Molly’s teacher and her parents observed that she did not seem to be bothered by her difficulties in the classroom. She seemed somewhat oblivious to those around her and seemed to be in her own world.

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Assessment The assessment was conducted over four sessions. The first session involved Molly’s parents for the clinical interview described earlier. For the second session, Molly participated in neuropsychological testing for about 90 min and then played with her mother while the neuropsychologist observed her play behaviors. In the third session, Molly finished her neuropsychological test battery and then played with her father while being observed. During the fourth session, Molly’s parents met with the neuropsychologist to discuss feedback on the test results, diagnostic formulation, and recommendations. Molly hid behind her mother when introduced to the examiner, and did not cooperate when asked to separate from her mother for the testing session. Her mother accompanied her to the testing area, and with some positive motivational techniques, Molly was able to separate to begin the testing. She fidgeted often during testing and was easily distractible but responded well when redirected to the task. She echoed many of the examiner’s phrases verbatim throughout the evaluation. Sometimes, when asked a question, she repeated the question instead of answering it. For example, when asked how she was doing, she responded, ‘‘How are you doing?’’ in the same prosody the examiner had used. She was also observed to speak in a manner that sounded similar to speech one might use if teaching. For example, when given a triangle-shaped block, she stated, ‘‘Triangle. Let’s count the sides: 1, 2, 3.’’ After saying this, she did not indicate by looking at or showing that she was interested in a response from the examiner but rather continued to repeat the phrase with the same prosody until redirected. As was later corroborated by her mother, it was possible that phrases like this were phrases Molly had remembered from school or educational videos. Throughout testing, she appeared to enjoy the presentation of each new task and the accompanying stimuli; however, she did not share her enjoyment with the examiner. She made eye contact only minimally and did not use her facial expressions or other gestures to communicate with the examiner. In spite of this constellation of odd behaviors and minimal social interaction, her attention to each task and her compliance with instructions were, for the most part, adequate to ensure that the findings reported were a valid estimate of her current abilities.

Test Results Molly’s neuropsychological test battery was designed specifically to assess her core cognitive ability, as well as more specific domains of functioning (e.g., language, attention, executive functioning, visual-spatial perception, and motor performance) that have been found to be salient during her particular stage of development. In addition to formal tests of cognitive functioning, social and behavioral functioning was assessed by observing her during two separate interactions with each parent. Assessment of general cognitive abilities was performed using the Differential Abilities Scale – Preschool Version (Table 23.1). On this measure, Molly’s perfor-

23 When Quirks and Quick Learning Create a Quandary: Mild Autism Table 23.1 Intellectual Differential Abilities Scale – Preschool Version General conceptual ability score Verbal cluster Verbal comprehension Naming vocabulary Nonverbal cluster Picture similarities Pattern construction Copying Early number concepts a Standard score, (scaled score), [T-score], {z-score}.

Scorea 89 85 [29] [53] 92 [29] [48] [62] [47]

235

Percentile 23rd 16th 2nd 62nd 30th 2nd 42nd 88th 38th

mances combined to a composite intellectual ability score in the average range, with comparable verbal and nonverbal composite scores. Of note, the pattern of her performances on subtests demonstrated a relative strength (average performances) on tests that require concrete or often-practiced skills (e.g., labeling single objects, copying shapes, counting, and constructing designs), when compared with weaker (moderately impaired) performances on a test that required more flexible reasoning skills (e.g., indicating analogies between pictures) and a test that required following multistep instructions. These findings were consistent with her parents’ report of her impressive ability to remember specific facts but her marked difficulty in following instructions and solving simple problems. Molly’s behavioral responses were not ideally conducive on a fine motor dexterity test. Her performances were well below average bilaterally, likely indicating a combination of less-than-optimal compliance and possible fine motor control difficulties. She did not comply with the instructions on a fine motor speed planning task, resulting in above average time scores but below average accuracy scores. Performance on this particular task was considered to be indicative of her difficulty following instructions, rather than fine motor precision. She was able to imitate seven hand positions with each hand, yielding scores in the above average range bilaterally. Taken together, results of formal tests of motor functioning were variable and more representative of behavioral compliance than motor control. There were no indications of lateralized motor dysfunction. Formal tests of attention and executive functioning are relatively well-established for school-aged children and adolescents. However, assessment of these areas in preschool-aged children is at an earlier stage of refinement. Thus, some of the measures used in assessment of these functions remain in the experimental phase and were interpreted with caution (Table 23.2). Molly’s performance on a test of visual attention was average for both time and accuracy (Table 23.3). Her performance on a test of sustained impulse control, which required her to stand still like a statue in spite of several sound distracters, was slightly below average. On a measure of her ability to learn an alternating pattern of rewards, she demonstrated some understanding of the concept of searching in alternate wells for a reward and correctly anticipated the switch three times, which was slightly below average compared with other children of her age.

236 Table 23.2 Academic achievement Bracken Basic Concept Scale – Revised School readiness composite

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Score 124

Table 23.3 Other cognitive functions Tests Purdue Pegboard Preferred hand Nonpreferred hand Both hands NEPSY Sensorimotor domain Imitating hand positions Preferred hand Nonpreferred hand Visuomotor precision Train Time Errors Car Time Errors Attention/executive scale Visual attention Bunnies Time Omissions Commissions Cats Time Omissions Commissions Statue Delayed alternation Luria’s Tapping Test Clinical Evaluation of Language Fundamentals – Preschool Receptive language Sentence structure Concepts and following directions Basic concepts Expressive language Word structure Expressive vocabulary Recalling sentences Language content Language structure Total language

Percentile 95th

Score

Percentile

67 89 73

1st 23rd 4th

94 (14)

34th 91st >75th >75th 2nd

(4)

Age equivalent 5–1

Age equivalent

>75th 3rd–10th

91 (11)

>75th 3rd–10th 27th 63rd 26th–75th 26th–75th 11th–25th

(6) 82 80

>75th 11th–25th 11th–25th 9th 10th 9th

85 (2) (10) (10) 85 (7) (7) (8) 95 75 73

16th <1st 50th 50th 16th 16th 16th 25th 37th 5th 4th

<3–0 3–11 3–9 <3–0 <3–0 3–2

(continued)

23 When Quirks and Quick Learning Create a Quandary: Mild Autism Table 23.3 (continued) Tests Kaufman Assessment Battery for Children Gestalt closure Beery-Buktenica Developmental Test of Visual-Motor Integration Vineland Adaptive Behavior Scales – Second Edition Communication domain Receptive Expressive Written Daily living skills domain Personal Domestic Community Socialization domain Interpersonal relationships Play/leisure Coping Motor skills domain Gross Fine Adaptive behavior composite Achenbach Child Behavior Checklist Parent form Emotionally reactive Anxious/depressed Somatic complaints Withdrawn Sleep problems Attention problems Aggressive behavior Teacher’s report form Emotionally reactive Anxious/depressed Somatic complaints Withdrawn Attention problems Aggressive behavior

Score

Percentile

(9) 88

37th 21st

100

50th

237

Age equivalent

3–0

2–2 3–1 5–2 85

16th 2–9 3–5 3–1

66

1st 1–4 1–0 1–6

81

10th 2–4 3–2

80

9th

[55] [50] [50] [82] [53] [51] [58]

69th 50th 50th >97th 62nd 54th 79th

[66] [59] [50] [61] [64] [64]

95th 81st 50th 87th 92nd 92nd

Importantly, she got stuck on one (erroneous) response for seven consecutive trials and the beginning of the task. In other words, she continued to search in the same well seven times despite the fact that she did not find a reward in that well. On a measure of her ability to learn a rule set in response to the examiner’s tapping a certain number of times, she did not consistently demonstrate understanding of the more simple portion of the task. Instead of tapping according to the assigned rule set, she copied the examiner’s number of taps. Thus, overall, Molly’s approach to

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formal tests of attention and executive functioning corroborated her parents’ description of her difficulty maintaining consistent and appropriate responses when in situations that required flexibility. On a test of developmental language functioning, Molly demonstrated evenly developed (average) levels of performance on the receptive and expressive language composite, indicating that her basic use and comprehension of language was generally consistent with what would be expected given her age. However, she demonstrated below average performance on the language structure composite, indicating that the manner in which she constructed phrases and sentences was not as well developed as what would be expected given her age and her intact basic language functioning. This, again, was consistent with her parents’ report. Though she demonstrated intact vocabulary and labeling abilities, Molly had more difficulty in situations that required her to express thoughts and feelings using sentences. Overall, her basic language skills were intact, but her pragmatic use of those skills was delayed. Molly’s performance on a test of visual perception, in which she was asked to match increasingly complex geometric designs, was average. Preacademic skills were assessed using the Bracken School Readiness Assessment. Molly’s performance on this measure, which assessed her knowledge of colors, letters, numbers, sizes, comparisons, and shapes, was above average. Molly’s parents’ ratings of her adaptive functioning combined to form a score that was below average in comparison with her same-age peers. A significant discrepancy between skill levels was apparent on this measure, with communication skills as a relative strength, in the average range, and socialization skills as a relative weakness, in the significantly below average range. Of note, the communication subscale score was relatively higher because of above average written communication skills, but expressive and receptive communication skills were rated as lower. Daily living skills and motor skills were rated to be slightly below average. The two play interactions were included to provide an opportunity for observation of Molly’s play behaviors and social interaction skills. The interaction with the primary caregiver was more structured, including a snack and a specific task to complete, and the interaction with the secondary caregiver was unstructured, including only free play with toys. This design allowed for comparison of Molly’s behavior across two different settings, with varying levels of stress. First, Molly and her mom were given a snack to share together. Her mom asked, ‘‘Where does Mom sit?’’ and Molly responded, ‘‘There,’’ but did not look or point to indicate what she meant by this. Her mom asked again, and Molly gave the same response. Then her mom asked a third time, and Molly said, ‘‘There’’ while pointing to a chair, but she did not look to her mom or the chair. Molly and her mom were given several puzzles to complete together. As they worked on the puzzles, her mom asked, ‘‘How’s it go?’’ and Molly repeated this several times in sing-song prosody. She showed interest in the puzzles and accepted her mom’s help, but she did not make eye contact with her and did not use nonverbal gestures to ask for help. As they worked together, her mom created conversation and Molly made verbal responses but no spontaneous addition to the conversation. Rather, she pointed and labeled colors and animals in the puzzles.

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During a puzzle depicting social situations, Molly did not seem to understand the scenarios and did not independently succeed at completing it. Her mom asked concrete leading questions about specific details of the pictures to help Molly succeed. When they transitioned together to a shape-sorter puzzle, Molly demonstrated interest in the shapes. She labeled a pentagon, and said, ‘‘Triangle. A triangle has three sides: 1, 2, 3,’’ as if repeating something she had heard previously. Throughout this structured task, Molly protested verbally quite often, saying, ‘‘No, no!’’ in a loud voice when her mom made suggestions, but her emotion and behavior did not match. She complied easily and quickly after saying, ‘‘No, no!’’ Molly engaged in very minimal eye contact with her mom throughout this interaction. When she did look to her mom she seemed to be looking through her, rather than sharing an interaction with her. She demonstrated frequent use of echolalia, repeating words her mom had spoken, with similar prosody. She engaged in intermittent interactive communication with her mom, but her communication included labeling and repetition of phrases heard earlier (either immediately previous or somewhat delayed) rather than spontaneous initiation of conversation. Her social interaction was limited and somewhat scripted. Overall, Molly’s affect was limited and she demonstrated no emotional reciprocity in her interactions with her mom throughout this observation. Molly and her dad were given several boxes of toys to play with together. A doll house was set up on the table, and Molly immediately took the blocks that had formed the house and stacked them as if they were individual shapes rather than a pretend house. She repetitively stacked the blocks for several minutes and then abandoned this without apparent completion or closure to the task and walked to the other side of the room, pulled a puzzle out of a box, put it on the table but did not attempt to complete it. Her dad encouraged her to complete the puzzle, asking leading questions and structuring the task for her. She labeled one shape as a pentagon, but it was in fact an octagon and her dad explained this to her. She frequently repeated the phrase, ‘‘A g-reen t-riangle,’’ without apparent meaning and continued repeating this phrase until she was redirected. Molly then pulled toy mountains out of the dinosaur toy box and placed them carefully on the table. Instead of taking dinosaurs out to have them play on the mountains, Molly carefully lined up toy rocks on the mountains. Then she stood back, paused, raised her arms briefly with her fingers in stereotyped positions, and continued lining up toy rocks. Throughout this interaction, Molly did not initiate conversation or joint attention, but she engaged and seemed to be most comfortable in scripted speech interactions with her dad. When her dad would attempt to solicit spontaneous speech or conversation, she would not respond appropriately and sometimes engaged in repetitive behaviors that suggested she felt stress. Molly repeated the ends of her dad’s phrases several times, getting stuck by certain phrases. She made no eye contact with her dad and did not engage in any spontaneous make-believe play throughout this interaction.

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Formulation and Recommendations Having gathered information from the clinical interview, behavioral questionnaires, formal testing, and behavioral observation, the neuropsychologist integrated multifaceted findings from Molly’s evaluation. The following paragraphs describe the formulations regarding cognitive functioning and diagnostic impressions regarding behavior. Recommendations are described in the final paragraphs and were discussed with her parents during a formal feedback session. Results of formal testing indicated that Molly’s overall cognitive ability level was average in comparison to her same-age peers, with evenly developed verbal and nonverbal reasoning abilities. Results of formal tests of motor functioning were variable and more representative of behavioral noncompliance than motor control. There were no indications of lateralized motor dysfunction, meaning that one hand was not significantly worse that the other hand at performing these tasks. Her scores on formal tests of attention and executive functioning were somewhat lower than what would be expected for her age. She demonstrated average language functioning overall, but qualitative observation and formal testing indicated less welldeveloped use of structure in language, difficulties with pragmatic use of language for conversation, and very frequent idiosyncratic use of language. Adaptive behavior functioning was assessed by her parents’ report and revealed a significant discrepancy between communication skills as a relative strength (average, reflective of well above average alphabet knowledge) and socialization skills as a relative weakness (significantly below average). Molly’s current behavioral profile was consistent with a diagnosis of autism. This diagnosis requires evidence of impairment in three primary areas: (1) social functioning and an interest in social relationships; (2) communication (including language and communicative intent); and (3) repetitive or stereotyped interests or behaviors. Research with very young children suggests that the initial indicators of the autistic spectrum disorders include lack of consistent use of pointing to show objects of interest or to request objects out of reach, lack of eye contact, and inconsistent response to their names. For Molly, impairment in social functioning was evidenced by her inconsistent use of eye contact, inconsistent response to her name, lack of emotional reciprocity, preference to play alone rather than with others, and subsequent lack of developmentally appropriate peer relationships. Impairment in communication was evidenced by a lack of demonstrated ability to use her generally intact vocabulary to engage in interactive conversation. Her stereotyped use of language (e.g., immediate and delayed echolalia), lack of use of nonverbal gestures for communication (e.g., pointing to show), and lack of spontaneous make-believe play fall under this category as well. Finally stereotyped patterns of behaviors and restricted patterns of interest (reported by her parents and observed during evalua-

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tion) included odd motor mannerisms with her fingers, a repetitive ‘‘ticka-ticka’’ noise, excessive interest in animals and letters (to the exclusion of other developmentally appropriate and interactive play), persistent adherence to routine, and a tendency to seek out and eat nonedible items. Within psychology, both clinicians and researchers continue to discuss specific diagnostic delineations along the spectrum of Pervasive Developmental Disorders. Autism, being the most ‘‘classic’’ of these diagnoses, has received the most attention over the years. Asperger’s Disorder, another diagnosis along the same spectrum, is very similar in some ways to autism. The two diagnoses are so similar that professionals continue to debate whether they should be described as separate entities or whether one actually fits better as a subset of the other. This debate becomes especially relevant when clinicians observe children like Molly, who actually fit some of the criteria for both disorders. Because Molly’s basic language functioning was relatively intact, some might question whether she met diagnostic criteria for Asperger’s Disorder instead of autism. Diagnostic delineation is an important task that is necessary for continued growth in psychology. Nevertheless, the most crucial part of the clinician’s job is to provide truthful, meaningful, and helpful information to the patient and the patient’s family. Thus, the primary task for helping Molly and her parents was to provide the most accurate diagnosis possible, acknowledging the limitations of the diagnostic process, while relying on the strengths of the process to understand Molly’s behavior and develop appropriate recommendations. In light of this, the neuropsychologist explained to Molly’s parents the areas in which Molly’s presentation did not fit into the diagnostic criteria. It was emphasized that even though Molly demonstrated sufficient impairment in social interaction to meet diagnostic criteria for autism, she demonstrated interest in attending to some of her parents’ activities, and she participated in simple interactions with them. However, many of her interactions with her parents and much of her language use were stereotyped and repetitive. Also, although her impairment in using gestures to regulate social interaction met diagnostic criteria for autism, she did look to caregivers at times, though her eye contact was fleeting and not as functional as would be expected for a child of her age. These aspects of her social interaction suggested that she would likely respond well to treatment. Also, one of Molly’s relative strengths was her ability to learn through repetition and practice. She demonstrated knowledge and application of concrete skills (especially early preacademic skills). Other skills (e.g., pointing to show, making eye contact, using her language to engage in interactive conversation) did not seem to come naturally for her. Thus, it was expected that, through therapy and ongoing support from her parents, Molly would continue to make gains, especially in skills that can be learned through concrete training and practice. Given the results of this evaluation, it was recommended that Molly begin participating in applied behavioral analysis (ABA) therapy, sometimes referred to as Lovaas therapy. This involves 20–40 hours per week of intensive one-on-one

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behavioral modification to work on her communication, social, cognitive, and emotional development, with the goal of allowing her to more effectively participate in developmentally appropriate structured situations, especially school classrooms. As an alternative or adjunct treatment, Molly’s parents were also encouraged to look into an additional form of therapy called Floor Time, which uses the child’s play to teach her to communicate with others. By mirroring the child’s actions, the therapist eventually teaches the child that she has an impact on other people and can change or alter the actions of others, based on her own. Floor Time therapy then builds on this fundamental principle of communication and teaches parents how to develop more functional interactions with their child, based on play. Speech and language therapy services were recommended. Molly had participated in speech and language therapy in the past, but when she made adequate progress in vocabulary development, it was determined that she no longer needed services. Although Molly’s basic receptive language and vocabulary were intact, she demonstrated impairments in her ability to use language to communicate with others. At one level, these impairments were related to her idiosyncratic use of language, but Molly demonstrated difficulty with the pragmatic and structural aspects of language beyond her idiosyncratic communication style. For these reasons, it was recommended that speech and language therapy services be recommenced.

Additional Resources Key Concepts and Terms Social Engagement During the preschool years, social engagement facilitates the child’s development on multiple levels, including language and cognitive functioning as well as interpersonal relationships. Some indications of social engagement at these early ages are joint attention (i.e., pointing or using eye contact to share the experience of seeing something with another person) and social referencing (i.e., looking to another for affirmation regarding an action the child is about to perform). Applied Behavioral Analysis (ABA) Therapy A technique that uses repetition, behavioral reinforcement, and concrete rewards to help the child learn and incorporate more adaptive communication skills into his or her social interactions, while attempting to minimize the frequency of less adaptive behaviors. Floor Time Therapy A technique developed by Stanley Greenspan, M.D., in which the therapist follows and attempts to mimic the child’s play in hopes of teaching the child through experience that his or her actions can impact the behavior of another person. This approach emphasizes relationship and interaction with the overall goal of facilitating more adaptive interpersonal skills.

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References Resources for Clinicians Filipek, P. A., Accardo, P. J., Baranek, G. T., Cook, E. H., Dawson, G., Gordon, B., et al. (1999). The screening and diagnosis of autistic spectrum disorders. Journal of Autism and Developmental Disorders, 29, 439–484. Macintosh, K. E., & Dissanayake, C. (2004). Annotation: The similarities and differences between autistic disorder and Asperger’s disorder: A review of the empirical evidence. Journal of Child Psychology and Psychiatry, 45, 421–434. Toth, K., Munson, J., Meltzoff, A., & Dawson, G. (2006). Early predictors of communication development in young children with autism spectrum disorder: Joint attention, imitation, and toy play. Journal of Autism and Developmental Disorders, 36, 993–1005. Volkmar, F. R., Lord, C., Bailey, A., Schultz, R. T., & Klin, A. (2004). Autism and pervasive developmental disorders. Journal of Child Psychology and Psychiatry, 45, 135–170. Wetherby, A. M., Woods, J., Allen, L., Cleary, J., Dickinson, H., & Lord, C. (2004). Early indicators of autism spectrum disorders in the second year of life. Journal of Autism and Developmental Disorders, 34, 473–493.

Resources for Families Attwood, T. (2006). The complete guide to Asperger’s Syndrome. London: Jessica Kingsley. Autism Society of America, http://www.autism-society.org Baron-Cohen, S. (1993). Understanding other minds; perspectives from autism. London: Oxford Medical.

Chapter 24

On Eggshells: Pediatric Bipolar Disorder Jennifer Niskala Apps and Dawn Pflugradt

Jeff was referred for a neuropsychological evaluation because of longstanding concerns regarding his cognitive and emotional development. In fact, Jeff’s family had been searching for diagnostic clarification and treatment since he was an infant. Jeff’s mother noticed something was ‘‘wrong’’ with him in early infancy, although her pregnancy and delivery were without complication. He was described as an infant who cried frequently and was not responsive to touch. Early assessments identified Jeff as being approximately 3–6 months behind in many of his developmental abilities. He was placed in a Birth-to-Three program where he received cognitive, physical, occupational, and speech therapy. During the course of these services, Jeff reportedly normalized in his developmental process as a toddler. However, already he was demonstrating violent tantrums. Specifically, Jeff exploded with sudden, violent mood swings directed toward family members and pets, and at an early age, he began fire setting. At age 5, his behaviors made attending school difficult, as he was often sent home. Jeff’s family often felt they were walking on eggshells around him, because of his unpredictable emotional reactions and aggression. During one outburst, Jeff’s pregnant mother had to physically restrain him as he was threatening to kill her and his unborn sibling. This prompted the family’s first attempt to get him psychiatric services. Jeff was initially diagnosed with Attention Deficit Hyperactivity Disorder (ADHD) and was prescribed risperidone. After starting the medication, he reportedly did not sleep for 3 weeks and began hallucinating. He was placed in his first psychiatric hospitalization, during which he was prescribed dextroamphetamine sulfate. Again he experienced hallucinations and a decreased need for sleep and was eventually placed on divalproex. Approximately, at age 7, Jeff threatened to hurt himself and his family. The police were called, and it took several family members and police officers to restrain him in order to transport him to the psychiatric hospital. Following this hospitalization, during which more medications were added to his treatment regimen, Jeff had an incident, which his mother described as appearing seizure-related. While waiting for the school bus, he stated that he did not feel good, stiffened, and lost consciousness briefly. He was taken by ambulance to the hospital.

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Magnetic resonance imaging (MRI) and computerized tomography (CT) scans were normal, and no seizure activity was diagnosed. He was released relatively quickly, with a decrease in his ongoing medications. Interestingly, his mother reported Jeff experienced several months of normalcy after this event, with no tantrum behaviors and a more stable mood. However, over several months his emotional and behavioral symptoms returned, including his combative tantrums, and medications were once again increased. One evening when Jeff was 9 years of age, he was struggling with his homework. His mother tried to help him, while also encouraging him to take breaks and attempt to manage his frustration level. However, on this night, Jeff was unable to moderate his emotional highs and lows. He became unbearably angry and in a fit of hostility charged out of the house. His mother ran to the window in time to see Jeff emerge from the garage with a sledgehammer and head for the house. Fearing for her life, his mother fled from her own child, leaving him at home and running to the police. At this point, Jeff was hospitalized and then placed in a group home, where he has remained and earns visitation with his family. Jeff was eventually diagnosed with Bipolar Disorder, a pattern that runs in his mother’s side of the family and for which she had also been treated. However, despite diagnosis and various medication treatment combinations, he continued to experience significant emotional and behavior problems both at the group home and with his family on visits. Jeff had frequent mood swings, irritability, anger, aggressive outbursts, as well as periods of time when he appeared depressed. His depressed moods could last from several minutes to many days. During the depressive episodes, Jeff reportedly displayed no interest in activities and would become morbid. His thought processes were tangential and circumstantial, with excessive or constant talking and pressured speech. Additionally, he had a history of sleep difficulties when off medication, with an average of 3–4 hours of sleep a night followed by constant energy throughout the day and no apparent fatigue. Socially, Jeff had difficulty making friends. His dangerous and reckless behaviors often alienated other children. Academically, Jeff started an Individualized Education Program (IEP) for emotional and behavioral disability when he was in first grade. He had advanced to the fifth grade but worked at a much lower grade level. Most of his accommodations and modifications educationally were impacted by his ongoing residential treatment status. At the time of the neuropsychological evaluation, Jeff was experiencing a medication washout, to reassess his health status and potential options for future medication treatment. However, he remained on quetiapine 200 mg for sleep, olanzapine as needed, desmopressin acetate 0.2 mg for occasional nocturnal enuresis, and propranolol 40 mg. Additional medical history included a genetic syndrome, which affected the growth of Jeff’s hair, skin, teeth, nails, and the function of his sweat glands. However, no indications in research suggest that this disorder should affect emotional functioning. Otherwise, Jeff was considered a healthy teenager.

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Test Results Given Jeff’s past documented cognitive delays and his current emotional problems, these areas were the focus of the neuropsychological assessment. The assessment approach was kept flexible, and a positive reinforcement system for effort was used in an attempt to manage Jeff’s low level of frustration tolerance. Jeff was a 13-year-old young man with a prominent upper lip and no teeth. His genetic syndrome reportedly resulted in the loss of his teeth, and he would destroy any false teeth given to him. He left his mother to go to the examination room willingly and established rapport quickly. However, Jeff’s social conversation was tangential and difficult to follow. He frequently engaged in long, disjointed, and repetitive stories. At times, these stories were delusional in nature, centering around themes of getting rich quick or renovating his home into a casino. Sustained attention was short, and he was distracted by both internal and external stimuli. Significant impulsivity was also noted throughout the evaluation. Jeff was only able to listen to directions and inhibit behaviors with overt prompts and assistance. Jeff’s overall intellectual abilities were not well represented by his overall Full Scale IQ Score, because of a significant difference between his verbal and nonverbal skills (Table 24.1). Jeff’s verbal intellectual abilities were in the low average range, while his nonverbal intellectual abilities were significantly higher and in the average to above average range. This degree of ‘‘split’’ between nonverbal and verbal abilities occurs in approximately 2.6% of the population. Current level of academic skills acquisition was lower than what could be expected given his intellectual functioning as measured on the WISC-IV. His academic abilities were in the borderline to mildly impaired range (Table 24.2). Further evaluation Table 24.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal Comprehension Index Perceptual Reasoning Index Working Memory Index Processing Speed Index Full Scale IQ Similarities Vocabulary Comprehension Block Design Picture Concepts Matrix Reasoning Digit Span Letter-Number Sequence Coding Symbol Search a Standard score, (scaled score), [T-score], {z-score}.

Scorea 83 110 71 94 88 (7) (7) (7) (14) (10) (11) (9) (1) (7) (11)

Percentile 13th 75th 3rd 34th 21st

248 Table 24.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition, Form A Academic skills Academic applications Letter-word Passage comprehension Calculation Applied problems Spelling Writing samples

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Score

Percentile

71 80 76 79 65 89 64 72

3rd 9th 5th 8th 1st 22nd 1st 3rd

Grade Equivalent 2.2 2.8 2.6 2.4 2.1 3.8 1.7 1.9

revealed Jeff had consistent strengths in visual processing, organization, and reasoning. However, he experienced several significant cognitive weaknesses, including weaker verbal than visually-based skills, as well as significant difficulties with learning (Table 24.3). Jeff’s visual-motor integration abilities were in the low average range. He demonstrated low average abilities to recreate geometric designs of increasing difficulty. Visual perception skills were consistent with other performances and were in the average range. Basic fine motor dexterity was in the low end of the average range. Among executive skills, Jeff’s verbal fluency abilities were average; however, during this task, he made a significant number of perseverative errors. Visual fluency performances were in the average range as well but also included a significantly high number of perseverative errors. Processing speed on a visual scanning and sequencing task was in the overall average range. Jeff had mild difficulties with rapid naming tasks, performing in the borderline to low average range. He had significantly greater difficulties, performing in the severely impaired range, filtering nonessential information on rapid naming tasks and responding based on preset rules. More complex problem solving and concept formation were slightly problematic, and he performed in the overall low average range. Mild difficulties generating novel problem-solving approaches were noted. He also demonstrated some difficulty utilizing spatial planning and learning from rules provided, as well as minor difficulties inhibiting incorrect responses. Jeff endorsed many depression symptoms including thoughts of hurting himself or other people (Table 24.4). However, on further questioning, Jeff stated he had no plans to hurt himself or another person and did not want to get into trouble. Jeff also reported a decreased need for sleep. Emotional evaluation utilizing projective measures supported behavioral observations, suggesting that Jeff struggled with frequent delusional and unusual thought processes. His thought processes were often tangential and circumstantial and became disorganized quickly, particularly when he began to experience any form of emotional stimuli. This disorganization resulted in many of Jeff’s conversations and emotional expressions becoming unusual and inappropriate.

24 On Eggshells: Pediatric Bipolar Disorder Table 24.3 Other cognitive functions Tests Beery-Buktenica Developmental Test of Visual-Motor Integration Visual Perception Wisconsin Card Sorting Test Total number errors Perseverative responses Perseverative errors Nonperseverative errors Categories completed Trials to complete first category Failure to maintain set Delis-Kaplan Executive Function System Trail Making Test Condition 1: visual scanning Condition 2: number Condition 3: letter Condition 4: switching Condition 5: motor speed Behavior Rating Inventory of Executive Function Parent Form Inhibit Shift Emotional Control Behavioral Regulation Index (BRI) Initiate Working Memory Plan/Organize Organization of Materials Monitor Meta-Cognition Index (MI) Global Executive Composite (BRI þ MI)

249

Score 85 102

Percentile 16th 53rd

116 119 134 104 >16th >16th >16th

(11) (12) (9) (12) (13)

[88] [85] [83] [92] [76] [75] [74] [69] [81] [78] [86]

Formulation and Recommendations The formulation of Jeff’s cognitive functioning cannot be considered without accounting for his significant emotional difficulties. Jeff experienced cognitive disorganization likely related to a mild level of psychosis. Jeff demonstrated both current symptoms and a developmental history consistent with Bipolar Disorder. It is not uncommon for children and adolescents with severe Bipolar Disorder to experience symptoms of psychosis. When evaluating an adolescent with such significant symptoms of mental illness, it is important to utilize history and the reports of caretakers to determine the primary symptom presentation. In Jeff’s case, his mood symptoms included both manic and depressive episodes, which in the past and currently occurred either independently or simultaneously with psychotic symptoms. The lack of symptoms of psychosis without ongoing mood symptoms

250 Table 24.4 Emotional functioning Tests Children’s Depression Inventory Negative mood Interpersonal problems Ineffectiveness Anhedonia Negative self-esteem Total Behavior Assessment System for Children Parent rating scale Hyperactivity Aggression Conduct problems Anxiety Depression Somatization Atypicality Withdrawal Attention problems Adaptability Social skills Leadership Externalizing problems Internalizing problems Behavioral symptoms index Adaptive skills

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Raw score

Score

3 5 1 9 2 20

[54] [78] [44] [71] [51] [63]

[65] [96] [110] [48] [86] [47] [105] [41] [71] [30] [32] [32] [97] [63] [91] [29]

Percentile

92nd 99th 99th 48th 99th 45th 99th 18th 97th 3rd 4th 3rd 99th 90th 99th 2nd

ruled out a primary psychotic disorder such as Schizophrenia or Schizoaffective Disorder. Cognitively, all of these major mental illnesses have been identified as correlating with deficits in executive functions, with a wide variety of research suggesting but not yet clearly defining other likely areas of cognitive weakness. At the time of this evaluation, Jeff was on minimal medication, and he appeared to have decompensated in his functioning, experiencing a return of symptoms, including psychosis. Such disturbed emotional functioning probably negatively impacted his overall ability to perform on cognitive testing but also demonstrated the significant impact that his emotional problems have on his overall cognitive abilities. Individuals like Jeff with significant mental illness often experience global learning difficulties, memory problems, and problems with executive skills. Jeff demonstrated better developed nonverbal than verbal abilities, along with learning deficits related to processing and executive functioning problems. He appeared to have significant difficulties with his initial attention to and processing of information. He did not appear to transition information well from working to long-term memory and, as a result, learned a lesser amount of information when initially exposed than otherwise would be expected. Symptomatically, this appeared as a globally based learning deficit. Additional testing of executive functions indicated

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that Jeff had a difficult time with inhibition of behaviors, planning and organizing his behaviors, and responding frequently in perseverative or inappropriate manners. These difficulties often relate to problems with cognitive flexibility, planning and organizing, and other executive abilities. In addition, they correlated highly with the behavioral disturbances symptomatic of his mental illness. The recommendations following an evaluation of an individual with a severe mental illness such as Jeff have to educate those living and working with the individual to recognize the extent of the illness and encourage consistent and ongoing treatment. In Jeff’s case, a strong warning needed to be made that even when in treatment, he remained at risk for aggressive and harmful behaviors toward himself and others. As a result, he would require some amount of supervision and monitoring, perhaps throughout his lifetime. Given Jeff’s emotional state, as well as his pervasive difficulties with learning and cognitive function, he would likely continue to struggle to achieve adequate academic progress. As a result, his educators were encouraged to shift his academic focus toward functional academics. He needed overt instruction to learn functional academics, socially appropriate behaviors, and other skills necessary for independent living. Recommendations included that Jeff’s school continue to provide an IEP under the qualification of an emotional behavioral disability. The IEP needed to acknowledge his difficulty functioning in a regular classroom environment and likely need for a specialized classroom, with one-to-one or small group instructions. In addition, Jeff continued to require a specialized behavioral plan to manage his emotional reactions and aggressive behaviors. He should be provided a strict, structured behavioral program, and it was important for the school to have a safety plan in place, both for Jeff’s safety as well as the safety of those around him. Although he was likely not a danger on a daily basis, he would continue to be at risk for decompensation, particularly under times of stress, which could result in aggression. Jeff continued to need medication treatment and monitoring for his mood disorder and significant emotional difficulties. Although medication treatment for Bipolar Disorder can be successful in managing many of the ongoing mood symptoms, often individuals with such a lifelong history of symptoms appear more treatment resistant. Individuals with these disorders often have to try many medications to find the most successful, and likely require multiple medications. Jeff’s family was strongly encouraged to work with a pediatric mental health professional with experience working with Bipolar Disorder and to monitor his medications closely. In addition to confirming Jeff’s diagnostic status, this evaluation helped his family better understand the impact that such a significant mental illness has on all aspects of his functioning. Jeff’s family decided to begin considering various long-term treatment options for him. Although it is difficult to say definitively what Jeff’s prognosis will be, his longstanding history of emotional instability, violent behaviors, and developmental delays suggest that he will likely continue to experience these difficulties and require some level of supervision and treatment throughout his life. While attempts will continue to be made to encourage Jeff to increase his coping skills and develop independent and functional living skills, his family

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plans to determine potential long-term care and legal options for guardianship. They also realize their continuous role to act as an advocate for him.

Additional Resources Key Concepts and Terms Attention Deficit Hyperactivity Disorder (ADHD) A disorder most often diagnosed in childhood, requiring symptoms occur prior to age seven. Symptoms include inattention and/or hyperactivity and impulsivity. Bipolar Disorders A group of disorders that are characterized by extreme fluctuations in mood. There are three major diagnoses that fall in this area: Bipolar I, Bipolar II, and Cyclothymia. Individualized Education Program (IEP) In the USA, an Individualized Education Program, commonly referred to as an Individual Education Plan, is a mandated requirement of the Individuals with Disabilities Act (IDEA). An IEP is required for any person in the public schools who is found to meet the federal or state requirement for special education and related services. This individual document mandates modifications or services in order for the student to achieve academic success.

References Resources for Clinicians American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders, fourth edition, text revision. Washington, DC: American Psychiatric Association. Birmaher, B. (2004). New hope for children and teens with bipolar disorder. New York: Three Rivers Press. Birmaher, B., Axelson, D., Strober, M., Gill, M. K., & Valeri, S. (2006). Clinical course of children and adolescents with bipolar spectrum disorders. General Psychiatry, 63, 75–183. Danielyan, A., & Kowatch, R. A. (2005). Management options for bipolar disorder in children and adolescents. Pediatric Drugs, 7(5), 277–294. Fristad, M. A., & Arnold, J. S. (2004). Raising a moody child: How to cope with depression and bipolar disorder. New York: The Guilford Press. Geller, B., & DelBello, M. (2003). Bipolar disorder in childhood and early adolescence. New York: The Guilford Press. Leibenluft, E., Charney, D. S., & Pine, D. S. (2003). Researching the pathophysiology of pediatric bipolar disorder. Society of Biological Psychiatry, 53, 1009–1020. Masi, G., Perugi, G., Toni, C., Millepiedi, S., Mucci, M., Bertini, N., et al. (2004). Predictors of treatment nonresponse in bipolar children and adolescents with manic or mixed episodes. Journal of Child and Adolescent Psychopharmacology, 14(3), 395–404.

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Pavuluri, M. N. (2003). Early recognition and differentiation of pediatric schizophrenia and bipolar disorder. Adolescent Psychiatry, 39, 1–11. Suppes, T., & Keck, P. E. (2005). Bipolar disorder: Treatment and management. Missouri: Compact Clinicals. Vinton, D., Hommer, R., Fromm, S., & Berghorst, L. (2006). Fear circuit flares as bipolar youth misread faces. Atlanta, GA: Georgia State University Press.

Resources for Families Carlson, T. (2000). The life of a bipolar child: What every parent and professional needs to know. Duluth, MN: Benline Press. Greene, R. W. (1998). Explosive child: A new approach for understanding and parenting easily frustrated, ‘‘chronically inflexible: children.’’ New York: HarperCollins. Lederman, J., & Fink, C. (2003). The ups and downs of raising a bipolar child. New York: Simon and Shuster. Papolos, D. F., & Papolos, J. (2007). Bipolar child: The definitive and reassuring guide to childhood’s most misunderstood disorder (3rd ed.). New York: Broadway Books. Singer, C., & Gurrentz, S. (2003). If your child is bipolar: The parent-to-parent guide to living with and loving a bipolar child. London, UK: Perspective Publishing. Waltz, M., & Lamb, L. (Eds.). (2000). Bipolar disorders: A guide to helping children and adolescents. O’Reilly & Associates.

Chapter 25

It Helps to Know Genetic Basis: Williams Syndrome as an Example of Cognitive Disability Bonita P. Klein-Tasman, Frank J. Gallo, Kristin D. Phillips, and Kathi M. Fine

When an adequate knowledge base is present, an assessment and intervention approach that takes into account genetic disorder etiology can be particularly powerful for children with genetically based neurodevelopmental disorders (Dykens & Hodapp, 2001). To illustrate the importance of this etiological approach, this case depicts the tailoring of assessment and intervention suggestions to a particular intellectual disability syndrome, Williams syndrome. Williams syndrome is a genetic disorder resulting from a hemizygous microdeletion on the long arm of chromosome 7. Genetic testing became available commercially in the early 1990s, consisting of a FISH test (fluorescence in situ hybridization) for the deletion of elastin, one of the genes in the Williams syndrome region. Although the level of intellectual functioning in individuals with Williams syndrome can vary greatly (from moderate intellectual disability to average range intellectual functioning in some), characteristic cognitive and behavioral phenotypes have been identified (Klein-Tasman & Mervis, 2003; Mervis et al., 2000). At the time of assessment, Elvis was a 16-year-old boy with Williams syndrome, whose parents sought additional insight about appropriate educational placement to best meet his vocational, academic, and emotional needs. Elvis’ parents also expressed concern regarding anticipatory anxiety and accompanying behavioral outbursts, as well as difficulties maintaining attention. He was placed in a special education tenth grade, with some integration into a regular education tenth grade classroom. He received speech and language therapy, occupational therapy, social skills training, and behavior management consultation at school. Prior to the assessment, he also received physical therapy and adapted physical education. Elvis was born full term at 7 lb and 11 oz, with a length of 20 inch. His Apgar scores at 1 and 5 min were 10. His mother did not experience any health problems during pregnancy and maintained adequate prenatal care. She did not smoke, drink alcohol, or take any medications during her pregnancy. There were no medical complications at birth or following delivery. As an infant, Elvis was quite irritable and cried a lot. Developmental milestones were generally achieved late. He first sat alone at eight months, stood upright at 15 months, and walked alone at J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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approximately 22 months. Elvis began using single words at approximately 17 months and phrases at approximately 30 months. He was trained for bladder at 5 years and bowel at 6 years. Elvis was diagnosed with Williams syndrome at age 22 months on the basis of clinical criteria (facial features, medical features), because there was no genetic test at that time. His medical history was positive for hay fever and allergies to cats. He had chicken pox at approximately 6 years and numerous ear infections. As a young preschooler, he experienced some eating difficulties that included frequent vomiting. Likely related to his diagnosis of Williams syndrome, he had hernia and ganglion surgery at age 3½. At the time of assessment, he had sustained no significant injuries. Elvis wore corrective lenses. He had received special education services since age 3½ and repeated kindergarten one time. Upon arrival at the clinic, Elvis introduced himself to each member of the assessment team with a happy handshake. His eye contact was intense. He routinely engaged others in conversation about topics of interest to him, sometimes inquiring about their own experiences. He spoke in full, fluid sentences with generally appropriate language content and somewhat exaggerated prosody. Elvis showed some difficulty with sustaining attention. At such times, he was likely to engage in conversation with the examiner rather than complete the task at hand. He occasionally asked whether testing would be over soon but did not appear overly fatigued. He was also somewhat concerned with making sure that he would get a break for lunch. However, he was redirected without much difficulty and generally persisted in the face of challenging tasks. Overall, the results of this evaluation likely provided an accurate sense of Elvis’ abilities.

Test Results Two measures examining intellectual abilities were administered, the Differential Ability Scales (DAS) and the Kaufman Brief Intelligence Test (K-BIT). The DAS is a broad measure of cognitive functioning that allows for separate examinations of verbal, nonverbal reasoning, and spatial functioning, and is particularly useful for children with Williams syndrome because of the wide range of subtest standard scores that can capture patterns of strength and weakness. The K-BIT is a brief cognitive assessment that does not include visuospatial constructive tasks, which are particularly challenging for people with Williams syndrome. Using the combination of these measures allows for examination of overall functioning both including and excluding visuomotor tasks. At the overall level, Elvis’s intellectual abilities were in the mildly to moderately impaired range (Table 25.1). As expected, his performance on the K-BIT was stronger than on the DAS. At the cluster level, his performance on measures of verbal and nonverbal ability was consistent across measures, with particular difficulty in spatial abilities. Elvis’ pattern of strengths and weaknesses was further illustrated by his performance on individual subtests of the DAS. He showed a relative weakness with tasks involving visuospatial construction (drawing

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Table 25.1 Intelligence Tests Differential Abilities Scale General Conceptual Ability Composite Verbal Cluster Word Definitions Similarities Nonverbal Cluster Matrices Sequential and Quantitative Reasoning Spatial Cluster Recall of Designs Pattern Construction Recall of Digits (Supplemental)

Scorea

Percentile

49 57 [25] [22] 59 [29] [20] 50 [20] [20] [43]

<1st 1st 1st 1st <1st 2nd 1st <1st 1st 1st 24th

Kaufman Brief Intelligence Test Composite IQ 58 Vocabulary 63 Matrices 60 a Standard score, (scaled score), [T-score], {z-score}. Table 25.2 Academic achievement Differential Abilities Scale Word reading Basic number skills Spelling

Score 84 55 61

<1st 1st <1st

Percentile 14th <1st <1st

reconstructing patterns with blocks), with performance on these tasks falling at or below the ‘‘floor’’ of this measure. He also showed a relative weakness in numerical reasoning. Performance on these tasks fell in the moderately impaired range. He showed a significant relative strength in auditory verbal short-term memory, as indicated by his performance on the Recall of Digits task, which fell in the solidly average range. In sum, results of the assessment indicated that Elvis showed a pattern of cognitive functioning that is characteristic of individuals with Williams syndrome (Mervis et al., 2000). The academic achievement subtests of the DAS were administered to gain a sense of Elvis’ basic reading, mathematics, and spelling abilities. Elvis’ word reading abilities fell in the low average range and were considerably stronger than expected on the basis of his overall cognitive abilities. His mathematical abilities fell in the mildly deficient range, consistent with his overall level of cognitive abilities. His spelling abilities fell in the mildly deficient range (Table 25.2). However, it should be noted that his performance on this measure was likely affected by his difficulty with handwriting. He would likely have spelled more successfully if he did not need to write the words but instead could type them on a computer. Overall, Elvis showed a relative strength in word reading ability, which is common for individuals with Williams syndrome.

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The Clinical Evaluation of Language Fundamentals – Fourth Edition (CELF-IV) was administered to gain a more comprehensive sense of Elvis’ language abilities. Although Elvis spoke in fluid and complete sentences, structured assessment indicated generally impaired language fundamentals. His strongest performance on the CELF-IV was on tasks involving rearranging words to make a sentence (borderline range), answering concrete questions about a paragraph presented orally (low average range), repeating sentences verbatim (mild impairment), and composing sentences with a target word (mild impairment). He showed his weakest performance on tasks involving conceptual understanding, such as when asked about the shared conceptual meaning of words or when asked to define the words, with significant impairment on each of these tasks (Table 25.3). Overall, results of the assessment suggested that his concrete language abilities were considerably stronger than his more abstract or conceptual understanding of language. As is common among individuals with Williams syndrome, Elvis’ somewhat stronger concrete verbal abilities may cause others around him to overestimate his abilities in other areas of functioning. Although Elvis expressed a clear interest in social interaction, his mother reported that he had very few truly reciprocal friendships. This is often seen among individuals with Williams syndrome, who may appear on the surface to have strong social skills due to a heightened drive to interact socially. However, the ability to sustain meaningful social interactions is typically not strong. Elvis appeared to have some awareness that he was not consistently socially successful. Elvis’s mother completed the Child Behavior Checklist to provide a sense of possible social, emotional, and behavioral difficulties. Elvis’s Total Problems and Internalizing scores were both in the clinical range above the 90th percentile, indicating a clinically significant difficulty in this area compared with others of his age. His Externalizing score was in the borderline clinical range, indicating mild difficulties compared with others of Elvis’ age. On specific ‘‘syndrome’’ scales, he scored in the clinical range on the Thought Problems scale (e.g., he often could not get his mind off certain thoughts or obsessions, had nervous movements like shaking his legs and picking at his fingers, and bit his arm when he became particularly frustrated). His scores on the Anxious/Depressed, Withdrawn/ Table 25.3 Other cognitive functions Clinical Evaluation of Language Fundamentals Recalling sentences Formulating sentences Word classes receptive Word classes expressive Word classes total Word definitions Understanding spoken paragraphs Sentence assembly Semantic relationships Number receptions total Familiar sequences

Score (5) (5) (2) (1) (1) (1) (6) (7) (1) (4) (3)

Percentile 5th 5th 1st 1st 1st 1st 9th 16th 1st 2nd 1st

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Depressed, Social Problems, Attention Problems, and Aggressive Behavior scales were in the borderline range. More specifically, Elvis had multiple fears, was described as nervous, and worried a lot. He sometimes lacked energy and preferred to be alone. He was at times overly dependent on others, yet sometimes did not get along with others. On the ‘‘DSM-oriented’’ scales, which are more relevant to traditional clinical diagnoses, he scored in the clinical range on the Affective Problems and Anxiety Problems scales. His scores on the ADHD Problems and Oppositional Defiant Problems scales fell in the borderline range. It should be noted that this measure was designed primarily for use with typically developing children; it is possible that some of the difficulties endorsed (e.g., attention problems, social problems) would be common for most individuals with less than average intellectual abilities. Elvis’ teacher also completed a measure of problem behaviors. Her responses indicated very few problem behaviors of concern. Scores did not fall in the borderline or clinical range for any scales. It is possible that his behavior was contrasted to other children in his classroom with special needs. Given parental concerns related to anxiety, the Anxiety Disorders Interview Schedule for Children – Parent Version was administered with Elvis’ mother as respondent to get a sense of his emotional experiences, particularly related to anxiety. She indicated that her main concerns about his emotional well-being were that he showed strong anxious anticipation regarding both positive and negative upcoming events and activities and was overly reactive to changes in schedule. He showed some moderate social anxiety, particularly when talking with peers over the telephone. He was also fearful of high places, having shots and blood tests, and visiting the dentist. These fears did not interfere significantly with his life, although there were some activities (e.g., diving off a high diving board) that he would be unlikely to participate in. Elvis’ mother indicated that he did not show any symptoms of depression at the time of assessment. Although Elvis’ mother would not characterize him as a worrier in general, she reported that he showed a significant amount of anticipatory anxiety. Specifically, this worry, which might better be described as a preoccupation, was centered on upcoming events and whether his daily activities would go according to plan. He would become overly talkative and ask the same question about these activities repeatedly, especially if there was any uncertainty that the events would actually occur. This preoccupation often interfered with his ability to engage in any other behaviors or activities. Elvis had great difficulty stopping this preoccupation once it started and worried about upcoming events more days than not. When he was worrying, Elvis was unable to relax, had particular difficulty concentrating, and became easily upset. When he was disappointed at a change in plans, he bit his hand at times. He would also cry or yell at people or at himself. This anticipatory worry interfered significantly at school, with friends, and moderately at home. It also bothered him greatly if he could not talk about it. Moreover, most of his conversations with others focused on getting reassurance regarding these events. It appeared that when he was engaged in a motivating activity, he experienced fewer preoccupied thoughts. However, he did experience some of these thoughts

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even when engaged in interesting tasks. The specificity of this anticipatory anxiety did not meet formal diagnostic criteria for an anxiety disorder but appeared to be at the juncture of generalized anxiety and obsessive‐compulsive behavior. On the basis of informal report, Elvis’ teacher indicated that she too had concerns about his emotion regulation. At the time of her report, she had seen improvements over the previous year; whereas prior to that, if asked to change seats, for example, he might argue, and now he would comply much more readily. Even given this improvement, she saw him as at least somewhat anxious almost all of the time and believed this must be difficult for him. He had clear difficulties regulating his emotional responses and appeared frustrated and angry. He had significant difficulty with what he perceived as large changes in routine (e.g., not going to a work placement when he expected to go) and milder difficulty with smaller changes in the classroom routine. He had a tendency to dramatically interpret minor changes (e.g., ‘‘We’ll never be able to do that ever again!’’) and melt down regarding bigger changes. His meltdowns consisted of verbal outbursts and perseveration, as well as biting his hands and fingers. Elvis had never missed school due to anxiety at the time of her report. Symptoms consistent with Attention Deficit Hyperactivity Disorder—Predominantly Inattentive Type were also endorsed by Elvis’ mother, as are commonly seen for children and adolescents with Williams syndrome. In particular, he often made little mistakes on schoolwork or chores, was easily distracted, had trouble listening to others, had difficulty finishing things that he started, had difficulty organizing schoolwork, and disliked doing schoolwork due to concentration issues. He also often lost things that he needed (e.g., gloves, hats). These kinds of difficulties were present since preschool. His mother indicated that it seemed as if it were difficult to get him to focus on tasks as needed, at least partly because of his strong preoccupations; it was as if it were difficult to get him to disengage. Elvis’ mother reported continued difficulties related to toileting. He had two accidents at school during the time of the assessment. He sometimes had a strong sense of urgency and could not make it to the bathroom in time. Generally, he appeared to urinate frequently, which is not uncommon for individuals with Williams syndrome. He became anxious about getting to the bathroom on time, and his mother usually planned carefully to make sure that she knew where bathrooms were and that they were easily accessible (e.g., she would seek aisle seats when attending a play). There were not any activities that the family avoided because of this issue. Elvis periodically took medication to address his bladder control difficulties. Difficulties with bladder and bowel control are not uncommon for individuals with Williams syndrome and may be related to the genetic underpinnings of the disorder.

Formulation and Recommendations In summary, Elvis was an engaging adolescent with Williams syndrome. He showed a pattern of cognitive and behavioral functioning that is characteristic of individuals with Williams syndrome. His overall intellectual abilities fell in the

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mildly to moderately impaired range. He showed the distinctive pattern of relative strength in auditory verbal short-term memory and relative weakness in visuospatial constructive abilities. His performance on a measure of intellectual abilities that does not include visuospatial construction was stronger, falling clearly in the range of mild impairment. Although he spoke in fluid and complete sentences, there was evidence from assessment of his language skills that he showed stronger concrete than abstract language abilities, as may be expected in those with developmental delay. At the time of assessment, parental and teacher concerns related to anxiety and coping skills were paramount. Elvis appeared to have significant difficulty coping with major and minor changes in routine. He also experienced significant difficulty with attention and concentration. Although there were improvements in these areas, emotional regulation continued to be a challenge for him.

Recommendations for Academic Progress Elvis’ strong concrete verbal abilities may cause others around him to overestimate his abilities in other areas of functioning. It is important that the adults in his life take care not to develop expectations for him that are unattainable, particularly in academic tasks. Elvis showed a relatively strong word reading ability. On the basis of his poorer performance on more abstract language tasks, it is expected that he will show more difficulty with reading comprehension, and further assessment is needed. If he shows difficulty in this area, it would be beneficial to teach him step-by-step strategies to improve his reading comprehension skills. The discrepancy between his concrete and abstract language abilities should be steadily considered when designing homework assignments for him. Elvis is most likely to be able to complete assignments that concentrate on understanding concrete details about what he reads independently. It is important that the expectations about the independence of homework completion be made clear to him. If more abstract understanding is necessary, he will need teacher or parental support. Given his graphomotor difficulties, the use of a computer would likely support Elvis’ written language skills. Increasing his use of the computer may also have important vocational benefit. Whenever possible, it would be helpful for him to receive an electronic copy of assignments that is prepared by the teacher on a word processor. This would greatly improve his ability to work on assignments independently, because he would not need to coordinate use of an assignment paper and computer. A significant focus of Elvis’ academic curriculum was on developing vocational skills. This is very appropriate and important in preparation of an ultimate transition away from school and into the community when he graduates. According to his mother’s report, he has responded well to the interventions implemented to improve his job performance (e.g., work on compliance with job coach). It should be kept in mind that many jobs for individuals with less than average intellectual abilities are

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designed for people whose relative strengths are in motor skills and visuomotor integration, precisely the tasks likely to be the most difficult for Elvis. It is recommended that efforts continue to be made to concentrate his work training opportunities in areas that do not rely heavily on motor abilities, when such possibilities exist. If there is a motor planning component to job-related tasks, he is most likely to succeed if there is also a social component, because he finds social interaction extremely motivating. Because Elvis’ word reading abilities are strong, jobs involving basic reading (such as sorting mail, for example) can be a good match for Elvis. Elvis takes considerable pride in the responsibility of having a job.

Recommendations for Psychosocial Function Mature social interaction is a challenge for Elvis. Although it may appear on the surface that he has strong social skills, because he is quite interested in social interaction, his ability to sustain social interactions is not truly an area of relative strength. The gaps in social understanding between children with developmental disabilities and their typically developing peers tend to grow exponentially in adolescence. Elvis appears to have some awareness that he is not consistently socially successful. Continued involvement in structured activities with both typically developing peers and, importantly, with peers with developmental disabilities is likely the best way to work against social isolation. Fostering relationships with other peers with special needs may be particularly beneficial because many of his typically developing peers may go off to college in a few years. If possible, it would be helpful to have Elvis practice social interaction skills such as calling peers to arrange for outings or generating ideas for outings within the context of his social skills groups (e.g., by having members of the group call each other). To address Elvis’ anticipatory anxiety, it would be helpful to have him practice adjusting to unexpected changes in routine and transitions. A hierarchy of transition situations could be developed. He should be consulted so that he can provide an understanding of what transitions or changes he imagines might be most difficult and which ones might be easier. Each situation should be clearly rated according to how difficult he thinks it would be to manage well in that situation. Care should be taken with these ratings, as individuals with Williams syndrome tend to want to please others, which may affect their subjective self-report. At the bottom of the hierarchy might be a change to which clothes he can wear on a given day or what food he will have for breakfast (from one thing he likes to another thing he likes). At first, coping with such a change should be role played, without actually requiring that there be a change in routine. These initial tasks should be very easy for Elvis to cope with. This gives him a chance to practice responding appropriately to the change without the stress of the actual change. Next, he could be asked to agree on a possible activity where the plans might change (so that in fact he knows the change is coming), and then asked to practice coping when the change in plans actually takes place. It is critical that a reward system be put in place to reinforce him

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when he attempts to cope (rather than when he succeeds at coping), even in the very easy situations. Once Elvis demonstrates an ability to cope in these more contrived situations, then the coping task can be made incrementally more difficult, moving slowly up his hierarchy of difficult transitions and changes in plans. A similar approach could be used to improve his responses to corrective statements at home, in school, and in the context of vocational experiences. It would be helpful to Elvis to make increasing attempts to regulate his own emotions. One way of improving his self-regulation abilities is to help him develop a repertoire of ‘‘adaptive self-statements.’’ These are phrases he can say that help him to cope effectively with stressors. For example, when he says something dramatic (e.g., ‘‘I will never get to do that again,’’ when plans are changed), it would be helpful for adults around him to model the kind of statement that they would hope he would make, which would reflect good emotional regulation. The statement should be made calmly. For example, ‘‘Oh well, it looks like you won’t be doing that activity after all today. You’ll probably get a chance to do it some other time.’’ Sometimes it may be helpful to provide him with an adaptive response to a request from another person and ask him to try it out and see how it works for him. For example, if he is asked to move to another location and objects, his mother might try saying, ‘‘Elvis, try ‘Okay, Mom. I will help you out.’ And see how it feels.’’ These approaches take advantage of his strong rote language skills. Many individuals with Williams syndrome tend to become preoccupied with negative events. For Elvis, he tends to become preoccupied with upcoming events more generally but particularly when he doubts whether an upcoming event will actually happen. Sometimes, individuals end up getting more attention from others when they express these preoccupations than when they do not (at such times others are just relieved that the child does not seem preoccupied and so remain quiet). It would be helpful to ensure that Elvis is differentially reinforced for coping statements and for not being preoccupied by a transition, because he may come to learn that if he makes negative statements, he gets more attention. For example, if on a particular day he does not ask about when he will go to the regular education classroom, he should be rewarded. This will require practice, but over time it may become less of a habit to ask about the upcoming event.

Discussion As is evident from this case description, Elvis has a number of cognitive, emotional, and behavioral support needs. One central question for many families of children with Williams syndrome is the extent to which their child’s behavior is similar to others with Williams syndrome. Elvis’ pattern of needs may indeed be more closely anticipated by his Williams syndrome diagnosis than by his current level of cognitive functioning. In particular, he shows a characteristic cognitive profile of relative strength in rote memory and relative weakness in visuospatial construction. Within his language abilities, greater conceptual demands were associated with poorer performance.

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In terms of academic functioning, word reading was an area of strength with greater difficulty with more complex reading comprehension tasks. Mathematical abilities were an area of relative weakness. His pattern of socio-emotional and behavioral functioning was also emblematic of that seen in many individuals with Williams syndrome. He experiences considerable anticipatory anxiety, emotional regulation difficulties, and attention problems. Additionally, as this family prepares for transition to adulthood, there are concerns about how best to support his developing independence. Interventions that are useful for the kinds of difficulties shown by people with Williams syndrome, taking into account patterns of cognitive strength and weakness, are suggested for supporting Elvis’ functioning and are strengthened by incorporating an understanding of the cognitive and behavioral phenotype associated with this neurodevelopmental disorder.

Additional Resources Key Concepts and Terms Hemizygous Microdeletion A microdeletion means that one or more genes are missing on a chromosome. These missing genes are typically not visible when the chromosomes are all examined together under a microscope. Rather, more complex molecular genetics approaches are required to see that the genes are missing (see FISH test below for one such approach). Humans usually have two copies of each gene—one on each of the pair of chromosomes. If only one copy of a gene is missing, that is a hemizygous deletion. If both copies are missing, that is a homozygous deletion. Homozygous deletions are rare because they usually affect the viability of the fetus more strongly. FISH Test FISH is an acronym for the laboratory process of ‘‘fluorescence in situ hybridization,’’ which helps determine whether specific genes are missing from chromosomes. There are many different FISH tests for different disorders. For Williams syndrome, the FISH test is a blood test to detect the presence of the elastin gene, which is one of the genes typically deleted in individuals with Williams syndrome. The blood sample is treated with two specific colored markers that give off a fluorescent light when exposed to ultraviolet light. One of the markers attaches to each of the two copies of chromosome 7 in a cell. Another colored marker attaches to the elastin gene on each copy of chromosome 7. For people with Williams syndrome, only one copy of chromosome 7 will show the colored spot for elastin—the other copy will be missing the elastin gene. Graphomotor Difficulties Difficulties with the mechanics of writing. In the case of Williams syndrome, these are likely related to motor planning, visuomotor coordination, as well as more basic fine motor difficulties related to muscle function.

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Visuospatial Construction The most consistent difficulty seen in people with Williams syndrome. It involves attempting to reproduce a visual image on the basis of a sample (with blocks or by drawing) and incorporates both visual perception (seeing the component parts and how they fit together) as well as visuomotor coordination and motor planning.

References Resources for Clinicians Achenbach, T. M., & Rescorla, L. A. (2000). Manual for the ASEBA preschool forms and profiles. Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families. Dykens, E. M., & Hodapp, R. M. (2001). Research in mental retardation: Toward an etiologic approach. Journal of Child Psychology and Psychiatry and Allied Disciplines, 42, 49–71. Elliot, C. D. (1990). Differential abilities scales. Sand Diego, CA: Harcourt Brace Javanovich. Kaufman, A. S., & Kaufman, N. L. (1990). Kaufman brief intelligence test. Circle Pines, MN: American Guidance Service. Klein-Tasman, B. P., & Mervis, C. B. (2003). Distinctive personality characteristics of 8-, 9-, and 10-year-olds with Williams syndrome. Developmental Neuropsychology, 23, 269–290. Mervis, C. B., & Klein-Tasman, B. P. (2000). Williams syndrome: Cognition, personality, and adaptive behavior. Mental Retardation and Developmental Disabilities Research Reviews, 6, 148–158. Mervis, C. B., Robinson, B. F., Bertrand, J., Morris, C. A., Klein-Tasman, B. P., & Armstrong, S. C. (2000). The Williams syndrome cognitive profile. Brain and Cognition, 44, 604–628. Semel, E., Wiig, E. H., & Wayne, S. A. (2003). Clinical evaluation of language fundamentals – fourth edition. San Antonio, TX: The Psychological Corporation.

Resources for Families www.williams-syndrome.org Dykens, E. M., Hodapp, R. M., & Finucane, B. (2000). Genetics and mental retardation syndromes: A new look at behavior and interventions. Baltimore, MD: Paul H. Brookes. Semel, E., & Rosner, S. R. (2003). Understanding Williams Syndrome: Behavioral patterns and interventions. Mahwah, NJ: Erlbaum.

Chapter 26

Mixed Bag: Tics, Compulsions, and More Kenneth L. Grizzle

When Ricky was 6-years old and completing kindergarten at a parochial school, his parents, teacher, and pediatrician were concerned because he was recognizing less than half of the lowercase letters of the alphabet and inconsistently recognizing numbers through 50. However, he could count beyond 100. Ricky made mild articulation errors in spontaneous speech and continued to show difficulties sequencing multisyllabic words, features thought to reflect subtle remaining characteristics of his previously diagnosed developmental apraxia of speech. At home and school Ricky avoided all tasks requiring literacy. Although he allowed his parents to read to him, he became restless and walked away when asked to read decodable books or to engage in any type of writing. Although not consistent, he sometimes became frustrated to the point of having emotional meltdowns that included screaming and throwing items. Similar behaviors were seen during initial testing. Ricky had been diagnosed by a Speech and Language Pathologist (SLP) with Developmental Speech Apraxia and Mixed Receptive/Expressive Language Disorder at age 18 months. He received speech and language therapy from that time until age 4½ years through a private SLP and Early Childhood Education. Therapy was discontinued after a language evaluation found language comprehension and formulation to be at age level. Language testing by an SLP at 7 years and 3 months included administration of the Test of Auditory Processing – Revised (TAPS-R); memory span deficits were reported. A follow-up language evaluation was completed at age 9. Receptive and expressive language were within normal limits, though based on a considerable split between receptive and expressive vocabulary, and during conversation, word finding deficits were identified. Memory span deficits persisted. Maternal and paternal family histories were positive for dyslexia and mood disorder. Comorbid with mood and learning disorders, Ricky’s father had a history of anxiety and substance abuse. Developmental milestones with the exception of language were within normal limits. Ricky’s mother and father agreed that their son’s vocabulary developed normally until 9 months of age; he was using single words and vocabulary consisted of approximately 15 words. However, Ricky

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developed no new vocabulary over the next 9 months. At that time his parents sought Ricky’s first language evaluation, and work with a private SLP began. Ricky’s parents were separated when he was 4 years of age. He lived with his mother and had periodic contact with his father for 12 months. Beginning at 5 years he split time between his parents, spending 7 days with his mother and 7 days with his father. Both parents were actively involved in Ricky’s life, including medical, psychological, and educational care. Ricky’s mother retained primary custody even though his placement was split. His parents’ relationship had historically been poor, and a clear dislike for each other was palpable during treatment sessions. Nevertheless, both regularly attended all medical and psychological appointments and were actively involved in Ricky’s care and treatment.

Initial Test Results Initial testing with Ricky was completed during the summer after kindergarten. He was 6 years and 5 months of age. The biggest concern was limited progress developing early reading skills, so testing focused primarily on literacy. Ricky was generally cooperative and persistent on tasks, but when asked to engage in reading-related activities, he was resistant and attempted to distract the examiner by asking irrelevant questions. When struggling to recognize letters and read two- or three-letter words, he was noticeably distressed, including becoming tearful and attempting to calm himself down with deep breathing. Ricky regularly licked his fingers and rubbed them beneath his eyes. No other habitual behaviors were noted or reported by his parents, but when this behavior was pointed out, both parents agreed that they had seen it but thought it was the result of allergies. Cognitive testing globally fell within the average range, though nonverbal reasoning and spatial skills were high average (Table 26.1). All subtest scores fell within the average range with the exception of Recall of Objects-Delayed, which fell in the fifth percentile. It was the examiner’s impression that previously identified word retrieval deficits contributed to this low score. Poor early reading and spelling skills were reflected in his performance on the Wechsler Individual Achievement Test‐II (WIAT-II); scores from the Word Reading and Spelling subtests fell at the tenth percentile (Table 26.2). Perhaps more importantly for a child just completing kindergarten, Ricky could not complete any rhyming activities and was unable to write sounds associated with select letters and letter combinations. Poor phonological processing was having a direct impact on Ricky’s development of reading and spelling skills. Performance on the Comprehensive Test of Phonological Processing (CTOPP) suggested mild to moderate deficits in phonemic awareness, phonological memory, and rapid automatized naming (Table 26.3). On the basis of test results and subjective review of writing samples, there was no evidence of deficits in motor manipulation or integration of visual and motor domains.

26 Mixed Bag: Tics, Compulsions, and More Table 26.1 Intelligence – first evaluation Differential Abilities Scale Scorea Verbal cluster 99 Nonverbal cluster 110 Spatial 114 General cognitive ability 109 Word definitions [51] Similarities [48] Matrices [59] Sequential and qualitative reasoning [53] Recall of designs [62] Pattern construction [56] Recall of digits [45] Recall of objects Immediate [51] Delayed [34] Speed of information processing [60] a Standard score, (scaled score), [T-score], {z-score}. Table 26.2 Academic achievement – first evaluation Wechsler Individual Achievement Test – Second Edition Word reading Spelling Numerical operations Math reasoning

Table 26.3 Other cognitive functions – first evaluation Tests Comprehensive Test of Phonological Processing Phonological Awareness Phonological Memory Rapid Naming Beery-Buktenica Developmental Test of Visual-Motor Integration

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Percentile 47th 75th 82nd 73rd 54th 42nd 62nd 57th 88th 73rd 31st 54th 5th 54th

Score 81 81 96 122

Percentile 10th 10th 39th 93rd

Score

Percentile

79 82 82

8th 12th 12th

117

87th

Ricky’s mother’s response to the behavior checklist resulted in a profile in which all factors were elevated to the borderline range. Her response to the ADHDspecific questionnaire showed no elevated concerns for ADHD symptoms. Ricky’s teacher also completed rating scales, and reported no problem areas.

Initial Formulation and Recommendations On the basis of test results, a preliminary diagnosis of a reading disorder (dyslexia) was made. It was recommended that Ricky become involved in an intense and systematic intervention to improve phonological awareness and sound and symbol

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correspondence. Although the level of commitment required to develop educationrelated skills outside the school setting can be excessive in some circumstances, because this child attended a parochial school that did not have a reading specialist or teachers who felt trained to teach reading effectively to a child at extreme risk for dyslexia, the decision was made to offer this recommendation. Ricky’s repetitive behaviors of licking his index fingers and wiping them under his eyes were noted. Because of the relatively recent onset of these behaviors together with the fact that the behaviors were having no functional impact on him, the recommendation was made to monitor symptoms and return if they worsened or began to interfere with daily functioning.

Second Test Results Ricky was evaluated again 3½ years later. In the interim, he had received considerable intervention for reading and literacy in general. The private school he attended had hired a learning specialist who worked regularly with him. On the basis of discussion with Ricky and his parents and a review of the reading specialist’s records, it appeared that the specialist used a code-based intervention to address reading and spelling deficits. She worked with Ricky on average three times per week for approximately 30 min each session. She completed testing at age 9 and suggested that Ricky’s reading and spelling profile, ‘‘loosely fit a dysphonetic pattern, although errors are not as striking as often seen.’’ As part of the evaluation, she administered the CTOPP and reported the following composite scores: phonological awareness 124, phonological memory 88, and rapid naming 100. In addition to seeing the reading specialist, Ricky received reading interventions from a reading specialty clinic at a local liberal arts college. Ricky’s parents reported that his reading skills had shown dramatic improvement since the initial testing. In fact, the school reading specialist felt that she no longer needed to work with him. He was also no longer receiving educational services from the community reading clinic. Ricky’s mother, however, expressed ongoing concern about difficulties that he was having in the school curriculum. Although his grades typically ranged from As to Cs, he continued to be easily frustrated, refused to engage in independent reading, and demanded that a parent help him at all times with homework. During the school day, accommodations were made because of the identified learning disability: all tests were read to him; written portions of exams were allowed to be dictated; teachers did not down-grade for poor spelling. Updated intellectual testing was consistent with the initial test results. Nonverbal reasoning skills were high average to superior, and verbal reasoning skills were average (Table 26.4). Relative weaknesses were seen in auditory memory-related tasks and psychomotor speed. Verbal memory functioning was also consistent with previous test results. In addition to relatively weak auditory working memory scores, Ricky performed poorly on all portions of the verbal learning subtests (Table 26.5), suggesting poor encoding of discrete pieces of auditory information.

26 Mixed Bag: Tics, Compulsions, and More Table 26.4 Intelligence – second evaluation Wechsler Intelligence Scale for Children – Fourth Edition Full Scale IQ Verbal Comprehension Similarities Vocabulary Comprehension Perceptual Reasoning Block Design Picture Concepts Matrix Reasoning Working Memory Digit Span Letter-Number Sequencing Processing Speed Coding Symbol Search

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Score 104 102 (10) (11) (11) 121 (16) (13) (11) 88 (6) (10) 91 (7) (10)

Percentile 61st 55th

92nd

21st

27th

An updated language evaluation completed by an SLP included administration of the TAPS-3 (Table 26.5) and showed severely deficient memory span and working memory and poor phonological blending, which was likely related to poor memory span. Visual memory and narrative memory were intact. Academic testing showed continued poor word decoding, low average word reading, and moderately deficient spelling skills (Table 26.6). Of note was Ricky’s ability to derive meaning from written text. Reading comprehension fell in the second percentile, which contrasted with verbal reasoning skills in the 92nd percentile, narrative memory in approximately the 70th percentile, and average receptive vocabulary. Ricky’s poor reading comprehension required that the entry level be pushed back, thus not allowing the examiner to obtain a measure of reading speed. Test results and behavioral observation suggest that poor reading comprehension reflected a number of factors: poor phonetic decoding, relatively weak working memory skills, and limited metacognitive strategy application, including self-directed learning. Additional support for factors other than broad structural language processing affecting reading comprehension was Ricky’s expressive writing. Conceptual writing, including idea development and story formulation, was strong, as reflected by his performance on the Test of Written Language-3 (TOWL-3). Writing mechanics, specifically spelling and punctuation, had the greatest impact on the readability of his written output. Measures of phonological processing were inconsistent. Performance on the CTOPP suggested above average phonemic awareness. However, using the Process Assessment of the Learner (PAL), which employs a deletion task to evaluate phonemic awareness, Ricky showed weak skills (<20th decile) at the syllable and phoneme level. On the TAPS-3 he also showed poor phonological blending skills. Results suggested continued weak phonemic awareness. Efficient and automatic recognition and retrieval skills as reflected by RAN tasks were adequate

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Table 26.5 Other cognitive functions – second evaluation Tests Score Wide Range Assessment of Memory and Learning – Second Edition Verbal memory 97 Story memory (12) Story memory recall (11) Story memory recognition (11) Verbal learning (7) Verbal learning recall (5) Verbal learning recognition (6) Visual memory 124 Design memory (13) Picture memory (15) Finger windows (11) Test of Auditory Processing Skills – Third Edition Overall score Phonologic Memory Cohesion Word discrimination Phonological segmentation Phonological blending Number memory Forward Reverse Word memory Sentence memory Auditory comprehension Auditory reasoning

79 94 62 93 (9) (12) (5)

Percentile 42nd

95th

8th 34th 1st 32nd

(1) (1) (6) (2) (10) (7)

Table 26.6 Academic achievement – second evaluation Tests Wechsler Individual Achievement Test – Second Edition Word reading Reading comprehension Pseudoword decoding Numerical operations Spelling

Score

Percentile

89 69 82 107 73

23rd 2nd 12th 68th 3rd

Test of Written Language – Third Edition Spontaneous writing Contextual conventions Contextual language Story construction

94 (7) (9) (11)

35th 16th 37th 63rd

across measures, though phonological memory continued to be relatively weak. In addition to phonological deficits, Ricky showed poor orthographic coding as reflected by his performance on the Receptive Coding (<10th decile) and Word Choice (<10th decile) subtests from the PAL.

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Second Formulation and Recommendations Test results confirmed the previous preliminary diagnosis of Specific Reading Disorder. Phonetic word decoding deficits continued to be affected by weak phonological processing, including memory deficits, limited phonemic awareness, and poor orthographic coding, which has been connected to poor development of sight word automaticity in connected text and inefficient reading of irregular words (Manis, Seidenberg, Doi, McBride-Chang, & Peterson, 1996). The inconsistency in phonological processing scores obtained during the last set of testing warrants discussion. Ricky clearly showed considerable gains in components of phonological awareness, as reflected by his CTOPP score and the Phonological Segmentation subtest from the TAPS-3. In contrast, phonological memory continued to be problematic, as reflected by all measures. This suggested that there was a strong memory span component to Ricky’s poor phonological processing that was directly impacting word decoding skills. In fact, broader active working memory deficits were likely a contributing factor (with poor phonological decoding) to Ricky’s poor reading comprehension. During the second round of testing, Ricky showed pronounced eye blinking, sniffing, and throat clearing. These behaviors were exacerbated during times of stress, such as when he was asked to engage in any task that required reading. His parents made no mention of tics during the follow-up interview; however, upon discussion following testing, both parents acknowledged a gradual worsening of motor and vocal tics, both of which had been in place for approximately 18 months. It was recommended that Ricky return to receiving reading instruction. In addition to continued development of phonetic decoding skills, which should be the focus of word reading instruction at this point, it was recommended that phonemic awareness and orthographic coding not be ignored. Recommendations were also made for enhancing reading fluency skills through the use of repeated reading, a rapid word recognition chart, and choral reading. Although reading comprehension was felt to be negatively impacted by decoding and memory deficits, metacognitive strategies for increasing Ricky’s independent reading and learning were offered. Referrals were made to neurology and a psychologist specializing in treatment of tic disorders. Three months after the second evaluation was completed, Ricky’s father reported that Ricky was having difficulty getting into vehicles. Each time he entered a vehicle, he had to make sure the door was shut by closing and opening the door exactly three times. This behavior eventually spread to all doors he passed through. Ricky also refused to remove plastic bracelets he received from swimming pools, leaving them on for a month or more. When either parent eventually insisted to remove the bracelet, Ricky became extremely agitated and insisted that the bracelet not be thrown out. They discovered that he had saved multiple bracelets and ticket stubs from various events. Both parents acknowledged that when their son was at the other’s house, it was not unusual for them to periodically go through his hoard

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of belongings and throw items out that they did not think he would miss because they felt it would be too difficult to remove them when he was present. A diagnosis of Tourette’s syndrome (TS) with Obsessive‐Compulsive (OC) features was added to Ricky’s clinical picture. The decision was made to limit the number of health care providers working with Ricky. His medical care was transferred to a pediatric psychiatrist, and his behavioral therapies were provided by a single provider at the same center as the psychiatrist. The pediatric psychiatrist initiated sertraline, which at 25 mg addressed the OC features. Guanfacine was eventually added and titrated up to 6 mg per day with no added benefit for tic reduction. Behavioral therapy, including cognitive-behavioral treatment (CBT) using an exposure and response prevention protocol was implemented. Medication and CBT provided effective control of OC symptoms, but Ricky was becoming resistant to ongoing treatment, including minimizing the presence and effect tics were having on him. The decision was made to slow down the treatment process with hopes that a break might result in Ricky’s renewed motivation to address tics. Consistent with known sequelae of Tourette’s syndrome (Hoekstra, Steenhuis, Kallenberg, & Minderaa, 2004; Silva, Munoz, Barickman, & Friedhoff, 1995), the severity of Ricky’s tics varied on the basis of environmental stressors. Unfortunately, in his life there were multiple stressors. Homework that required any type of reading or writing consistently resulted in excessive tics, to the point of homework taking 2– 3 hours to complete. Tics were most pronounced when he was with his father. Ricky was his only son, and his father acknowledged feeling responsible for genetically transmitting anxiety and reading problems to him. He went to considerable lengths to minimize the impact of learning and psychiatric difficulties on Ricky, including asking teachers to allow him to skip homework on nights he had athletic activities or appointments, videotaping his tics, and making multiple weekly phone calls to mental health providers with updates on tics and OC features. After 8–10 weeks without psychotherapy, Ricky’s mother called, asking that he reenter treatment. She and his father were returning to court, each seeking sole custody of Ricky. Despite periodic lapses from both parents in their communication with Ricky about the court hearing, Ricky was somewhat sheltered during this process. Placement was eventually changed from Ricky splitting time 50/50 between his parents to a 70/30 split, which produced less disruption but allowed continued, consistent contact and involvement with both parents. Change in placement was to take place approximately 2 weeks before school started, which meant he had an entire summer with the extant placement. During the court hearing and the 4 weeks that followed, there was no apparent exacerbation of tics. Within 2 weeks after the court hearing, school was dismissed for the summer, and Ricky went on a 10-day vacation with his mother and then a 2-week vacation with his father. Both parents reported that Ricky was nearly ticfree during the vacations. After returning home, Ricky developed an intense, complex tic pattern that included popping his right shoulder, rubbing his eyes, sniffing, and a hard, nonproductive cough. He went on a weekend camping trip with his father and was unable to complete a short hike, having to stop regularly and engage in the complex tic pattern.

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Upon returning to treatment Ricky asked, for the first time, what he could do to stop the tics. A habit reversal training (HRT) protocol was initiated consisting of five components: awareness training, competing response training, contingency management, relaxation training, and generalization training.

Update Ricky is currently involved in the behavioral treatment of his tics. An interesting clinical observation is that his tics appear to have taken on a rather pronounced functional component. With the onset of severe tics, Ricky’s parents have been united, for the first time, in the need to support their son in his attempts to manage this psychiatric condition more effectively. Although this is felt to have a potentially beneficial effect on the treatment process, the clear existence of symptoms that nominally had the potential of decreasing conflict between caregivers could also have the unintended consequence of maintaining symptomology. Care must be given to monitor and directly address this dynamic if it occurs. This will most likely be accomplished in a direct manner by pointing out the phenomenon to Ricky’s parents and working with them to maintain an amicable relationship if for no other reason than the health of their son.

Additional Resources Key Concepts and Terms Tourette’s Syndrome (TS) Presence of motor and vocal tics that occur every day or nearly every day for greater than one year and cause distress or impairment in functioning. Obsessive–Compulsive Disorder (OCD) Recurrent obsessions or compulsions that cause distress or interfere with daily routine. Unlike adults, children need not be aware that the symptoms are unreasonable for the diagnosis to be made. Reading Disorder A learning disability that is characterized by unexpected reading accuracy or comprehension that is considerably weaker than other cognitive skills and below grade level, and occurs despite effective reading instruction. Dyslexia, the most common type of reading disorder, includes difficulties with accurate and/ or fluent word recognition, and poor spelling and word decoding abilities. TS, OCD, and Learning OCD is a common comorbidity for individuals with TS, occurring even more frequently among individuals with severe TS (Coffey et al., 2000). There has been less research recently on the learning patterns associated with TS. Although research to date suggests that as a group, individuals with TS are

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at greater risk to have a learning disability of some type relative to the population at large, evidence is mixed on the type of learning profile commonly found among TS children. There is support that learning challenges are exacerbated by Attention Deficit/Hyperactivity Disorder (Denckla, 2006), another common comorbidity of TS. In Ricky’s case, there was a strong paternal and maternal family history for dyslexia, which alone places him at much greater risk for reading problems. His father also shows obsessive–compulsive behaviors but does not warrant a diagnosis of OCD or OCPD.

References Resources for Clinicians Blachman, B. A., Ball, E. W., Black, R. B., & Tangel, D. M. (2000). Road to the code: A phonological awareness program for young children. Paul H. Brookes: Baltimore. Coffey, B. J., Biederman, J., Smoller, J. W., Geller, D. A., Sarin, P., Schwartz, S., & Kim, G. S. (2000). Anxiety disorders and tic severity in juveniles with Tourette’s disorder. Journal of the American Academy of Child and Adolescent Psychiatry, 39(5), 562–568. Dawson, P., & Guare, R. (2004). Executive skills in children and adolescents: A practical guide to assessment and intervention. Guilford Press: New York. Denckla, M. B. (2006). Attention deficit hyperactivity disorder: The childhood co-morbidity that most influences the disability burden of Tourette syndrome. In: J.T. Walkup, J.W. Mink, & P.J. Hollenbeck (Eds.), Advances in neurology: Volume 99: Tourette syndrome. Lippincott, Williams & Wilkins: Philadelphia. Goodman, W. K., Storch, E. A., Geffken, G. R., & Murphy, T. K. (2006). Obsessive-compulsive disorder in Tourette syndrome. Journal of Child Neurology, 21(8), 704–714. Hoekstra, P. J., Steenhuis, M. P., Kallenberg, C. G. M., & Minderaa, R. B (2004). Association of small life events with self reports of tic severity in pediatric and adult tic disorder patients: A prospective longitudinal study. Journal of Clinical Psychiatry, 65, 426–431. Manis, F. R., Seidenberg, M. S., Doi, L. M., McBride-Chang, C., & Peterson, A. (1996). On the bases of two subtypes of developmental dyslexia. Cognition, 58, 157–195. March, J. S., & Mulle, K. (1998). OCD in children and adolescents: A cognitive-behavioral treatment manual. Guilford Press, New York. Minskoff, E., & Allsopp, D. (2003). Academic success strategies for adolescents with learning disabilities and ADHD. Paul H. Brookes: Baltimore. Silva, R. R., Munoz, D. M., Barickman, J., & Friedhoff, A. J. (1995). Environmental factors and related fluctuation of symptoms in children and adolescents with Tourette’s disorder. Journal of Child Psychology and Psychiatry, 36, 305–312. Walkup, J. T., Mink, J. W., & Hollenbeck, P. J. (2006). Advances in neurology: Volume 99: Tourette syndrome. Lippincott, Williams & Wilkins: Philadelphia.

Resources for Families Fitzgibbons, L., & Pedrick, C. (2003). Helping your child with OCD. New Harbinger Publications, Oakland. I Have Tourette’s, But Tourette’s Doesn’t Have Me. DVD available through Tourette Syndrome Association, http://www.tsa-usa.org/

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Learning Disabilities Association, 4156 Library Road, Pittsburgh, PA 15234, 412–341–1515, http://www.ldaamerica.org The Obsessive-Compulsive Foundation, 676 State St., New Haven, CT 06511, 203–401–2070, http://www.ocfoundation.org Tourette Syndrome Association, Inc., 4240 Bell Blvd, Suite 205, Bayside, NY 11361–2820, 718– 224–2999, www.tsa-usa.org Road to the Code: A Phonological Awareness Program for Young Children (Blachman, Ball, Black, & Tangel, 2000). Masonic Learning Center, http://www.childrenslearningcenters.org/. A multi-sensory code-based intervention. Children involved in this Orton-Gillingham-based program are expected to attend hour long sessions twice a week for 2 years. Services are offered without charge.

Part III

Things that Go Bump in the Night: Interesting Questions and Controversies for Our Field

Part III illustrates several interdisciplinary questions, divergent points of view, and practical questions regarding the utilization of services with limited availability, such as pediatric neuropsychological evaluations, among the overall expenditure of health care resources. Exciting and challenging ideas come onto the table in any field as vibrant as pediatric neuropsychology, and it is important to openly consider new developments and potential contributions from other related fields. Gradual accumulation of collaborative interdisciplinary evidence is needed more than passionate arguments. As editors, we tend to be conservative in considering these issues, but we also seek forums such as the present book to spark valuable discussion among disciplines. Case presentations provide an important avenue through which to examine these ideas, although we acknowledge that systematic research is also needed to test hypotheses and build support for the construct validity of ideas from other fields. One of the most salient of such issues is the debate among clinicians about the validity of theories emphasizing basic sensory processing in accounting for children’s problems with learning, behavior, and emotions. On one hand, the importance of primary and secondary sensory processing in neural circuits has been argued persuasively throughout the history of clinical neuropsychology (Lurija, 1973). On the other hand, advances in basic neuroscience and cognitive neuroscience research have not always been integrated fully into clinical approaches promoting such arguments. The debate over the nature of the fundamental processing deficits that were presumed to be addressed by the computer-based intervention Fast ForWord (Tallal et al., 1996; Mody et al., 1997) illustrates the dilemma of marketing treatments when replication of initially promising outcome results is not yet available. In addition, many interventions that are now widely disseminated in the fields of education and rehabilitation have not received ample research on specific treatment effects. In particular, it is difficult in clinical research to control for non-specific treatment effects such as hope on the part of the adults involved, general

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encouragement and support delivered to children, and time spent with professionals. Unfortunately, the exasperation that some pediatric neuropsychologists feel about how some treatments can be promoted without thorough consideration of alternative explanations (such as the developmental disorders highlighted in Part II) or the specificity of treatment effects can sometimes degenerate into sarcasm or flippant jokes. We wish to examine these issues from a higher ground at the level of individual cases here. Part III begins with a pair of chapters each on sensory processing and auditory processing, to illustrate and further stimulate the discussion among the various disciplines related to pediatric neuropsychology. We have carefully chosen contributions to this section in light of their attention to issues of psychometric methodology, the range of normal functioning, construct validity, and discriminant validity in relation to other more commonly recognized constructs and disorders in clinical neuropsychology, rather than just relying on clinical or anecdotal impressions. As editors, we neither critique by absence nor uncritically endorse these ideas; rather, we include them in the discussion. In addition, we do not intend to promote new diagnoses or disorders, per se. In our evolving field, divergent ideas need to be considered with sufficient open-mindedness to allow theoretical growth and interdisciplinary collaboration, while at the same time we promote methodological rigor and evidence-based intervention practice. The last two chapters illustrate questions about the role of neuropsychological testing in overall clinical evaluations of children’s learning, behavior, and emotional problems. One involves a case with clear neuropathology; the other, psychopathology. It is important to consider the place of ‘‘negative’’ or normal neuropsychological findings in teasing out ambiguous clinical presentations. We hope these final cases illustrate our clinical responsibility to avoid excessive testing and highlight that tests should not always be given just because they are available and might tell us something.

References Lurija, A. R. (1973). The working brain. New York: Basic Books. Mody, M., Studdert-Kennedy, M., & Brady, S. (1997). Speech perception deficits in poor readers: Auditory processing or phonological coding? Journal of Experimental Child Psychology, 64, 199–231. Tallal, P., Miller, S. L., Bedi, G., Byma, G., Wang, X., Nagarajan, S. S., Schreiner, C., Jenkins, W., & Merzenich, M. M. (1996). Language comprehension in language-learning impaired children improved with acoustically modified speech. Science, 271, 81–84.

Chapter 27

A ‘‘Sensational’’ Way to Understand and Serve Children: Illustration of a Sensory Processing Model Winnie Dunn

Introduction A growing body of literature suggests that the way people respond to sensory experiences in their everyday lives can both be characterized and serve as a useful assessment and intervention planning template (Dunn, 2001, 2007a, b; Dunn, Myles, & Orr, 2002; Myles et al., 2004; Rogers, Hepburn, & Wehner, 2003). Receiving and processing sensation is a core feature of brain activity, and therefore has the potential to influence many areas of performance and behavior. Understanding the patterns of sensory processing identified from research, and how these patterns affect children’s behavior, can be extremely useful for understanding complex situations with families and schools. Sensory processing knowledge broadens our ability to consider what might be influencing a child’s behavior and therefore provides insights about effective intervention possibilities. As this may be a new area of consideration for readers, we will review the basic concepts before presenting the case.

Historical–Theoretical Perspectives Approximately 50 years ago, Dr. A. Jean Ayres proposed a theoretical model for applying sound neuroscience principles to interventions for some children with ‘‘minimal brain dysfunction’’ (Ayres, 1979). She discussed the relationships among sensory input, brain activity, and the production of a response as important factors to understand when designing effective interventions for children. Because her focus was on organizing sensory information for use, she used the term sensory integration, a term used in neuroscience to reflect the interaction among the sensory inputs to create meaning in the brain, which in turn could drive the production of a response. The phrase ‘‘sensory integration’’ has come to stand for both the J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008

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neuroscience function and the applied science that Ayres proposed and tested in her extensive body of research (e.g., Ayres, 1989; Ayres & Tickle, 1980). As was consistent with the thinking of the decades of her work, children were considered to have ‘‘dysfunction’’ when their sensory integrative mechanisms seemed unable to support them in producing adaptive responses within their life activities. Rather, the children would react too intensely or not enough for the needs of particular situations (which would be termed ‘‘dysfunctional’’ behavior patterns). Sensory integration assessment identified children’s strengths and weaknesses in receiving and understanding various sensory inputs and linking them to poor adaptive response patterns. Sensory integration treatment focused on improving sensory inputs and adaptive responses (Fisher, Murray, & Bundy, 1991). Many authors have contributed to the sensory integration body of research (e.g., Case-Smith & Bryan, 1999; Cohn & Cermak, 1998; Coster, Tickle-Degnen, & Armenta, 1995; Dunkerley, Tickle-Degnen, & Coster, 1997; Fallon, Mauer, & Neukirch, 1994; Lai, Parham, & Johnson-Ecker, 1999; Schaaf & Anzalone, 2001; Smith-Roley, Blanche, & Schaaf, 2001; Tickle-Degnen & Coster, 1995), advancing the ideas over the last decades. In the current environment, just as during Ayres’ formative work, there are contextual issues as well as additional knowledge to consider when studying these constructs and when applying them to current practice demands. For example, there is a growing awareness of the importance of everyday contexts as critical to a child’s ability to generalize learning and use it when needed (Dunst & Bruder, 2002; Dunst et al., 2001; Dunst, Hamby, Trivette, Raab, & Bruder, 2000, 2002; Dunst, Trivette, & Cross, 2002). Currently available knowledge and new demands in practice settings press researchers and practitioners forward to consider concepts both more specifically and more broadly.

Contemporary Conceptual Perspectives With solid foundations of neuroscience and the application of neuroscience to research and practice in sensory integration literature, new ideas could emerge to advance these ideas. Dunn (1997) proposed a conceptual model about sensory processing, which reflects the interaction of one’s neurological thresholds for responding and selfregulation strategies. The model suggests four basic patterns of sensory processing: seeking, avoiding, sensitivity, and registration, each of which has a unique threshold and self-regulation combination (Table 27.1 illustrates these relationships). Table 27.1 Dunn’s model of sensory processing Neurological thresholds High thresholds Low thresholds Adapted with permission from Dunn (1997).

Self regulation strategies/behavioral responses Passive Active Registration Seeking Sensitivity Avoiding

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Dunn’s model evolved from the findings in a national sample of children without disabilities (Brown, Tollefson, Dunn, Cromwell, & Filion, 2001; Dunn, 1999, 2002, 2006, 2007a,b) using the Sensory Profile measure, a nationally standardized parent reporting questionnaire indicating the frequency a child reacts to the sensory events in everyday life represented in the items. Dunn and colleagues (Brown et al., 2001; Dunn, 1999, 2002, 2006, 2007a, b) used the model to test infants, adolescents, and adults, validating that the constructs in Dunn’s model seem to exist across the life span. Table 27.2 provides examples of items from the Sensory Profile and the Sensory Profile School Companion (used in this case). A neurological threshold is the point around which the nervous system is likely to notice and respond to stimuli; high thresholds require a lot of stimuli, while low thresholds require very little input. The self-regulation continuum reflects a person’s tendency to be active or passive in reacting; when active, a person engages in behavior to control sensory input and when passive, a person allows input to occur and then responds. When these continua interact, four patterns emerge (i.e., seeking, avoiding, sensitivity, registration). These patterns are present in everyone; they reflect how human beings manage sensory input, which occurs throughout the day. So these patterns do not represent a diagnosis or dysfunction; they merely describe the way the person tends to take in and respond to sensory events. The challenges with participation arise when a person’s sensory processing patterns begin to interfere with activities in everyday life. Seeking patterns reflect high neurological thresholds and an active self-regulation strategy; these persons enjoy sensory input and engage in behaviors to get more input so that they can meet their sensory thresholds. Seeking is helpful because the person is interested in detecting what is going on; this can lead to heightened awareness and even idea generation. Seeking can be challenging when all that searching for new input distracts the person from the tasks at hand, leading to diminished productivity. Avoiding patterns reflect low neurological thresholds and an active self-regulation strategy; these persons are easily overwhelmed by sensory input so they actively retreat

Table 27.2 Examples of items from the Sensory Profile measures Sensory Profile (completed by Sensory Profile School Companion parents) (completed by teacher) Seeking Takes excessive risks during play Watches other students when they move (movement) around the room (visual) Holds hands over ears to protect them from Avoiding Avoids getting ‘‘messy’’ such as in sound (auditory) paste, sand, finger paint, glue, tape (touch) Sensitivity Will only eat certain tastes (oral) Is fidgety or disruptive when standing in line or close to other people (touch) Registration Poor endurance, tires easily (body Is clumsy and awkward in movements (e.g., position) runs into desks and furniture when moving about) (movement)

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from experiences that are intense or unfamiliar. Avoiding is helpful because the person is skilled at finding isolated areas for completing work (if allowed to do so) and so can complete tasks in an effective manner. Avoiding can be challenging because learning and living environments are unpredictable, which can be overwhelming very quickly. Sensitivity patterns reflect low neurological thresholds with a passive selfregulation strategy; these persons are also easily bothered by stimuli and are more likely to experience the sensations and get upset rather than retreating. Sensitivity is helpful because the person’s high detection and need for precision can create very nice work products. Sensitivity can be challenging because the need for precision can interfere with deadlines and tasks requiring an overview perspective. Registration patterns reflect high thresholds and a passive self-regulation strategy; these persons miss a lot of sensory input because of high thresholds, and may seem oblivious because they fail to detect what others notice. Registration is helpful because the person is not distracted by environmental stimuli and so can concentrate on a task. Registration can be challenging because the person can miss directions or other cues indicating how something needs to be done. Research has indicated that children with certain disorders have a significantly higher frequency of behaviors related to these sensory processing patterns when compared with peers without disabilities, including children with autism, Asperger’s disorder, Attention Deficit Hyperactivity Disorder, and Learning Disabilities (Dove & Dunn, submitted; Dunn et al., 2002; Ermer & Dunn, 1998; Kientz & Dunn, 1997; Myles et al., 2004; Rogers et al., 2003; Tomchek & Dunn, 2007; Watling, Dietz, & White, 2001). Researchers have also hypothesized that there is a link between sensory processing patterns and successful participation (Dunn, 2005, 2007a, b; Myles et al., 2004; Rogers et al., 2003). Several studies have also demonstrated a link between the patterns of sensory processing in Dunn’s model and nervous system activity. Using skin conductance, Brown, Cromwell, Filion, Dunn, and Tollefson (2002) showed significantly different patterns of responsivity and habituation in young adults with distinct patterns of sensory processing from the Adolescent Adult Sensory Profile. Researchers obtained a similar result with children by recording skin conductance and Short Sensory Profile results; there were significant differences on the SSP in children with typical and abnormal skin conductance responses (McIntosh, Miller, Shyu, & Hagerman, 1999; McIntosh, Miller, Shyu, & Dunn, 1999; Schaaf, Miller, Sewell, & O’Keefe, 2003). Links have also been hypothesized between the patterns of sensory processing in Dunn’s model and patterns of temperament (Dunn, 2001). Using Rothbart’s temperament scales, Daniels (2003) showed significant relationships between sensation seeking and surgency and between low neurological threshold response patterns and negative affectivity. Horsey (2003) and Koenig (2003) also report that there are moderate relationships between temperament and sensory processing. Perhaps the behaviors we associate with various temperament patterns

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are a reflection of how the nervous system is processing sensory information (Dunn, 2001).

Sensory Profile Measures There are five different Sensory Profile measures that have been used to study these concepts, to validate patterns in disability groups, and to guide practice decision making. Each measure has been nationally standardized, has reported validity and reliability (within the manuals and in the literature), and discriminates among disability groups who have a high rate of differences in sensory processing when compared with peers. They are parent (for infants, toddlers, and children) or selfreporting (for adolescents and adults) measures. They use a 5-point Likert scale (almost always, nearly 100% of the time; frequently, about 75% of the time; occasionally, about 50% of the time; seldom, about 25% of the time; and almost never, 0% of the time) to report the frequency of responding to sensory events in everyday life in the manner described in each item. Infant Toddler Sensory Profile (birth to 3 years) Sensory Profile (3–10 years) and Sensory Profile Supplement Short Sensory Profile (3–10 years) (for screening and research protocols) Sensory Profile School Companion (3–11 years) Adolescent Adult Sensory Profile (11 years to 90 þ years) It has been traditional to accept direct observation of children’s behavior as the most accurate form of reporting. However, some behaviors are difficult or impossible to observe directly in constructed, contrived, or time-restrained situations, including how a child might act within a particular setting and in front of a particular observer (De Los Reyes & Kazdin, 2005). Some scholars suggest that because children’s behaviors vary across contexts, familiarity and roles (including professional backgrounds) can affect children’s behaviors, including making it impossible to observe some behaviors (Kohen, Brooks-Gunn, McCormick, & Graber, 1997; DuPaul, 2003). It is important to obtain multifaceted information. To minimize the effect of context, Kraemer et al. (2003) recommend using informants across contexts and with different roles. The Sensory Profile measures are informant reporting measures that take advantage of a parent’s or teacher’s familiarity with a child across time. Many of the behaviors that children exhibit indicating their patterns of sensory processing emerge within daily life and so would be difficult to trigger in a direct observation situation. Dunn (2007a,b) reports convergent and discriminant validity between the Sensory Profile from the parents and the Sensory Profile School Companion from the teacher report, suggesting that behaviors in context are important to understand. In practice, occupational therapists combine standardized testing with skilled observation and interviews to create hypotheses for intervention planning. When used in this way, the Sensory Profile measures offer external validation for the hypotheses being developed and tested.

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History and Reason for Referral Paul was a 6-year-old entering first grade at his neighborhood school. He lived with his parents and 4-year-old sister. Paul and his family received early childhood services during his preschool years, and he was diagnosed with Asperger’s Disorder after preschool as he entered kindergarten. During his kindergarten year, Paul’s parents placed him in a small private school; his teacher designed a very tight and individual structure for the half day experience, and Paul had a good year at school. His day care situation was more variable, with some good and some bad days. Paul’s parents enrolled him in the neighborhood public school for first grade. He started having multiple outbursts during the day, and his parents had to pick him up several times before the day was over. Paul’s parents and teacher wanted him to be successful in regular education, but they were frustrated about knowing what to do. Paul’s teacher talked to his parents, and they agreed that the teacher would involve the Planning Team in her building to help with some creative problem solving (school districts have different names for these teams; they consist of other teachers, an administrator, and usually a special education professional; their task is to support the classroom teacher by identifying alternatives for instruction and behavior management). They implemented some of the strategies the school uses for discipline, but these were not successful with Paul. For example, when Paul became agitated, he left his seat and roamed around the room. He got increasingly more aggressive and eventually destroyed materials and supplies in the classroom. He also screamed at the teacher and other children that they were not following the rules. When other children were disruptive, the teacher sent them to a quiet space set aside for calming down and talked calmly to them about what they needed to think about while sitting there. When the teacher tried to get Paul to go to the quiet space, he became more agitated and louder, and the teacher was afraid he might harm someone. The Planning Team, teacher, and Paul’s parents decided they wanted to involve special education services to provide additional supports for the classroom. The formal process proceeded, and the school psychologist, behavior specialist, and occupational therapist became involved in the process.

Test Results The school psychologist administered the Wechsler Intelligence Scales for Children – Fourth Edition and the Woodcock Johnson Psychoeducational Test Battery. The findings from these measures are summarized in Tables 27.3 and 27.4. She reported that Paul had average to above average intelligence, with somewhat stronger verbal skills. On the psychoeducational testing, Paul was stronger in math (above average expectations) than reading and writing (average expectations). This testing indicated that Paul was capable of being a successful student. The behavior specialist conducted several skilled observations to record Paul’s classroom behaviors, look for antecedents to his outbursts, and note what was going

27 A ‘‘Sensational’’ Way to Understand and Serve Children Table 27.3 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal comprehension index Perceptual reasoning index Working memory index Processing speed index Full scale IQ a Standard score, (scaled score), [T-score], {z-score}.

Table 27.4 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition, Form A Letter–word Reading fluency Passage comprehension Calculation Math fluency Applied problems Spelling Writing fluency Writing samples

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Scorea 115 108 110 92 106

Score 111 85 90 125 130 119 110 91 115

Percentile 84th 70th 75th 30th 66th

Percentile 77th 16th 25th 95th 98th 90th 75th 27th 84th

on when Paul was doing well. He also interviewed Paul’s teacher to gain her insights about the situation. He reported that Paul was more successful when there was a very specific task to complete, when the room was quiet, and when Paul was out of the highest activity areas of the classroom. Changes in routines, multiple activities, and being in proximity to others seemed to be the triggers for his outbursts. The occupational therapist’s primary concern along with her teammates was Paul’s successful participation as a student. She wanted to investigate what factors might be contributing to or interfering with Paul’s ability to manage every day. In reviewing the psychologist and behavior specialist evaluations, she noted that Paul was capable of participating in first grade, so basic cognitive concerns could be ruled out. The behavior specialist brought up some issues she wanted to pursue further related to his ability to manage in particular situations. She knew from the literature that children like Paul who have Asperger’s Disorder were likely to have significantly different sensory processing patterns from peers, and this difference in experiencing the world could be contributing to his outbursts in the classroom (Dunn, 2005; Dunn et al., 2002; Myles et al., 2004). She decided to evaluate using skilled observation and interviews with Paul’s teacher and parents to gather the necessary information. Table 27.5 summarizes some of the findings from these measures. Skilled observation and interviews revealed that Paul seemed to get overloaded very quickly with sounds in the environment. Whenever the teacher made transitions

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in activities, the students made more noise, and this seemed to set off Paul. He also had a difficult time when the class would get in line and sit on the floor together, and in the lunch room. The therapist observed that these were times when Paul was much more likely to get bumped by peers or jostled as things happened around him. He did better during structured classroom time in chairs with teacher direction and a specific task to accomplish. Paul’s parents reported that they had a very orderly structure at home for him, including his own places to play; they acknowledged that they organized this way out of their need to keep him calm at home. The findings verified that Paul had sensory processing patterns that were significantly different from his peers. As can be seen on Table 27.5, his parent and teacher reports were similar, but not exactly the same. This is because the measures are an assessment of the child in context; therefore, scores reflect the interaction between the child’s patterns of responding and the demands of that context. For example, Paul’s parents reported ‘‘typical’’ responses in sensitivity, while his teacher reported ‘‘more than others.’’ The classroom environment was much more active and variable than home for Paul; it was likely that he encountered his sensitivity to sounds and touch more frequently at school than at home. They both agreed that he was an avoider (he had very low thresholds and actively tried to get away from stimuli that bothered him), with a ‘‘much more than others’’ rating. It was quieter at home, but his parents still reported that Paul noticed auditory stimuli more than other children; the teacher noted that he detected sounds more than 98% of his peers. They both also noted that he responded to touch more than other children. His parents commented how hard it had been to bathe and dress him, although they had figured out what he would wear and they no longer forced the issue. The teacher structured the classroom so Paul was not close to the other children during large parts of the day, and this reduced the impact of touch sensitivities on his behavior.

Table 27.5 Summary of the Sensory Profile and Sensory Profile School Companion findings Selected scores from Sensory Profile findings Sensory Profile School Companion those available (from parents) findings (from teacher) Sensory processing patterns Seeking Typical Typical Avoiding Much more than others Much more than others Sensitivity Typical More than others Registration More than others Much more than others Sensory systems Auditory Visual Vestibular Touch Multisensory Oral

Much more than others Typical Typical More than others Typical More than others

Much more than others Typical More than others More than others Not applicable Not applicable

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Both Paul’s parents and the teacher reported that his behavioral responses to sensory stimuli were significantly more frequent than typical peers.

Formulation and Recommendations The team met to discuss the findings and preliminary impressions. Everyone was happy that Paul’s cognitive and educational abilities indicated he was capable of being successful at school. It was a particular relief to his parents, who were worried that he might be in over his head. The team focused on how to tap into Paul’s cognitive and educational abilities by examining other factors that might be interfering with his participation. The behavior specialist and occupational therapist both reported helpful data about Paul’s behaviors within the classroom. For example, they agreed that he had more challenges during transition times. The behavior specialist hypothesized that Paul needed clear contingencies during these times. The occupational therapist hypothesized that Paul was overwhelmed during transitions because these situations provide high intensity auditory and touch opportunities, which testing revealed were difficult inputs for Paul. His parents talked about challenging situations at home, like family gatherings, and agreed that these active times were hard for Paul. The team worked on a plan that combined creating contingencies and structure and also honored Paul’s need to limit sound and touch input during the school day. They worked with the teacher’s classroom schedule to identify ways to create routines, anticipatory cues, and positive feedback. The occupational therapist spent time with the teacher in the classroom to identify ways to make sure Paul’s sensation avoiding tendencies did not interfere with his learning. With the teacher understanding that Paul had very low thresholds for touch and sounds, they discussed ways to reduce these opportunities throughout the day so that Paul could continue to participate successfully. For example, they decided to send Paul ahead of others when they went to the library so he did not hear the noise of the class moving down the hall and did not get bumped by other children. When they went to lunch and recess, Paul could stand behind the classroom door, holding it open for the other children. He could see the children through the window, but the door protected him from bumping and dampened the sounds as well. The occupational therapist also explained Paul’s significantly different registration score to the team. In addition to being overwhelmed easily, Paul missed other important sensory inputs throughout the day. The team did not understand how Paul could both be overwhelmed and miss things. The occupational therapist explained that children like Paul have a very narrow range of noticing and tolerating stimuli. He did not notice, and then when the sensory input became bigger, he easily shut down (by either withdrawing or acting out to get away from the aversive stimuli). This was a dilemma for Paul’s parents and teacher because it left a very narrow range for paying attention and learning. His teacher and parents acknowledged that although his outbursts are the most difficult to deal with, they had all seen him be

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oblivious sometimes too (e.g., they would call his name numerous times to get his attention). The occupational therapist volunteered to spend more time observing to identify the range of stimulus intensity that was just right to keep Paul’s attention and not overwhelm him. The teacher wondered about the vestibular score. This score represented Paul’s ability to respond to movement stimuli; his parents said he was just like other children, while the teacher scored his responses in the ‘‘more than others’’ range. The occupational therapist explained that Paul’s clumsiness when moving about the school, like running into desks and knocking off supplies, were everyday behaviors that illustrated his challenge with responding to movement. On the basis of the teacher’s responses, he seemed to miss movement cues and so moved in a less coordinated way than would be expected of a child of his age. The behavior specialist said that his outbursts could be more disruptive because of this clumsiness, rather than anger or rage. They all agreed that understanding his sensory processing patterns helped them understand the nature of his behaviors a different way and would be useful in their planning. All of the additional explanations about Paul’s behaviors were helpful to his parents too. Without understanding the concepts, they had been making some of the adjustments in their family life just to get by with Paul. For example, they gave Paul a portable media player that he could use at family gatherings. He had been picky about clothing, so they had identified the socks, underwear, and clothing that he tolerated, and much of it was form fitting, without seams or tight elastic. They offered to provide rewards at home for good days at school, and the behavior specialist agreed to follow up and help them with a plan.

Additional Issues It is critical for teams to consider multiple explanations for the behaviors experienced in children like Paul. He could easily have been considered defiant or labeled as a child with a conduct or oppositional disorder. Additionally, strictly behavioral approaches to intervention were not likely to be successful since Paul also needed environmental, instructional, and contextual adjustments that were respectful of his sensory processing needs. As Paul had intact cognitive abilities, the team applied cognitive-behavioral strategies to help him understand his own needs in addition to providing integrated behavioral and sensory processing strategies throughout the school day. For example, the teacher and occupational therapist identified antecedents to his outbursts (e.g., transition times beginning) and taught Paul to get up and complete a task for the teacher during this time to take his focus away from the sounds and get him away from the children moving about. Giving Paul awareness increased his capacity to manage other situations.

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Box 27.1 Specific Recommendations for Paul, His Teacher, and His Parents 1. Managing Paul’s Avoiding Pattern (particularly related to sound and touch) ▪ Create a cue between Paul and the teacher to mark the beginning of transitions so Paul can complete a task during transitions (e.g., erase the board, get books to hand out, sharpen pencils) and rejoin the class when it gets settled. ▪ Place Paul’s desk in a location out of traffic so he does not get bumped during the day. ▪ Let Paul wear earphones during seatwork. ▪ Teach Paul to ask to go to the study area or library when he begins to get overwhelmed by the activity in the room. ▪ Send Paul ahead of others when moving to new locations (bathroom, lunch). ▪ Let Paul hold the door for other students to reduce bumping and dampen sounds. ▪ Organize structured activities at recess to minimize random sounds and touch while playing. ▪ Talk to Paul’s parents about form-fitting clothing for school because this will reduce the impact of intermittent light touch throughout the day. ▪ Teach Paul phrases he can use to ask others to be quieter; write a social story about them so he can practice outside of situations that he needs to use them. 2. Managing Paul’s Registration Pattern (particularly related to movement input [vestibular]): ▪ Give Paul a movable cushion for his desk chair to help keep him activated while sitting at his desk to work. ▪ Talk to Paul’s parents about the importance of form-fitting clothing to provide Paul with even and continuous sensory input to his skin and joints as he moves. ▪ Let Paul stand at a counter to work sometimes; this provides additional movement input and will help keep him adequately alert and attentive in class. l Let Paul keep his backpack on during class; the extra weight on his body will provide organized sensory input that enables him to keep focused.

Additional Resources Key Concepts and Terms Avoiding A pattern of sensory processing characterized by low neurological thresholds and an active self‐regulation strategy for responding. Neurological Threshold The point that the nervous system notices sensory stimuli. Registration A pattern of sensory processing characterized by high neurological thresholds and a passive self-regulation strategy for responding. Seeking A pattern of sensory processing characterized by high neurological thresholds and an active self-regulation strategy for responding. Self-Regulation The way that a person responds to sensory stimuli. Sensitivity A pattern of sensory processing characterized by low neurological thresholds and a passive self-regulation strategy for responding. Sensory Processing The way the brain receives, organizes, and makes meaning out of the sensory input received by the sensory organs.

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Sensory Profile Measures A family of nationally standardized measures that characterize a person’s patterns of sensory processing (i.e., Infant/ Toddler Sensory Profile, Sensory Profile, Short Sensory Profile, Adolescent/Adult Sensory Profile, Sensory Profile School Companion).

References Resources for Clinicians and Families Dunn, W. http://www.sensoryprofile.com Dunn, W. (2007a). Living sensationally: Understanding your Senses. Jessica Kingsley, United Kingdom. This book is written for the public, and explains with many examples, how sensory processing affects our everyday lives. Dunn, W. (2007b). Supporting children to participate successfully in everyday life by using sensory processing knowledge. Infants and Young Children, 20(2), 84–101. This article provides a summary of the work completed to date related to sensory processing, and provides examples for everyday practice. Dunn, W., Saiter, J., & Rinner, L. (2002). Asperger syndrome and sensory processing: A conceptual model and guidance for intervention planning. Focus on Autism and other Developmental Disabilities, 17(3), 172–185. This article provides a discussion about how to collaborate with educational and therapeutic strategies, and provides case examples. Myles, B. S., Hagiwara, T., Dunn, W., Rinner, L., Reese, M., Huggins, A., et al. (2004). Sensory issues in Asperger syndrome and autism. Education and Training in Developmental Disabilities, December. This article discusses the similarities and differences between children with autism and Asperger syndrome. http://classes.kumc.edu/sah/resources/sensoryprocessing/index.htm a learning website about sensory processing concepts, research and case studies www.sensoryprofile.com information about the Sensory Profile measures

Appendix Ayres, A. J. (1979). Sensory integration and the child. Western Psychological Services: Los Angeles. Ayres, A. J. (1989). Sensory integration and praxis tests. Western Psychological Services: Los Angeles. Ayres, A. J., & Tickle, L. S. (1980). Hyper-responsivity to touch and vestibular stimuli as predictor of positive response to sensory integration procedures by autistic children. American Journal of Occupational Therapy, 34, 375–381. Brown, T., Cromwell, R., Filion, D., Dunn, W., & Tollefson, N. (2002). Sensory processing in schizophrenia: Missing and avoiding information. Schizophrenia Research, 55(1–2), 187–195. Brown, C., Tollefson, N., Dunn, W., Cromwell, R., & Filion, D. (2001). The adult sensory profile: Measuring patterns of sensory processing. American Journal of Occupational Therapy, 55, 75–82.

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Case-Smith, J., & Bryan, T. (1999). The effects of occupational therapy with sensory integration emphasis on preschool-age children with autism. The American Journal of Occupational Therapy, 53(5), 489–497. Cohn, E. S., & Cermak, S. A. (1998). Including the family perspective in sensory integration outcomes research. The American Journal of Occupational Therapy, 52, 540–546. Coster, W., Tickle-Degnen, L., & Armenta, L. (1995). Therapist-child interaction during sensory integration treatment: Development and testing of a research tool. Occupational Therapy Journal of Research, 15(1), 17–35. Daniels, D. (2003). The relationship between sensory processing and temperament in young children. Doctoral Dissertation. In preparation as a journal article. De Los Reyes, A., & Kazdin, A. E. (2005). Informant discrepancies in the assessment of childhood psychopathology: A critical review, theoretical framework, and recommendations for further study [Electronic version]. Psychological Bulletin, 131, 483–509. Dove, S., & Dunn, W. (submitted). Sensory processing in children who have specific learning disabilities. Journal of Learning Disabilities. Dunkerley, E., Tickle-Degnen, L., & Coster, W. J. (1997). Therapist-child interaction in the middle minutes of sensory integration treatment. American Journal of Occupational Therapy, 51(10), 799–805. Dunn, W. (1997). The impact of sensory processing abilities on the daily lives of young children and their families: A conceptual model, Infants and young children, 9(4), 23–35. Dunn, W. (1999). The sensory profile manual. Psychological Corporation: San Antonio. Dunn, W. (2001). The sensations of everyday life: Theoretical, conceptual and pragmatic considerations. American Journal of Occupational Therapy, 55(6), 608–620. Dunn, W. (2002). The infant toddler sensory profile. Psychological Coorporation: San Antonio, TX. Dunn, W. (2005). A sensory processing approach to supporting students with autism spectrum disorders. In R. M. Simpson, & B. Myles (Eds.), Educating children and youth with autism. Pro Ed Publishing: Austin. Dunn, W. (2006). Sensory profile school companion. Psychological Corporation: San Antonio. Dunn, W. (2007a). The sensory profile supplement. Psychological Corporation: San Antonio. Dunn, W. (2007b). Supporting children in everyday life using a sensory processing approach. Infants and Young Children, 20(2), 84–101. Dunn, W., Myles, B., & Orr, S. (2002). Sensory processing issues associated with Asperger syndrome: A preliminary investigation. American Journal of Occupational Therapy, 56, 97–102. Dunst, C. J., & Bruder, M. B. (2002). Valued outcomes of service coordination, early intervention, and natural environments. Exceptional Children, 68(3), 361–375. Dunst, C. J., Bruder, M. B., Trivette, C. M., Hamby, D., Raab, M., & McLean, M. (2001). Characteristics and consequences of everyday natural learning opportunities. Topics in Early Childhood Special Education, 21(2), 68–92. Dunst, C. J., Hamby, D., Trivette, C. M., Raab, M., & Bruder, M. B. (2000). Everyday family and community life and children’s naturally occurring learning opportunities. Journal of Early Intervention, 23(3), 151–164. Dunst, C. J., Hamby, D., Trivette, C. M., Raab, M., & Bruder, M. B. (2002). Young children’s participation in everyday family and community activity. Psychological Reports, 91(3, Pt 1), 875–889. Dunst, C. J., Trivette, C. M., & Cross, A. H. (2002). Mediating influences of social support: Personal, family, and child outcomes. [References]. In J. Blacher, & B. L. Baker (Eds.), The best of AAMR: Families and mental retardation: A collection of notable AAMR journal articles across the 20th century (pp. 119–133). American Association on Mental Retardation: Washington, DC. DuPaul, G. J. (2003). Assessment of ADHD Symptoms: Comment on Gomez et al. (2003). [Editorial] [Electronic version]. Psychological Assessment, 15, 115–117.

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Ermer, J., & Dunn, W. (1998). The sensory profile: A discriminant analysis of children with and without disabilities. American Journal of Occupational Therapy, 52, 283–290. Fallon, M. A., Mauer, D. M., & Neukirch, M. (1994). The effectiveness of sensory integration activities on language processing in preschoolers who are sensory and language impaired. Infant Toddler Intervention, 4(3), 235–243. Fisher, A. G., Murray, E. A., & Bundy, A. C. (1991). Sensory integration theory and practice. F. A. Davis: Philadelphia. Horsey, N. (2003). The relationship between temperament and sensory processing in children diagnosed with autistic spectrum disorder (ASD) and children who are non-ASD. Clinical Psychology. Kientz, M. A., & Dunn, W. (1997). Comparison of the performance of children with and without autism on the sensory profile. American Journal of Occupational Therapy, 51, 530–537. Koenig, K. (2003). Behavioral responsiveness: The relationship between temperament and sensory processing. American Occupational Therapy Association National Meeting, Washington, DC. Kohen, D. E., Brooks-Gunn, J., McCormick, M., & Graber, J. A. (1997). Concordance of maternal and teacher ratings of school and behavior problems in children of varying birth weights. Developmental and Behavioral Pediatrics, 18(5), 295–303. Kraemer, H. C., Measelle, J. R., Ablow, J. C., Essex, M. J., Boyce, W. T., & Kupfer, D. J. (2003). A new approach to integrating data from multiple informants in psychiatric assessment and research: Mixing and matching contexts and perspectives [Electronic version]. American Journal of Psychiatry, 160, 1566–1577. Lai, J., Parham, D., & Johnson-Ecker, C. (1999). Sensory dormancy and sensory defensiveness: Two sides of the same coin? Sensory Integration Special Interest Section Quarterly, 22, 1–4. McIntosh, D. N., Miller, L. J., Shyu, V., & Dunn, W. (1999). Overview of the short sensory profile (SSP). In W. Dunn (Ed.), The sensory profile (pp. 59–74). Psychological Corporation: San Antonio, TX. McIntosh, D., Miller, L., Shyu, V., & Hagerman, R. (1999). Sensory modulation disruption, electrodermal responses and functional behaviors. Developmental Medicine and Child Neurology, 41, 608–615. Myles, B. S., Hagiwara, T., Dunn, W., Rinner, L., Reese, M., Huggins, A., et al. (2004). Sensory issues in children with Asperger syndrome and autism. Education and Training in Developmental Disabilities, 3(4), 283–290. Rogers, S., Hepburn, S., & Wehner, E. (2003). Parent report of sensory symptoms in toddlers with autism and those with other developmental disorders. Journal of Autism and Developmental Disorders, 33(6), 631–642. Schaaf, R., & Anzalone, M. (2001). Sensory integration with infants and young children In S. Roley, E. Blanche, & R. Schaaf (Eds.), Sensory integration with developmental disabilities. Psychological Corporation: San Antonio, TX. Schaaf, R. C., Miller, L. J., Sewell, D., & O’Keefe, S. (2003). Preliminary study of parasympathetic functioning in children with sensory modulation dysfunction and its relation to occupation. American Journal of Occupational Therapy, 57(4), 442–449. Smith-Roley, S., Blanche, E., & Schaaf, R. C. (Eds.). (2001). The Nature of sensory integration with diverse populations. Psychological Corporation: Tuscon, AZ. Tickle-Degnen, L., & Coster, W. (1995). Therapeutic interaction and the management of challenge during the beginning minutes of sensory integration treatment. Occupational Therapy Journal of Research, 15(2), 122–141. Tomchek, S. D., Dunn, W. (2007). Sensory processing in children with and without autism: A comparative study utilizing the short sensory profile. American Journal of Occupational Therapy, 61(2), 190–200. Watling, R., Dietz, J., & White, O. (2001). Comparison of sensory profile scores of young children with and without autism spectrum disorders. American Journal of Occupational Therapy, 55(4), 416–423.

Chapter 28

Sense and Sensibility: Relating Behavior Control Issues with Self-Regulation of Sensory Input Robert F. Newby and Winnie Dunn

Jennifer was referred for a neuropsychological evaluation as a supplement to a psychological evaluation for behavior problems and a previous history of academic difficulties. Her prenatal development, birth, and early infancy were normal. Early developmental milestones were attained at the normal rate for both motor and language functions. There were no significant head injuries and no other neurological insults. Since 5 years of age, during times of stress, she has gone through episodes lasting up to a month of often closing her eyes and shaking her head. Her primary care physician had ruled out Tourette’s disorder, but she had not had a neurological evaluation. Jennifer’s parents reported in interview that she has always shown very high sensitivity to sound, touch, and smell. Vision and hearing screening tests were normal. Jennifer was home-schooled for kindergarten through second grade, when her father was working for an international corporation in Thailand. She then repeated second grade when the family returned to the United States, so she was older than typical students in fourth grade at the time of her neuropsychological assessment. Questions arose during the early elementary years about her performances in all academic areas, although her reading had improved since and she was said to be successful in math ‘‘if she figured it out her own way.’’ Individually administered achievement testing by her school when she started her repeat year of second grade was below average, but both group-administered standardized achievement testing of all students and individually administered achievement tests at school during fourth grade had risen to the average or above average range in all areas. Intelligence testing by a private psychologist 1 month prior to the neuropsychological evaluation was in the average range, with equivalent verbal reasoning skills and visual-spatial problemsolving skills (Table 28.1). Among core verbal subskills, Jennifer showed high average vocabulary knowledge, average common-sense social reasoning, and above average word comparison skills, or abstract verbal conceptualization. She was well above average on a cluster of subtests sensitive to verbal working memory. Among core visual-spatial subskills, she was average in abstract visual

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296 Table 28.1 Intelligence Wechsler Intelligence Scale for Children – Fourth Edition Full Scale IQ General Ability Index Verbal Comprehension Index Similarities Vocabulary Comprehension Perceptual Reasoning Index Block Design Picture Concepts Matrix Reasoning Working Memory Index Digit Span Letter-Number Sequencing Processing Speed index Coding Symbol Search a Standard score, (scaled score), [T-score], {z-score}.

R.F. Newby, W. Dunn

Scorea 108 108 110 (14) (13) (9) 104 (11) (11) (10) 126 (17) (12) 80 (6) (7)

Percentile 70th 70th 75th 91st 84th 37th 61st 63rd 63rd 50th 96th 99th 75th 9th 9th 16th

construction when copying block design patterns, average in discerning concepts in pictures, and average in visual analogies. She was mildly impaired on a cluster of subtests that reflected visual-perceptual processing speed. These findings were virtually identical to previous testing at school in second grade. Jennifer’s behavior patterns over the years included arguing, temper tantrums, frequent oppositional behavior, difficulty with change or with disruptions in how she wants things, and overfocused interests (not including anxious obsessions or compulsive patterns). She had shown strong social interest since early childhood, but her social perception and social skills were poor, so her teacher requested a consultation with the school occupational therapist. The focus of occupational therapy assessment and intervention was on Jennifer’s ability to participate in activities and settings of interest or need in everyday life, including home, school, and community. The comprehensive assessment initially focused on identifying participation successes and challenges. Then the therapist investigated what factors were likely to support or interfere with her participation. Intervention planning evolved from the interaction of these findings. Observations by the occupational therapist documented that Jennifer seldom interacted with peers in the classroom and in the hallways. The occupational therapist recommended that the teacher arrange Jennifer’s seating so she would have as few interruptions from peers and other classroom activities as possible while trying to complete seatwork, yielding improved productivity when she was working on simpler review material. They also arranged for Jennifer to sit at the end of a table at lunch to reduce bumping and jostling with peers; with this change, she ate well and interacted with peers for brief episodes during lunch. Jennifer was more agitated in the classroom than other students. She talked to herself while working, scratched out and crumpled up papers, and finished more

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challenging work after peers. The teacher had placed her in a location to minimize bumping (out of the aisle, away from the door, away from supplies) and to reduce the impact of noise (with her desk next to the back of a freestanding bookshelf, facing away from other students). The teacher also told the occupational therapist that Jennifer was free to go to the library to work if she wished, and the teacher sometimes cued this move if she noticed Jennifer escalating in the classroom. Two other contextual factors were important to consider in the integrated neuropsychological evaluation: First, several professionals involved in the other recent evaluations raised a question of Asperger’s Disorder. Second, conflicts and stresses in family interactions were acknowledged, and Jennifer’s family history included attention problems, anxiety, depression, behavior patterns similar to Jennifer’s (social difficulties and what her parents labeled ‘‘obsessive–compulsive traits’’ but on careful questioning were more like rigid responses in social situations), and epilepsy.

Test Results The recent psychological evaluation and the previous multiple assessments of academic achievement allowed a focus on a broad array of other neuropsychological functions, including motor, attention, executive, social reasoning, and memory, as well as additional assessment of mental processing speed. Jennifer’s behavior difficulties and reported oversensitivity in several sensory modalities were further documented with standardized behavior rating questionnaires completed by parents and teachers (Table 28.2). Concurrent with the neuropsychological testing at the medical center, the school occupational therapist also conducted further skilled observations and considered teacher, parent, and record information to verify sensory processing responses within daily routines. Jennifer’s effort and cooperation during testing were variable, so some of the results (as specified) seemed to underestimate her actual skills; the bulk of results were considered valid and reliable. Her low frustration tolerance was apparent immediately upon beginning testing, when she became overwhelmed by the first question, scowled, rested her head on the table, and muttered to herself angrily. She responded to the rest of the tests with similar frustration despite encouragement and praise from the examiner. Regardless of Jennifer’s irritability, she begrudgingly completed the tasks required of her. Her eye contact was intermittent, especially when frustrated, but during conversations with the examiner, her eye contact was more consistent. She did not smile often during testing, but she appeared briefly content when completing two tasks that were not particularly challenging for her. During the most challenging tasks, she was argumentative and agitated. She questioned the examiner repeatedly (e.g., ‘‘If you would just read the words slower, I would be able to remember them’’) and fidgeted in an agitated manner (e.g., coughing, kicking the table). Jennifer did not demonstrate excessive impulsivity, though she sometimes began tasks before the examiner completed the instructions.

298 Table 28.2 Other cognitive functions Tests Raw score Grooved Pegboard Test Trial 1 right hand Trial 1 left hand Trial 2 right hand Trial 2 left hand Gordon Diagnostic System Vigilance Task (9 min.) Correct 42 Commission errors 2 Restricted Academic Task Off-task Fidgeting Vocalizing Playing with objects Out of seat 0 Trailmaking Test Part A 65 s Part B 55 s Wisconsin Card Sorting Test Categories Total errors Perseverative responses Perseverative errors Nonperseverative errors Conceptual level responses Failure to maintain set Intermediate Booklet Category Test The Tower of London Test of Problem Solving Explaining inferences Determining causes Negative why questions Determining solutions Avoiding problems Total test Beery-Buktenica Developmental Test of Visual-Motor Integration Wide Range Assessment of Memory and Learning – Second Edition Story memory – immediate Delayed recall Delayed recognition Verbal learning – immediate Delayed recall Delayed recognition

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Score

Percentile

94 86 83 73

34th 18th 13th 4th

Interpretation

90th 30th 40th 85th

1st <1st

108

>16th 32nd 86th 84th 9th 25th >16th 70th

124

95th

94 88 67 97 82 79 103

34th 21st 1st 42nd 12th 8th 58th

(13) (12) (11) (8) (8) (7)

84th 75th 63rd 25th 25th 16th

93 116 115 80 90

(continued )

28 Self-Regulation of Sensory Input Table 28.2 (continued) Tests Design memory – immediate Delayed recognition Wide Range Assessment of Memory and Learning Visual learning – immediate Delayed recall Attention Deficit Hyperactivity Disorder Rating Scale – Fourth Edition Home version Total Hyperactivity-impulsivity Inattention School version Total Hyperactivity-impulsivity Inattention Achenbach Child Behavior Checklist Parent Form Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior Teacher’s Report Form Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior Sensory Profile Sensory Processing Patterns Seeking Avoiding Sensitivity Registration Auditory processing Visual processing Vestibular processing Touch processing

Raw score

299

Score (5) (9)

Percentile 5th 37th

(9) 107

37th 68th

Interpretation

90th 98th 80th 50th –75th 50th–75th 50th

100/130 95/145 89/100 68/75 27/40 33/45 50/55 78/90

[69] [66] [53] [60] [78] [67] [64] [86]

97th 95th 62nd 84th >97th 96th 92nd >97th

[68] [63] [50] [59] [66] [64] [62] [69]

97th 90th 50th 81st 95th 92nd 89th 97th

Probable difference Definite difference Typical performance Typical performance Probable difference Typical performance Typical performance Typical performance (continued )

300 Table 28.2 (continued) Tests Multisensory processing Oral sensory processing Sensory processing related to endurance/tone Modulation related to body position & movement Modulation of movement affecting activity level Modulation of sensory input affecting emotional responses Modulation of visual input affecting emotional responses and activity level Behavior and Emotional Responses Emotional/social responses Behavioral outcomes of sensory processing Items indicating thresholds for response Registration Seeking Sensitivity Avoiding School Companion Seeking Avoiding Sensitivity Registration Sensory Processing and Behavior Patterns Auditory Visual Movement Touch Behavior School factor 1 School factor 2 School factor 3 School factor 4

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Raw score 27/35 52/60 43/45

Score

Percentile

Interpretation Typical performance Typical performance Typical performance

41/50

Typical performance

25/35

Typical performance

19/20

Typical performance

15/20

Typical performance

56/85 13/30

Probable difference Definite difference

14/15

Typical performance

69/75 98/130 85/100 98/145

Typical performance Probable difference Typical performance Definite difference

55/60 52/85 60/80 74/85

Typical performance Definite difference Typical performance Typical performance

34/50 47/55 63/70 55/60 38/75 70/105 51/65 76/85 39/55

Probable difference Typical performance Typical performance Typical performance Definite difference Probable difference Typical performance Typical performance Probable difference

Jennifer used her right hand as her dominant hand for manual-motor activities. Her graphomotor (paper-and-pencil) skills were average when copying geometric drawings. Fine manual dexterity was average with her dominant hand and low average with her nondominant hand.

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Assessment of attention, impulse control, and related executive mental functions was generally adequate, with occasional inconsistencies associated with effort and/or feelings of frustration. When Jennifer was asked to respond to a specified number sequence flashing on a small computer screen over a several-minute period, her sustained attention was in the normal range, and her number of incorrect impulsive responses was normal. When asked to work on math problems alone while being observed through a one-way window for 10 min, she followed directions minimally, with excessive off-task time playing with distracter toys on the table, but her motor control was not outside the expected limits. Her planning and sequential problemsolving were well above average. Her abstract reasoning and concept formation with complex visual material were high average. She showed average basic concept formation, adaptive switching of mental sets, and maintenance of mental sets, but her performance was not as consistent as on the previous two tests because of frustration and apparent overcomplicated thinking for the task. Her visual searching speed was mildly to moderately impaired with a simple number sequence and moderately impaired with an alternating number and letter sequence; the lack of significant difference between these two findings implicated processing speed more than executive skills. Her social perception and social reasoning were mildly impaired. Several memory skills were assessed. All aspects of verbal memory were average to high average. Most visual memory indicators were average, with some inconsistencies when reproducing drawings from memory. Jennifer’s mother’s responses to the Home Situations Questionnaire indicated mild to moderate compliance problems in most home and community settings. The Home Version of the ADHD Rating Scale – IV showed clinically significant hyperactive/ impulsive symptoms and normal range inattentive symptoms. The multidimensional Child Behavior Checklist showed highly clinically significant oppositional/aggressive behavior; clinically significant anxious/depressed symptoms and particularly sleep problems; and clinically borderline attention problems and withdrawal. On a parallel set of questionnaires completed by Jennifer’s fourth grade teacher, moderate compliance problems were noted in all classroom settings and in several less-structured situations outside the classroom at school. The School Version of the ADHD Rating Scale – IV showed normal range hyperactive/impulsive symptoms and normal range inattentive symptoms. The Teacher Report Form of the Child Behavior Checklist showed clinically significant oppositional/aggressive behavior and anxious/depressed symptoms. On the one hand, in an accompanying note, the teacher expressed concern about Jennifer’s increased irritability, frustration, and anger across the school year, with occasional physical aggression. On the other hand, Jennifer was described as smart with a fun sense of humor. Jennifer’s parents completed the Sensory Profile, which is a nationally standardized measure that characterizes children’s responses to sensory experiences in everyday life (Dunn, 1997, 2006) (see Chap. 27 for details about these measures and about Dunn’s model of sensory processing). Most of Jennifer’s scores on the Sensory Profile were just like her peers’, suggesting that she was primarily processing sensory input the way other children of her age do. Her parents reported that she sought sensory input more than other children (i.e., þ1 to þ2 standard deviations [SD]), and

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avoided sensory input much more than peers (i.e., more than þ2 SDs). At first this might seem contradictory; however, according to Dunn’s model of sensory processing (Dunn, 1997), seeking and avoiding patterns indicate the child’s need for control over sensory input (seekers try to get more input by adding to or intensifying sensory experiences, while avoiders try to get less input by withdrawing or acting out to get removed from situations). The only specific sensory modality system that was of concern on the Sensory Profile was auditory processing (i.e., þ1 to þ2 SDs), although Jennifer’s parents verbally reported that she also overreacted to touch and smell at home. Other scores that fell into the ‘‘more than others’’ category were behavioral patterns that could be associated with difficulty with sensory processing (i.e., Emotional/Social responses and Behavioral Outcomes of sensory processing). The teacher completed the Sensory Profile School Companion, and reported that Jennifer required more teacher attention (School Factor 1) and was less tolerant (School Factor 4) than other students on the basis of Sensory Profile School Companion results. She showed an average amount of seeking, sensitivity, and registration, but she avoided stimuli much more than peers (more than þ2SDs). Just like the parents, the teacher indicated that Jennifer was challenged with auditory input and demonstrated much more behavioral outcomes of sensory processing than peers (more than þ2 SDs). Consistent with the parents’ comments, but not with their standardized scores on the Sensory Profile, the teacher reported that Jennifer had more challenges with touch than peers as well. In comparing the parent and teacher reports of sensory processing, it was notable that Jennifer’s parents reported more seeking than the teacher did. School provides more structure and therefore can provide boundaries for choosing behaviors that increase or intensify sensory experiences (e.g., hopping instead of walking, humming, or singing). The structure at school may be an important factor to build into learning and intervention activities.

Formulation and Recommendations Jennifer’s most prominent issues were felt to be best represented by the diagnosis of Oppositional Defiant Disorder (ODD), with some accompanying features of both Asperger’s Disorder (especially rigidity and social reasoning difficulties) and mood disorder (especially irritability). Her neuropsychological profile was essentially normal, except for mental processing speed and social reasoning, each of which was felt likely to be related to her behavioral issues. The ODD diagnosis was based on her classic, persistent pattern of negativistic, defiant, disobedient, or hostile behavior toward authority figures, with stubbornness, resistance to directions, and unwillingness to compromise. Although sometimes the oppositional behavior is directed particularly at certain adults but not other adults, and family interaction patterns often need to be examined to understand how to change the opposition, Jennifer’s ODD difficulties were prominent at both home and school. Jennifer had a lifelong pattern of being more sociable than children who receive a full diagnosis of Asperger’s Disorder or another

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autistic spectrum disorder, but her features of this spectrum would need to be taken into account in treatment planning. Her possible mood disorder was felt to deserve attention in psychotherapy and further evaluation by a psychiatrist, to determine whether medication would be recommended to stabilize her mood. The occupational therapist noted that Jennifer’s scores on the Sensory Profile were primarily relevant as they informed the team about the possible factors that interfered with or support participation. For Jennifer, working successfully at school and developing social interactions were the primary goals for her parents and teachers. In her case, sensory processing findings did not fully explain the magnitude of behaviors. On the one hand, as reported by the neuropsychologist and others, her behaviors were consistent with other diagnoses (ODD, Asperger patterns). On the other hand, Jennifer’s specific patterns of sensory processing would be expected to lead to some of the same behaviors. We would not eliminate these diagnoses by incorporating sensory processing constructs into intervention planning. However, with sensory processing knowledge, we could create activities and environments that were less challenging for Jennifer’s nervous system, thereby reducing the frequency and intensity of her negative responses. When Jennifer is not pushed past her capacity to deal with sensory experiences, she may be more available to complete schoolwork and learn social interaction strategies. In terms of broad functioning, Jennifer participated in several activities successfully at school when activities were more structured, including most academic instruction and practice. Social situations with peers and siblings were more challenging for her. Her teacher and parents wanted Jennifer to have a successful school experience both academically and socially. Fortunately, Jennifer did not show compelling signs of learning disability in recent academic assessments, even though questions in this area had arisen in the past. The latter may have been related to her atypical school experience during early elementary years, particularly considering her oppositional behavior during her home-schooling time. Ultimately, the important question is whether Jennifer’s heightened control responses to sensory input, interestingly seen both in the areas of seeking and avoiding, should be considered contributory, causative, or primary in reference to her oppositional, mood, and social difficulties. However, this question can be answered only at a theoretical level at this time. Further research on this sensory processing model needs to demonstrate methods such as careful longitudinal measurement of all behavior patterns involved, well-controlled intervention studies, and structural equation modeling. For instance, the sensory findings in this case could represent a constitutional, relatively stable, but potentially modifiable factor that is independent from (though likely correlated with) temperament or mood factors. Conversely, the sensory response patterns identified by the Sensory Profile could represent facets or ‘‘offshoots’’ of the predominant oppositional behavior patterns that Jennifer had developed in interpersonal relationships over the years. It would be difficult to identify which among a multiple-approach set of interventions for a case like Jennifer’s was most operative in producing adaptive change, if the interventions were successful. This represents the classic dilemma of interpreting cause and effect with anecdotal rather than experimental evidence. The follow-

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ing list of recommendations included ideas from the knowledge bases about both the psychiatric disorders and sensory processing patterns involved.

Supporting Schoolwork The findings suggested that sounds may interfere with Jennifer’s school participation. This could have been related to her relatively late immersion in classroom situations compared with other children of her age, in light of her early home schooling. Since classrooms are busy places, there are many sounds to contend with throughout the day. Jennifer may have already reached her capacity to manage auditory input from her bus ride and getting into her classroom, which may contribute to her challenging attitude at the start of the day. There were inconsistent findings about touch, but it seemed that at least in some situations, touch was also challenging. School and home introduce different challenges related to touch. On balance, children have more options for getting away from bothersome touch at home. Jennifer’s parents reported during an interview that she was sensitive to touch when she had to contend with crowded situations (family gatherings, social hour after church, waiting in line at the grocery store), even though, overall, her standard score was in the typical range on the Sensory Profile. At school, Jennifer encountered many more ‘‘crowding’’ situations, which combine auditory and touch experiences, and so the combination of these challenging inputs may be too much for her, contributing to unpleasant behaviors. The fact that seeking and avoiding are the sensory processing patterns associated with a need to control sensory input was consistent with other findings about Jennifer’s controlling behaviors. It was not clear from the comprehensive findings what the source of control needs were (i.e., behavioral or sensory), but nonetheless, knowing that she sought control could be a guidepost for building better work patterns. Giving choices is important; her teacher was already using some strategies to give her control, and the school team could build on these to increase her classroom work productivity. For example, she could be given several work assignments and be allowed to work in whatever order she likes. She could choose the location for work, giving her quiet and isolated options.

Supporting Social Participation Jennifer’s teacher and parents were also concerned about her social interactions. As with schoolwork, her sensory processing patterns could be contributing to her negative and rigid behavior. Children who are avoiders tend to isolate themselves as a strategy for reducing ‘‘random’’ sensory input. It is important to remember this because Jennifer would probably benefit from time alone every day, which should not be seen as an antisocial gesture but, rather, a time for her to regroup and calm down from what she perceives as an onslaught of auditory and touch input (avoiders have low thresholds for sensory input, so it does not take very much to feel overwhelmed).

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Structured social interactions would be more successful with sound and touch managed within the routine. If Jennifer did not have to worry so much about extra sensory input, then she would be more available to learn social skills within the context of school activities. An additional reason to manage sensory input for her is that her outbursts may be partially a reflection of her sensory processing as well; when she is feeling overwhelmed, acting out stops the overwhelming situation quickly. The team decided to incorporate both behavioral and sensory processing ideas to create structured social interaction opportunities for Jennifer. Being mindful about seating arrangements (e.g., placing her in the corner of the group facing out), background noise (quiet, isolated place), level of difficulty (practicing social skills with easy tasks because Jennifer did better when work was less complex), and requirements for taking turns were felt likely to help her participate successfully. Building social interaction skills systematically would be helpful for both Jennifer and the other children in her classroom. It could also be useful to identify situations in which Jennifer could be seen as an expert. Perhaps she could help someone with math, reading, or social studies since she did well in these areas. The individual peer-assistance situation naturally reduces touch and sound interferences, and serving as the ‘‘expert’’ can provide opportunities for being seen positively by peers.

Psychotherapy and Parent Coaching Children exhibiting oppositional and defiant behaviors along with attention problems typically benefit from some form of structured behavior management program implemented in both the home and school settings. For Jennifer, it was predicted that progress might be gradual as her mood and flexibility problems were also addressed in psychotherapy. Behavioral programming should include the use of clear and specific commands, noncontingent quality time with her parents, the targeting of positive behaviors to be increased with reinforcement, the consistent use of negative consequences delivered in an emotionally neutral manner in response to inappropriate behaviors, and a greater proportion of positive incentives than reprimands. Such strategies should help to keep behavior problems from escalating to a point at which tempers are lost. Family psychotherapy is often helpful in shifting relationships and communication patterns among family members, particularly regarding authority.

Further Medical Evaluation Jennifer’s occasional episodes of closing her eyes and shaking her head were not likely to represent a neurologic condition, such as seizures, but an evaluation by a neurologist was felt to be prudent to rule out this possibility. Jennifer’s overall picture was clearly complex, making the interdisciplinary collaboration particularly valuable, at both the assessment and intervention levels. A balanced combination of viewpoints in this case avoided polemic arguments about the primacy of one theory vs. another, while providing important clarification

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and putting into perspective some hypotheses that had been raised (particularly, the question of autistic spectrum disorder).

Additional Resources Key Concepts and Terms Sensory Processing The way the brain receives, organizes, and makes meaning out of the sensory input received by the sensory organs. Sensory Profile Measures A family of nationally standardized measures that characterize a person’s patterns of sensory processing (i.e., Infant/Toddler Sensory Profile, Sensory Profile, Short Sensory Profile, Adolescent/Adult Sensory Profile, Sensory Profile School Companion). Seeking A pattern of sensory processing characterized by high neurological thresholds and an active self regulation strategy for responding. Avoiding A pattern of sensory processing characterized by low neurological thresholds and an active self regulation strategy for responding.

References Resources for Clinicians and Families Dunn, W. (2007). Supporting children to participate successfully in everyday life by using sensory processing knowledge. Infants and Young Children, 20(2), 84–101. This article provides a summary of the work completed to date related to sensory processing, and provides examples for everyday practice. Dunn, W., Saiter, J., & Rinner, L. (2002). Asperger syndrome and sensory processing: A conceptual model and guidance for intervention planning. Focus on Autism and other Developmental Disabilities, 17(3), 172–185. This article provides a discussion about how to collaborate with educational and therapeutic strategies, and provides case examples. Dunn, W. (1997). The impact of sensory processing abilities on the daily lives of young children and their families: A conceptual model. Infants and young children, 9(4), 23–35. Dunn, W. (2006). Sensory Profile School Companion. San Antonio, the Psychological Corporation http://www.sensoryprofile.com Information about the Sensory Profile measures.

Chapter 29

Elusive, Inclusive, or Conclusive? (Central) Auditory Processing Disorder Susan Oliff Carneol

At age 4, Michelle was a cheerful and creative little girl, but something set her apart from her peers. She had an unremarkable birth history, had been relatively healthy, and achieved her early speech, language, and motor milestones within age expectations. Despite these positive indicators, her parents felt a nagging concern about her that they could not identify. Michelle’s paternal uncle had a history of learning and behavior difficulties, but her parents felt that his problems were different than hers. As a preschooler, she was immature, inattentive, and impulsive compared with other children of her age. She had difficulty maintaining eye contact and following directions, and did not always respond when her name was called. She often lacked energy to complete physical activities expected for her age. Her parents’ suspicions were somewhat confirmed when Michelle did not do well on the language and motor portions of a kindergarten screening. This prompted their quest to find out what was causing her developmental and social difficulties. Over the course of the next 5 years, she underwent two neuropsychological evaluations, three comprehensive Individualized Education Program (IEP) assessments, as well as informal assessments by academic tutors. None of the testing resulted in a satisfactory diagnosis. At age 9, Michelle was a personable and upbeat third grader with decent grades on her report card. She was doing grade-level work in reading, spelling, and writing. She worked with an after-school tutor primarily for math, but she also received help in science and social studies. Michelle did not receive any special education support at school because she did not meet the state’s eligibility criteria for programming. Her parents encouraged her to pursue hobbies that she enjoyed, such as drawing, writing, reading, and participating in drama activities. They and her teachers agreed that Michelle learned best in one-on-one situations vs. large group settings. She required verbal directions to be repeated and/or broken down into smaller units and benefited when visual cues were added to the spoken message. Michelle had a difficult time with verbal expression. She often used words that did not apply or seemed inappropriate for the topic. Although Michelle was described as outgoing and friendly, she had difficulty interacting with peers. All these issues were a source

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of concern and frustration for Michelle, her parents, and teachers, especially since she always demonstrated average performance on various test batteries for intelligence, learning, and communication. Her private tutor recognized that she was capable of understanding the subject material in their one-on-one tutoring sessions and wondered why Michelle was so lost in the classroom. The tutor questioned an Auditory Processing Disorder. After researching this diagnostic label on the Internet, Michelle’s parents requested a comprehensive evaluation to rule out a (Central) Auditory Processing Disorder [(C)APD] (Table 29.1). Michelle’s doctor referred her to the outpatient speech and hearing clinic at a major pediatric medical center for a multidisciplinary team assessment. Auditory Processing Disorder is a term that has been used by many professionals in learning and communication disorders for the past 50 years. It is a complex problem that can mean different things to different people. Generally speaking, ‘‘auditory processing’’ implies how the brain recognizes and interprets sound. ‘‘Disorder’’ implies that something adversely affects the processing or interpretation of the auditory signal. Some professionals propose that an ‘‘Auditory Processing Disorder’’ occurs when the acoustic stimuli becomes distorted or compromised before the cortex receives it for interpretation, despite normal hearing acuity. Comorbidity of language impairments, memory problems, attention deficits, low cognitive ability, learning disabilities, and other behavioral/emotional difficulties are common. This has led many to question whether the disorder actually exists as a stand-alone diagnosis. Some argue that the term is overused. The American Speech-Language-Hearing Association (ASHA) defines (C)APD as ‘‘difficulty in processing auditory information in the central nervous system’’ (hence the ‘‘C’’). A (C)APD is demonstrated by poor performance on one or more auditory skills. Children may be misdiagnosed with (C)APD because of lack of thorough assessment to best delineate the nature of the child’s difficulties. Other professionals feel that there may be children who have a ‘‘true’’ auditory processing disorder but go undiagnosed because those involved are not oriented to the disorder. Research is currently underway to better understand the neurological and cognitive variables of a (C)APD and the functional impact it can have on an individual’s life. Research is also focusing on the efficacy of evidence-based practices and the appropriateness of various interventions. ASHA (2005) states that there is enough scientific evidence to support (C)APD as a diagnostic entity. Table 29.1 Symptoms of a possible (C)APD l

Trouble listening and attending in noisier situations

l

Difficulty remembering information presented orally

l

Problems carrying out multi-step oral directions

l

Difficulty expressing thoughts and ideas in cohesive manner

l

When speaking, may confuse syllable sequences or transpose sounds in words

l

Difficulty developing and using vocabulary

l

Low academic achievement, particularly in language based subjects

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It is important to understand the neurophysiological constructs of hearing to better understand (C)APD. Hearing begins when sound energy is captured by the outer ear. It is then transformed into mechanical energy as the tiny bone structures of the middle ear begin to move. The energy is changed to compressed waves of fluid in the inner ear, which stimulate hair-like nerve cells transmitting impulses up the eighth auditory nerve to Heschl’s gyrus in the left temporal lobe of the brain. At this point, the brain recognizes the nerve impulse as sound and begins to interpret it as speech or nonspeech auditory stimuli. The auditory stimuli networks within different areas of the brain and across hemispheres depending on the task (i.e., listening, talking, reading, writing, etc.). The diagnosis can only be made by an audiologist with advanced training and experience in (C)APD, and that audiologist must collaborate with speech-language pathologists, educators, psychologists, and parents, for example, to determine the functional impact of the disorder. Although several variables impact listening, the diagnosis of (C)APD requires demonstration of a deficit in the neural processing of the auditory stimuli that is not due to peripheral hearing loss, language, cognition, or other related factors. Phonological awareness of, attention to, and memory of auditory information are reliant on an intact central auditory nervous system; however, these are considered to be higher order cognitive–linguistic skills and not included in the definition of (C)APD (Table 29.2). Objective neurological tests to diagnose (C)APD are in the experimental, research stages. The diagnosis is currently made through a battery of standardized behavioral audiometric tests administered by an audiologist. The purpose of the (C) APD evaluation is to evaluate the brainstem and cortical function of the central auditory nervous system by taxing the auditory system by reducing the external redundancy of the signal (Table 29.3). Even though verbal and nonverbal auditory stimuli are used on the tests, the effect of language, attention, and memory on several of the test tasks has been widely debated. Thorough speech and language and cognitive assessment outlining the patient’s skills in these areas aid in interpretation of audiometric results. If the audiological battery yields poor or inconsistent performance across all tests, higher order cognitive–linguistic, attentional, or motivational confounds should be suspected (Table 29.4). Table 29.2 Auditory skills assessed in (C)APD battery l

Sound localization: Identify from which direction the sound comes.

l

Discrimination: Recognize whether two sounds are the same or different.

l

Pitch Pattern Perception: Determine what sounds are high and low pitched.

l

Temporal Auditory Processing: Recognize breaks between sounds in words and words in sentences.

l

Dichotic Listening: Understanding speech when two competing signals are presented in each ear, simultaneously.

l

Auditory Figure Ground: Understand speech through background noise.

l

Recognized speech if the signal is degraded.

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Table 29.3 Criteria for (C)APD testing l

Normal to near normal peripheral hearing sensitivity

l

Normal to near normal cognitive functioning

l

Minimum age requirement – 7-years old

l

Sufficient receptive and expressive language skills

l

Fairly intelligible articulation

Table 29.4 Possible differential diagnosis l

Dyslexia/reading disability

l

Attention deficit disorder

l

Autism spectrum disorder

l

Developmental delay/cognitive disability

l

Speech and language impairment

l

Learning disability (language based and nonverbal)

l

Mood disorder

The (C)APD battery is recognized for its sensitivity in identifying that a problem exists, but it has been criticized for lacking specificity in providing a differential diagnosis for children with similar symptoms that may be better defined by another diagnostic label. The ASHA position statement (2005) concluded that ‘‘any definition of (C)APD that would require complete modality specificity as a diagnostic criterion is neurophysiologically untenable.’’ However, the perceptual deficit is expected to be most pronounced on auditory tasks vs. other modalities (ASHA, 2005, page 2). Returning to Michelle and her diagnostic experiences, she was referred for an IEP evaluation at age 4 as a result of her poor performance on the kindergarten screening. Results identified mild to moderate delays in receptive and expressive language skills for which she received speech and language therapy at school (Tables 29.5 and 29.6). Her parents also pursued private occupational therapy and physical therapy for her mild motor deficits and lack of physical endurance. She received those services for 1 year. Despite her progress, her mom and dad were still concerned about Michelle’s social development and pursued a pediatric neuropsychological evaluation at a major medical center to rule out Asperger’s Syndrome.

Test Results, Formulation, and Recommendations Psychometric testing identified average verbal and nonverbal skills. Inattentiveness, hyperactivity, and impulsivity were indicated. Although eye contact was reduced, Michelle’s social interaction with her parents and the examiner were

29 Elusive, Inclusive, or Conclusive? (Central) Auditory Processing Disorder Table 29.5 Intellectual – evaluation at ages 4–5 years Differential Abilities Scale Scorea Verbal cluster 83 Nonverbal cluster 81 General cognitive ability 82 Verbal comprehension [39] Naming vocabulary [41] Early number concepts [44] Picture similarities [36] Pattern construction [43] Copying [43] a Standard score, (scaled score), [T-score], {z-score}.

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Percentile 13th 10th 12th

appropriate. No odd or unusual social behaviors were observed or reported. Asperger’s Syndrome was ruled out. An Attention Deficit Hyperactivity Disorder (ADHD) was suspected. Parent training and classroom accommodations were recommended. Michelle’s parents felt that her attending difficulties were not due to ADHD but somehow related to lack of energy and reduced muscle tone. Michelle preferred quiet activities she could do on her own, such as drawing and reading. She was more socially engaging in one-on-one interactions. Her parents noticed that her attention difficulties were most evident in large group situations (i.e., classroom or play groups). Michelle began demonstrating signs of anxiety, often ruminating over others’ feelings or problems they had. Persistent concerns over Michelle’s well-being prompted her parents to pursue a neuropsychological reevaluation by the same professional who had seen Michelle 1 year earlier. Results from that reevaluation indicated that Michelle’s overall cognitive abilities were in the low average range (Tables 29.7 and 29.8). Nonverbal skills were reported to be solidly average, with verbal falling at the low end of average. Assessment of her motor skills indicated good motor planning abilities with slower processing speed. Performance on verbal tasks revealed difficulties with naming items and following multistep directions. Assessment of executive functioning indicated slow motor speed and impulsivity despite adequate motor planning and attention. The neuropsychologist felt that Michelle’s difficulties continued to be consistent with mild ADHD. Her impulsivity, pervasive thinking about the concerns of others, low motivation, and immaturity were related to regulatory difficulties associated with ADHD rather than an internalizing disorder. Although the neuropsychologist recognized the progress Michelle was making with her cognitive and behavioral skills, it was recommended that her family follow up with a therapist or attend a parent training group to help manage her behavioral difficulties as they arose. A trial course of stimulant medication was also recommended. These recommendations were not pursued, and in the middle of first grade, Michelle underwent a triennial IEP reevaluation (Tables 29.9 and 29.10). Psychological assessment by the school psychologist indicated that Michelle’s overall cognitive skills ranged from average to high average. Hyperactivity, impulsivity, and inattentiveness were again reported. There were also concerns about her

312 Table 29.6 Other cognitive functions – evaluation at ages 4–5 years Tests Raw Score score NEPSY Visual attention (11) Narrative memory (6) Imitating hand position (10) Phonological processing (11) Statue (6) Visuomotor precision (11) Train Time 32 Errors Car Time 25 Errors Visual Attention Bunnies Time 79 Omissions Commissions Cats Time 83 Omissions Commissions Imitating Hand Positions Preferred hand Nonpreferred hand Purdue Pegboard Preferred hand 79 Nonpreferred hand 68 Both hands 87 Vineland Adaptive Behavior Scales Communication 92 Receptive Expressive Written Daily living skills 80 Personal Domestic Community Socialization 89 Interpersonal Play and leisure Coping skills Motor skills 65 Gross motor Fine motor Adaptive behavior composite 76

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Percentile

Age equivalent

26th–75th

11th–25th

26th–75th 26th–75th

11th–25th 11th–25th 26th–75th 26th–75th

30th

30th

23rd

1st

5th

3–11 3–11 3–3 5–3 3–3 3–1 3–3 3–8 3–8 4–10 2–8 3–7 2–10 2–4 3–6 3–5 (continued)

29 Elusive, Inclusive, or Conclusive? (Central) Auditory Processing Disorder Table 29.6 (continued) Tests Clinical Evaluation of Language Fundamentals – Preschool Receptive language score Expressive language score Total language score Linguistic concepts Basic concepts Sentence structure Recalling sentences Formulating sentences Word structure

Raw score

313

Score

Percentile

89

23rd

96 92 (3) (9) (11) (7) (11) (10) 89

39th 30th

Table 29.7 Intellectual – evaluation at ages 5–7 years Differential Abilities Scale Score Verbal cluster 82 Nonverbal cluster 93 General cognitive ability 88 Verbal comprehension [58] Naming vocabulary [58] Early number concepts [51] Picture similarities [45] Pattern construction [46] Copying [41]

Age equivalent

23rd

Percentile 12th 32nd 21st

nervousness and worrying. Academic skills were average to above average. Speech and language testing indicated age-level skills, and she was discharged from speech and language therapy. In light of her average cognitive abilities and grade-level scores on achievement testing, Michelle’s IEP was discontinued, despite the attending and psychosocial issues. A student plan was implemented to provide her teachers with classroom modifications and accommodations (e.g., use of organization checklists, additional time for tests in small group settings). Michelle’s family moved when she was in second grade. Her student plan followed her to the new school in a different district. Despite grade-level academic skills, her teachers began to raise familiar concerns: Michelle had difficulty working independently in the classroom. She had problems following directions, and needed extra time to complete work. She often needed redirection, reminders, and visual aids such as written lists to help her remember steps to a task. One teacher reported that Michelle was exhibiting ‘‘stress-related’’ behaviors, such as fidgeting, short breaths when asking questions, and restarts and revision for both verbal and physical activities. She continued to have difficulty paying attention, following verbal instructions,

314 Table 29.8 Other cognitive functions – evaluation at ages 5–7 years Tests Raw score Score NEPSY Visual attention (5) Imitating hand position (10) Tower (10) Finger tapping (5) Comprehension of instructions (8) Visual precision (6) Car Time 19 Errors Motorcycle Time 44 Errors Visual Attention Cats Time 111 Omissions Commissions Faces Time 180 Omissions Commissions Imitating Hand Positions Preferred hand Nonpreferred hand Finger Tapping Test Repetitions 11–25% Sequences 11–25% Preferred hand 11–25% Nonpreferred hand 11–25% Luria’s Tapping Test Part I correct 16 Part II correct 12 Total correct 28 Delayed Alternation Task Trials 20 Correct responses 16 Correct alternations 12 Correct consecutive alternations 6 Consecutive perseverative errors 1

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Percentile

11th–25th

3rd–10th

11th–25th 26th–75th

3rd–10th 3rd–10th 26th–75th 26th–75th 11th–25th 11th–25th 11th–25th 11th–25th

remembering what people said, and understanding new ideas. Repetition was often needed before she would respond or understand new information. Verbal expression was becoming more difficult for her. Michelle had difficulty finding the right word to say, putting events in the right order when telling stories or relaying an

29 Elusive, Inclusive, or Conclusive? (Central) Auditory Processing Disorder Table 29.9 Intellectual – evaluation at age 7 years and 3 months Woodcock Johnson III Tests of Cognitive Abilities Score General intellectual ability (standard) 109 Verbal ability 108 Thinking ability 119 Cognitive efficiency 98 Processing speed 96 Short-term memory 101 Verbal comprehension 108 Visual-audio learning 104 Spatial relations 104 Sound blending 139 Concept formation 107 Visual matching 98 Numbers reversed 94 Decision speed 94 Memory for words 109

Percentile 72nd 71st 89th 45th 39th 53rd 71st 62nd 66th 99.5th 67th 44th 34th 35th 73rd

315

Age equivalent 7–10 8–2 9–6 7–1 6–11 7–4 8–2 7–9 8–5 8–0 7–1 6–6 6–8 9–0

Table 29.10 Academic achievement – evaluation at age 7 years and 3 months Tests Score Percentile Test of Early Reading Ability – Third Edition 117 87th Test of Early Written Language – Second Edition 135 99th Test of Early Mathematics Ability – Second Edition 93 32nd

event, expanding on an answer or providing details. Although her reading comprehension skills were reported to be good, she had difficulty explaining what she had read. She often got frustrated over her inability to effectively express herself. Interestingly, her written expression skills were reported to be good in comparison to her verbal expression. Michelle continued to work with her tutor during the summer between second and third grade. During this time, Michelle was referred to a speech and hearing clinic. This center provides multidisciplinary assessments for children with school performance and/or language-based learning difficulties. The team is composed of pediatric speech-language pathologists, audiologists, and child psychologists. The majority of referrals are to rule out a (C)APD. Review and careful consideration of the child’s history, parent and teacher reports, clinical observations, and evaluation findings should result in a differential diagnosis that will determine the most effective intervention. Collaboration among the professionals occurs at the time of intake, when the child’s records (such as previous evaluations, IEPs, report cards, parent and teacher checklists) are reviewed. The child is triaged to the appropriate professional(s) for additional evaluations, based on how current and complete the information is. The team reconvenes after the child has been assessed to review findings and make recommendations that are then shared with the family. This particular center has found that 5–10% of children referred for (C)APD actually

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met criteria for the diagnosis. The other more common diagnoses include specific language impairment, attention deficit disorder, anxiety disorder, low cognitive abilities, second language learners, and/or reading disabilities. After reviewing Michelle’s records, the team determined that her (C)APD evaluation would consist of speech and language testing and audiological assessment. Because extensive cognitive assessment had been done in the past, further psychological assessment would be deferred, pending the speech, language, and audiological findings. Michelle’s language evaluation took place just prior to the start of her third grade year. She impressed the examiner with her friendly demeanor and ability to self-correct her errors. Her eye contact was appropriate, and she was quite chatty. Michelle demonstrated good effort throughout the 2½-hour session, even for tasks that she said were hard for her. Although her sitting and on-task behavior were generally good, Michelle was observed to literally get out of her seat, kneel on the chair, and lean across the table for tasks that required confrontation naming or language formulation, suggesting some level of stress. The evaluation focused on assessing Michelle’s understanding and use of vocabulary, linguistic concepts, sentence structure, grammar, auditory memory, and conversational and other pragmatic language skills. Time constraints did not allow for assessment of her written language abilities; however, school records indicated grade-level reading decoding and comprehension, spelling, and written expression (Table 29.11). Michelle’s overall language scores were in the solidly average range with no significant difference between her receptive and expressive language scores. However, there was noticeable variability in her subtest performance. Her strongest scores were on those subtests that allowed for repetition of the verbal stimuli and/or provided visual cues such as words or pictures. She frequently corrected herself. This suggested a certain level of awareness of her error responses and perhaps a need for extra time to respond. Michelle scored in the low average range on the subtest that required her to listen to short narratives (no picture cues or repetition allowed) and answer questions about the passage. She had the most errors on questions that required her to recall specific details vs. responding to questions that required her to make inferences on the basis of common sense or prior knowledge. Michelle demonstrated increased motor restlessness and ‘‘out of seat’’ behavior for most of the expressive language subtests. When asked to formulate sentences about a pictured scenario using a specific target word in her sentence, she demonstrated speech dysfluencies in the form of part word repetitions, phrase revisions, and short inhalations before initiating her response. Despite these behaviors (which suggest some degree of stress) her ultimate sentences were well-formulated with good word choices. Michelle did poorly on a sentence repetition task (which is considered an expressive task on this test). However, sentence repetition also requires short-term auditory memory. No pictures were provided, and the examiner was not allowed to repeat orally presented stimuli. It was interesting to note that she did not demonstrate

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Table 29.11 Other cognitive functions – evaluation at age 7 years and 3 months Tests Score Percentile Age equivalent Test of Language Development – Primary – Third Edition Spoken language 96 39th Listening 100 50th Organizing 97 42nd Speaking 94 35th Semantics 94 35th Syntax 100 50th Expressive Language Test Sequencing 105 59th Defining 95 43rd Generating examples 120 91st Grammar/syntax 113 80th Concepts 90 26th Identifying categories 123 93rd Defining categories 96 44th Generating examples 124 95th Total test 113 79th Peabody Picture Vocabulary Test – Third Edition 106 66th 9–8 Expressive Vocabulary Test 101 79th 10–8 Clinical Evaluation of Language Fundamentals – Fourth Edition Concepts and following directions (10) 50th Word structure (8) 25th Recalling sentences (8) 25th Formulated sentences (15) 95th Word Classes Receptive (10) 50th Expressive (6) 9th Total (8) 25th Sentence structure (13) 84th Understanding spoken paragraphs (8) 25th Number Repetition Forward (11) 63rd Backward (10) 50th Core language composite 100 50th Receptive language composite 105 63rd Expressive language composite 101 53rd Language structure composite 106 66th

speech dysfluencies for the repetition task. On the subtest that assesses acquisition of English morphological rules in a sentence-completion task (picture cues and repetition allowed), she exhibited incorrect use of irregular plural nouns and past tense verb forms, which can be indicative of word retrieval difficulties. The irregular word form must be retrieved as a novel word, not following the expected morphologic rules. On a subtest of verbal fluency for which she had to state as many

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items as she could per category in 1 min, Michelle passed criteria. Although motor restlessness was again evident for this timed task, her responses were precise and organized (i.e., named classes of animals). Informal assessment revealed Michelle’s difficulty with verbal organization of her thoughts and ideas during discourse. Word retrieval difficulties were frequent, as evidenced by her word revisions and hesitations. She also tended to offer too much information (i.e., unrelated details). Verbal mazing included phrase revisions and fragmented utterances, which resulted in breakdowns in fluency, making it more difficult to follow her train of thought. Despite these discourse problems, her other pragmatic skills of turn taking, appropriate use of eye contact, requesting information, responding, predicting, and using repair strategies were judged to be appropriate for her age. Michelle demonstrated good articulation skills with the exception of a slight frontal distortion of /s-z/. Intelligibility was good. Occasional paraphasias were observed when she substituted a similar sounding word for an intended word (i.e., ‘‘thirst graders’’–she does not have a /th/f/ substitution pattern). In summary, Michelle once again demonstrated ‘‘average’’ receptive and expressive vocabulary and language skills on formal assessment measures. However, qualitative differences in her ability to use these skills were evident across various test tasks, resulting in ‘‘functional’’ language difficulties. Her ability to comprehend spoken language broke down as the length and complexity of the spoken message increased. She demonstrated significant difficulties with word retrieval and language formulation especially when asked to respond with specific information or verbalize more abstract concepts. These difficulties would be expected to impact her academic and social performance. A higher order language disorder was considered as a possible diagnosis. Previous psychological assessment indicated slow processing speed, impulsivity, and inattentiveness. It was felt that the slower processing speed could contribute to her inability to use her average language skills in an effective and efficient manner. Furthermore, her previous diagnosis of ADHD needed to be considered as another variable negatively impacting Michelle’s language skills. The question arose, Could all of this be related to an underlying higher order language disorder and/or comorbid ADHD? Perhaps, but children with Attention Deficit Disorders (with and without language impairments) often do poorly on the Concepts and Following Directions, Sentence Formulation, and Word Association subtests on the CELF-4. For Michelle, these particular tasks were relative strengths despite her increased fidgetiness during the tasks. Michelle was also observed to correct herself when she responded in error. This self-monitoring is often absent in children with ADHD. On the basis of her need for repetition of auditory stimuli, improved performance on tasks that had visual cues, and difficulty with language formulation in discourse, further assessment of her Central Auditory Processing skills was recommended before her diagnostic picture could be completed. She was seen for subsequent audiological assessment 2 months later because of scheduling conflicts.

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The audiologist’s preliminary assessment indicated that Michelle’s peripheral hearing and middle ear function were within normal limits, bilaterally. Speech recognition in quiet was excellent for both ears. Acoustic reflexes were also within normal limits. A different picture began to emerge as Michelle continued with the Central Auditory Processing test battery (Table 29.12). The generally accepted criteria for a (C)APD diagnosis is for a patient to score 2 standard deviations below the mean on at least two different test measures. Michelle did so on the Auditory Figure Ground Subtest and Time Compressed Words subtests on the SCAN. In addition, she scored in the borderline range on the Goldman-Fristoe-Woodcock Test of Auditory Discrimination. A diagnosis of (C) APD was given. The results from the audiology testing indicated that Michelle had difficulty listening in noisy environments when speech was degraded. Her profile fit the kinds of difficulties she had been experiencing over the years. (i.e., listening in large group situations due to increased background noise and reverberation of sounds). Listening would become even more difficult the further away she was from the speaker. When Michelle was unfamiliar with the vocabulary or given few visual or no contextual cues, she would be expected to have difficulty interpreting the message. The amount of concentration she needed to expend could result in fatigue or inattentiveness when listening. This fits with what her parents and teachers had been saying since preschool. Table 29.12 (Central) Auditory processing test battery and results Tests Purpose Findings Dichotic digits test Binaural temporal Right ear: 92% integration Left ear: 100% Word discrimination Quiet: 63% Goldman-Fristoe-Woodcock of similar sounding test of auditory discrimination Noise: 30%

Pitch pattern sequence

Random gap detection test

SCAN-C: Test for auditory processing disorders in children – revised (auditory-figure ground subtest) Time compressed words

Temporal ordering, pitch perception, and pattern recognition Temporal processing and temporal resolution Understanding speech in the presence of background multitalker noise Understanding speech when the signal is degraded

Right ear: 100% Left ear 100%

Interpretation Within normal limits (WNL) Quiet subtest: WNL Noise subtest: ‘‘borderline normal’’ range WNL

Random gap detection: 1.0 ms Standard score: 3 Percentile rank: 1%

WNL

Left ear: 80% at 30% compressed Left ear: 80% at 30% compressed

2 standard deviations below the mean in each ear

2 standard deviations below the mean for age

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Review of previous evaluations and current language and audiological findings indicated that Michelle had a (C)APD that was a major contributing factor to her academic, psychosocial, and attentional problems. Out of all the formal assessments she had undergone, the (C)APD battery yielded the most consistent findings. Although a subclinical ADHD and/or an anxiety disorder could not be ruled out as comorbid conditions, Michelle’s audiological profile could result in behaviors associated with these diagnoses. It also offered a plausible explanation for the language difficulties she exhibited. Reading comprehension was good; her listening comprehension was not but improved with repetition and visual support. Written expression was also good, but verbal expression was weak because of problems with word finding and formulation. These difficulties often reflect gaps in listening comprehension that make it difficult to get the gestalt of the message and apply higher order thinking skills. It also could contribute to trouble recalling salient details or use of specific words. To better illustrate Michelle’s struggles, here is an analogy of listening to a speaker with a strong foreign accent who is giving a lecture on a scientific topic that the listener knows little about. The lecturer has good command of English, but because the speaker’s speech is degraded by the accent, it takes more concentration on the listener’s part to understand what is being said. If it is a large lecture room, the distance between the speaker and the listener also takes away from the acoustic intensity of the speech signal. The distance makes it difficult to read facial cues to supplement the auditory message. The listener’s attention begins to wane after a while. It is even harder to understand the subject matter because the speaker is using vocabulary words related to the topic, of which the listener has little knowledge. The listener wishes that the lecturer could speak slower and/or repeat what was said, provide a written outline, give definitions, or at least provide a word list of the new vocabulary. That would make the listening and understanding a little easier. If listening comprehension is improved, recalling and retelling the novel information would be more efficient. Intervention for (C)APD should be implemented as soon as the diagnosis is made and should be comprehensive (Table 29.13). Per ASHA, ‘‘The accumulated auditory and cognitive neuroscience literature supports comprehensive programming, incorporating both bottom-up (e.g., acoustic signal enhancement, auditory training) and top-down (i.e., metacognitive and language) strategies’’ (2005, p. 11). However, management for (C)APD is not ‘‘one size fits all,’’ and intervention should match the individual’s strengths and needs. Some audiologists in the field use classifications Table 29.13 Interventions for (C)APD l

Perceptual training

l

Linguistic and cognitive strategies

l

Compensatory strategies

l

Environmental modifications

l

Assistive technology alternatives

l

Educational programming

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systems to profile the type of (C)APD an individual has. The team that evaluated Michelle did not. Rather, the team described the overall cognitive, emotional, linguistic, and auditory profile to prescribe appropriate management. Team recommendations were for Michelle to use an FM system in the classroom to give her a clearer auditory signal by increasing the signal to noise ratio and decreasing the detrimental effects of background noise on the signal. Classroom and environmental modifications were also recommended (Table 29.14), including improving classroom acoustics by using area rugs and wall hangings to reduce sound reverberations and using slit tennis balls on the bottom of chairs to help minimize the annoying and competing sound of furniture scraping on the tile floor. Preferential seating should be given so that Michelle is away from extraneous noise from the hallway, outside windows, or noisy heating and cooling systems and close to the teacher, but not so close that she misses out on classmates’ comments. Speech and language therapy was also recommended for auditory training in auditory closure, discrimination, and memory and in establishing strategies to improve active listening, self-cueing for word retrieval, and improving narrative discourse skills.

Update As a result of Michelle’s (C)APD test findings and recommendations, she underwent an IEP reevaluation with her school district to determine eligibility for special education support. During the school year, her teacher continued to express concern over her lack of focus in the classroom, difficulty following verbal directions, staying on task, and working independently. IEP testing was completed in Speech and Language Impairment, Learning Disabilities, Hearing Impaired, and Other Health Impaired (OHI). Psychological and educational testing ruled out a Learning Disability. Her cognitive and achievement scores were similar to previous Table 29.14 Instructional modifications l

Signal or cue to student to pay attention

l

Give information in short and concise steps

l

Small group or individual instruction time

l

Use of manipulatives, visual information, and written information (e.g., graphic organizers) to supplement auditory information

l

Preteaching of new concepts

l

Frequent comprehension checks

l

Extended time for tasks

l

Peer note taker

l

Give listening breaks

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findings. The school speech-language pathologist administered additional expressive language testing but found that Michelle scored within the average range on her narrative skills and verbal problem-solving abilities for the structured standardized testing. Therefore, she did not meet criteria for direct services under Speech and Language Impairment. The IEP team came to a unanimous agreement that Michelle needed direct support at school vs. accommodations through a student plan. An IEP was implemented under Other Health Impaired because her (C)APD was a chronic medical problem that resulted in limited strength and vitality during the school day. In addition, she met criteria for the Hearing Impaired program, which would allow the use of the FM system. Consultation by the speech-language pathologist would be provided as a related service. Despite recognition of Michelle’s struggles, it took nearly 5 years to formulate her much-needed IEP. A definitive diagnosis evaded numerous professionals, yet all appreciated the functional difficulties she was experiencing. Perhaps it can be argued that her parents kept shopping around until they received a diagnosis they could accept. In this case, the parents’ persistence led to their child finally getting the kind of support she needed at school. While terms such as auditory processing, language processing, cognitive processing, and informational processing disorders are frequently used in the educational arena, it may be a case of ‘‘the diagnosis is in the eye of the beholder.’’ Because of the overlapping symptomology of (C)APD and ADHD, the debate among psychologists, neuropsychologists, audiologists, and speech-language pathologists continues. Nonetheless, it cannot be argued that a ‘‘processing disorder’’ of any kind has a negative effect on learning. Thorough review of history, classroom and clinical observations, as well as comprehensive assessment are all needed to provide a differential diagnosis that fits the child’s strengths and weaknesses. In Michelle’s state, a diagnosis of a (C)APD does not qualify a child for any particular special education service. Yet in her case it better defined her profile, guiding professionals to provide appropriate support and intervention.

Additional Resources Key Concepts and Terms Acoustic Reflexes Measurement of the reflexes generated by the middle ear muscles in response to intense sound. Central Auditory Nervous System Brainstem and cortical structures including the eighth auditory nerve, cochlear nucleus, superior olivary complex of the pons or reticular formation, inferior colliculus, medial geniculate body up to Heschl’s gyrus in the left temporal lobe of the cortex. (Central) Auditory Processing Disorder Difficulty in processing auditory information in the central nervous system.

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Discourse Conversational interaction and/or ability to understand and construct monologues such as narratives, procedural texts, expository texts, persuasive speeches, descriptions and others. FM System A device that transmits acoustic signals by radio waves from the sound source directly to a receiver worn by the listener. Paraphasia An incorrect word is substituted for an intended or target word. Peripheral Hearing System Physiological function of the outer ear, ear canal, middle ear, and cochlea, which results in hearing acuity. Pragmatics Social language use. Signal to Noise Ratio A measure of signal strength relative to background noise. Specific Language Impairment A developmental language disorder in the absence of neurological, cognitive, sensory or social emotional deficits.

References Resources for Clinicians American Speech-Language-Hearing Association. (2005). (Central) Auditory Processing Disorders [Technical report]. Available at http://www.asha.org/members/deskref-journls/deskref/default. American Speech-Language-Hearing Association. (2005). (Central Auditory Processing Disorders— the Role of the Audiologist [Position statement]. Available at http://www.asha.org/members/ deskref-journals/deskref/default. Bellis, T. J. (2003). Assessment and management of central auditory processing disorders in the educational setting: from science to practice (2nd Ed.). Clifton Park, NY: Delmar Learning. Katz, J., & Tillery, K. L. (2005). Can Central Auditory Processing Tests Resist Supramodal Influences? American Journal of Audiology, 14, 124–127. Masters, M., Stecker, N., & Katz, J. (1998) Central auditory processing disorders: Mostly management. Boston: Allyn and Bacon. Richards, G. J. (2001). The source for processing disorders. East Moline, IL: LinguiSystems. Rosenberg, G. (2002). Classroom acoustics and personal FM technology in management of auditory processing disorders. Seminars in Hearing, 23(4), 309–318.

Resources for Families American Academy of Audiology. 11730 Plaza America Drive, Suite 3000. Reston, VA 20190. Toll Free: (800) 222–2336 at http://www.audiology.org American Speech-Language-Hearing Association. 10801 Rockville Pike, Rockville, MD 20852. Toll free: (800) 638–8244 at http://www.asha.org National Institute of Deafness and Other Communication Disorders at http://www.nidcd.nih.gov/ health/voice/auditory

Chapter 30

A Little of This, A Little of That: (Central) Auditory Processing Disorder Lisa R. Cox

Victoria was unusually quiet as an infant, seldom cooing or babbling. She spoke her first words a bit late at 18 months and began combining two to three words at age 2½ years. Her speech was very difficult to understand, but her parents assumed that her pronunciation would improve with age. An evaluation conducted by the local school district to determine school readiness indicated that Victoria had profound delays in articulation and phonological skills. She was subsequently enrolled in a phonology program at age 4 years. The program was minimally effective; her speech remained 80–85% unintelligible to everyone except her immediate family. In kindergarten, Victoria struggled to learn letter sounds and seemed to have more difficulty than her peers with following directions and answering questions. Her classmates understood very little of what she said and constantly teased her because she sounded ‘‘like a baby.’’ Both Victoria’s parents and teacher noticed that she seemed sad and withdrawn. Victoria’s speech was often monotone and she had a tendency to stress the wrong syllables of words. Her parents began to wonder about a hearing impairment. Shortly after her sixth birthday, Victoria was evaluated by an audiologist. Her peripheral and middle ear function were normal in both ears. However, further testing suggested that she had difficulty discriminating the subtle differences between sounds in words, especially in noisy environments. She also had difficulty understanding the meaning of words and phrases. Victoria was subsequently diagnosed with (central) auditory processing disorder ((C)APD). The audiologist provided detailed recommendations including the use of an FM system and referred Victoria to the school Individualized Education Program (IEP) team for an evaluation. The IEP team concluded that Victoria met eligibility criteria to receive special education services under the speech and language impairment classification. She began using an FM system and received pull-out speech and language therapy during the fourth quarter of her kindergarten year. For first grade, Victoria was transferred to an ‘‘open-concept design’’ or ‘‘classroom without walls’’ elementary school. At her new school, she continued to use the FM system, received pull-out services for speech and language therapy, and was

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enrolled in a specialized reading program. She made several new friends and her self-esteem, mood, and grades initially improved. However, by the middle of second grade, Victoria was distractible, had poor organizational skills, and her grades began to drop. At home, Victoria’s parents described her as always on the go, easily distracted, and somewhat impulsive. Homework was completed only after prolonged tantrums and/or direct supervision from her mother. At the time of referral in third grade, Victoria had significant difficulty keeping her school materials organized in her desk and backpack; she frequently lost and/or forgot to turn in assignments. Her parents described her speech as intelligible approximately 50% of the time; the previous year they were able to understand 90% of her speech. Her classmates teased her almost nonstop about her speech problems and often excluded her from activities. At recess, she typically played with second graders. She was embarrassed by the size of her FM system and, not surprisingly, had some compliance problems related to its use. A behavior management plan was implemented during the second quarter of third grade, since which time she used the system appropriately. Although Victoria was a generally happy child, her parents were very concerned about her persistent underlying sadness.

Test Results An evaluation conducted by the school district at the beginning of third grade indicated low average intellectual abilities with relative weaknesses in verbal skills and the ability to briefly hold and manipulate verbal information in mind (Table 30.1). Academic achievement testing indicated average mathematical and oral language skills, low average written language skills, and well below average Table 30.1 Intellectual Wechsler Intelligence Scale for Children (Fourth Edition) Full scale IQ Verbal comprehension index Similarities Vocabulary Comprehension Perceptual reasoning index Block design Picture concepts Matrix reasoning Working memory index Digit span Letter–number sequencing Processing speed index Coding Symbol search a Standard score, (scaled score), [T score], and {z score}.

Scorea 84 81 (9) (5) (6) 98 (10) (10) (9) 77 (7) (5) 94 (9) (9)

Percentile 14th 10th

45th

6th

34th

30 A Little of This, A Little of That: (Central) Auditory Processing Disorder Table 30.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition Broad reading Letter–word identification Passage comprehension Reading fluency Basic reading skills Letter–word identification Word attack Broad math Calculation Applied problems Math fluency Broad written language Spelling Writing samples Writing fluency Oral language Story recall Understanding directions Picture vocabulary Oral comprehension Story recall delayed Academic skills Academic fluency Academic applications Academic knowledge

Score 72 81 81 65 79 81 79 93 83 105 85 83 82 97 84 97 120 106 85 99 98 81 72 93 92

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Percentile 3rd 10th 11th 1st 8th 10th 8th 33rd 13th 63rd 15th 13th 12th 43rd 14th 43rd 91st 65th 15th 46th 44th 11th 3rd 33rd 29th

reading skills (Table 30.2). Throughout the testing at school, Victoria was fidgety and easily distracted by outside noise (e.g., children going out for recess). Reading appeared the most difficult for her. She seemed to rush through some of the reading passages but did not give up easily. Concerns about Victoria’s ongoing difficulties prompted her parents to request a neuropsychological evaluation to assess several other important cognitive domains that may have been contributing to her academic difficulties (Table 30.3). During this evaluation, Victoria was friendly but reserved. Her interests seemed age appropriate, though her conversation skills were somewhat below age expectations. Her speech was monotone and her articulation was at times very difficult to understand. She was inattentive, fidgety, and had difficulty with impulse control (e.g., she frequently attempted to touch testing stimuli at inappropriate times). When items became challenging for her, she appeared to become somewhat upset and complained of aches in her stomach; however, with encouragement from the examiner and her parents, Victoria proceeded with testing with adequate effort and cooperation. Victoria’s ability to sustain attention and inhibit impulsive responding during structured tasks was intact. She had significant difficulty and frequently violated the rules on a novel problem-solving task. Her ability to understand concrete, single words was in the average range. In contrast, her overall language ability was in the

328 Table 30.3 Other functions: evaluation at age 9–4 Tests Score Gordon Diagnostic System Vigilance Task (9 min) Correct 106 Commission errors 104 The Tower of London 65 Clinical Evaluation of Language Fundamentals – Fourth Edition Receptive language 81 Concepts and following directions (5) Word classes – receptive (8) Expressive language 83 Recalling sentences (5) Formulated sentences (6) Word classes – expressive (9) Language memory 74 Total language 78 Word classes – total (8) Peabody Picture Vocabulary Test – 92 Third Edition, A Comprehensive Test of Phonological Processing Phonological awareness 85 Elision (7) Blending words (8) Phonological memory 79 Memory for digits (6) Nonword repetition (7) Rapid naming 88 Rapid digit naming (8) Rapid letter naming (8) Attention Deficit Hyperactivity Disorder Rating Scale – Fourth Edition Home Version Total Hyperactivity–impulsivity Inattention School Version Total Hyperactivity–impulsivity Inattention Achenbach Child Behavior Checklist Parent Anxious/depressed [54] Withdrawn/depressed [60] Somatic complaints [68] Social problems [64] Thought problems [54] Attention problems [66]

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Percentile

66th 61st 1st

10th 5th 25th 13th 5th 9th 37th 4th 7th 25th 30th

16th 16th 25th 8th 9th 16th 21st 25th 25th

89th–90th 85th–88th 88th–89th 80th–84th 50th 88th

65th 84th 97th 92nd 65th 95th (continued)

30 A Little of This, A Little of That: (Central) Auditory Processing Disorder Table 30.3 (continued) Tests Score Rule-breaking behavior [63] Aggressive behavior [65] Teacher’s Report Form Anxious/depressed [51] Withdrawn/depressed [60] Somatic complaints [50] Social problems [62] Thought problems [57] Attention problems [67] Rule-breaking behavior [63] Aggressive behavior [61] Behavior Rating Inventory of Executive Function Behavioral regulation index [58] Inhibit scale [60] Shift scale [60] Emotional control scale [50] Metacognition index [61] Initiate scale [46] Working memory scale [62] Plan/organize scale [65] Organization of materials scale [66] Monitor scale [58] Global executive composite [59]

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Percentile 90th 93rd 54th 84th <50th 89th 76th 96th 90th 87th 77th 89th 56th 55th 83rd 44th 86th 91st 88th 80th 80th

well below average range with similarly developed receptive and expressive language skills. She had the most difficulty on tasks assessing her ability to repeat sentences and follow multiple-step commands. It is important to note that both of the latter tasks were particularly sensitive to variations in attention. Her phonological awareness (segmentation, deletion, and reintegration of orally presented words) and rapid naming (of visually presented digits and numbers) were low average, while her phonological memory (for orally presented nonwords or for series of orally presented digits) was well below average. Responses on parent and teacher questionnaires indicated some difficulties with sustaining attention. Responses on parent questionnaires indicated that Victoria experienced somatic symptoms (e.g., stomachache, headache) at a level slightly higher than her peers, had some difficulty with acting out behaviors, and significant difficulties with planning, problem solving, and organizing.

Formulation and Recommendations Victoria’s speech and language development had been atypical since infancy. Her persistent difficulty with articulation and poor intonation or prosody suggested developmental apraxia of speech and warranted further evaluation by a speech

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and language pathologist. Her language skills consistently lagged behind that of her peers, were significantly lower than her nonverbal intellectual abilities, and had a negative impact on her ability to acquire early reading skills. Victoria’s teachers and parents repeatedly expressed concerns about distractibility, poor impulse control, motor restlessness, and poor organizational skills. Her neuropsychological profile was notable for difficulties on tasks particularly sensitive to fluctuations in attention. While Victoria clearly exhibited some hyperactive, impulsive, and inattentive symptoms, the overlap between (central) auditory processing disorder and attention deficit hyperactivity disorder (ADHD) made it difficult to determine whether she met diagnostic criteria for one or both disorders. For this reason, a separate diagnosis of ADHD was deferred. It was felt likely, however, that deficits in attention and executive function contributed to her academic underachievement. From a neuropsychological perspective, Victoria showed global language impairments that went beyond auditory processing and attention deficits, and thus were more accurately characterized as a higher-order language disorder, specifically mixed receptive and expressive language disorder. From a psychosocial perspective, Victoria was a sensitive young girl who had experienced social interaction difficulties and low self-esteem as a result of her speech and language problems. She had a longstanding history of persistent underlying sadness and the amount of somatic complaints she reported was suggestive of increased stress. She was felt to be at increased risk for a mood and/or anxiety disorder. Due to Victoria’s weakness in comprehending and following through with directions, caution should be exercised to avoid giving her multiple-step commands. Most directions should be presented very clearly, with an opportunity for her to complete individual steps. When lengthier or more complex directions are necessary, she should be asked to repeat them. She also may benefit from direct instruction and practice in learning to better attend to and follow directions. Victoria’s auditory processing and listening comprehension may be addressed with explicit instruction in sequential, temporal, location, and conditional concepts (e.g., next to, between, before, after, under, first, if, then). The use of concrete, visual materials during these activities will help Victoria to both understand and remember the concepts. She should have continued instruction in receptive and expressive vocabulary and in adverb, adjective, and verb tense usage in oral language activities. Again, simultaneous visual and tactile processing during these activities will be beneficial. School staff can practice these language skills in oral language activities with Victoria, for instance, conversations; show-and-tell sessions; puppetry; dramatic play; telephoning; reporting; interviewing; telling stories, riddles, or jokes; and role-playing. Other possible oral language activities include ‘‘tell me how. . .’’ activities, helping her describe the attributes of objects, and finishing stories (i.e., beginning a story and letting her finish it). Work on traditional phonics skills should be emphasized in Victoria’s reading program. The most effective phonological development components in reading research at this time involve systematic review of basic phonics elements such as

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letter sounds when needed, practice in segmenting short words in print, blending methods such as orally stretching out the sounds in words, contextual application of analytic phonics methods such as word families, and an integration of reading and writing activities. Reading decoding work should focus on reading material that is at Victoria’s instructional level for word recognition, which was second grade according to testing at the school the previous fall. Victoria should also be taught reading strategies to enhance comprehension. Some examples include prereading questions at the end of chapters, previewing pictures in the story, and thinking in advance about what the story may be about. She should be cued to personalize the content when possible by thinking about her own knowledge of the types of characters, settings, or events described. Skimming major section headings before beginning to read would be helpful. She should stop periodically during reading to quiz herself on the main ideas and to attempt to abstract the concepts from the specific content of what she has read. Particularly with difficult or longer material, Victoria should make outlines to aid later study as well as conceptual reorganization of the material. She will need to be taught to apply different reading and comprehension strategies to different material (e.g., reading fiction for English vs. reading expository text for science) and to actively think about and utilize structures in text. For example, within narrative text, Victoria should learn that the story will contain a setting, one or more characters, some type of problem, events, and some solution. Within expository text, she can be prompted to look for a general topic, one or more main ideas, and some specific details about the main ideas. Comprehension can be facilitated by using story mapping strategies immediately following reading. She should monitor her reading speed and slow down when faced with more difficult or less familiar material. In everyday conversation, Victoria’s parents should encourage her to describe and detail stories for them, either of what she has read or what has happened to her during the day. They should make sure that these activities are fun and rewarding for Victoria, and they can make a game out of how many adjectives, adverbs, or relational concepts (e.g., if, then, next, before) she uses. They should also model these language skills for her by describing experiences or detailing stories back to her. Victoria may need more individualized assistance for a time to learn to implement particular strategies to guide her written language production. Ideally, this one-on-one assistance could be faded as Victoria becomes more independent, and reinforcement could be added for using the strategies with reduced cuing from adults. Written products will be enhanced by following a process of first brainstorming about the assigned topic, then jotting down ideas generated by this, beginning to sequence these ideas, adding details to the rough outline, and then proceeding to inserting verbs and transition words to form a prose product. This can most efficiently be done using a word processor, although it also could be completed by making a map or idea grid before writing. Next, the product should be revised, including presequencing if necessary. Only nearer to the end of the process should Victoria proofread spelling, capitalization, punctuation, and word usage

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(grammar) in the writing. Reading the composition aloud at this point also can help to illuminate errors in grammar and punctuation. Victoria needs further instruction in many aspects of written language, including spelling, sentence construction, paragraph structure, and organization of longer written products. It is often most helpful to have children focus on spelling words that are integrated with their reading curriculum and/or words that are grouped together in phonologically related word families. The use of a ‘‘word wall’’ would be helpful for Victoria. In classroom application, this consists of a list of words on the wall, grouped according to their beginning letters alphabetically, to which children can refer when doing written work. Children are expected to correctly spell all words on the word wall in their written work, and words are gradually added as they are taught in the curriculum. Easily confusable similar words (e.g., what, when) are printed on different colored paper and cut out with the outline shape closely following the configuration of the letters in the word. This type of system can be individualized with a chart on the child’s desk if the child is working at a lower level than the overall class or if this type of system is not usable in the whole class format. In the long run, the goal should be for Victoria to reliably spell a circumscribed list of commonly used words. The following may help Victoria improve her organization and planning: providing Victoria with clear places for necessary materials, such as color-coded subject folders, and reinforcing use of these organizers; breaking longer assignments or directions into parts, perhaps providing a checklist that Victoria can mark following completion of each step; providing guided practice for Victoria in planning how to complete assigned tasks (what is needed, how to break tasks into manageable parts, estimating time needed for each part); providing other organizational checklists for different needs, such as steps to get ready to go home after school; and reminding Victoria at the end of the day about what she needs for home and the next day. Victoria’s difficulties with tasks requiring abstract reasoning and logical problem solving suggest that she may have difficulty when faced with complex, novel tasks. Specifically, she may find it difficult to analyze the requirements of a task, thereby making it difficult to apply effective strategies toward a solution. Because of this, the acquisition of novel cognitive skills such as higher level academics may prove to be frustrating for Victoria. When faced with such novel situations, she should attempt to determine the requirements of the situation and subdivide them into a series of steps. Writing these down on paper is likely to be particularly helpful. She then will be in a better position to formulate a plan for approaching the new task. Victoria’s parents and teachers should closely monitor her symptoms of depression and anxiety. If her mood appears to be impacting her daily functioning, psychological counseling is strongly recommended. Taken together, Victoria’s case illustrates the diagnostic conundrum that (C) APD can present when it coexists in children with speech–language impairment, developmental disabilities, ADHD, learning disabilities, anxiety and/or mood

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disorder. While diagnostic clarity is important, the development of an appropriate education plan is paramount.

Additional Resources Key Concepts and Terms Attention. Includes engaging, sustaining, selecting, and disengaging attentional focus. Executive functioning. The abilities necessary to plan, organize, and control behaviors. Attention and executive functioning require similar skill sets and rely on overlapping neural circuitry. ADHD. Characterized by disturbances of age-appropriate impulse control, sustained attention, rule-governed or goal-directed behavior, activity level, consistency of performance and executive mental functions (e.g., planning, organization and adaptive shifting of mental set). Mixed receptive–expressive language disorder. Impairments in both the understanding and use of language.

References Resources for Clinicians American Speech–Language–Hearing Association. (2005). (Central) auditory processing disorders (Technical report). Available at http://www.asha.org/members/deskref-journls/deskref/ default. American Speech–Language–Hearing Association. (2005). (Central) auditory processing disorders – The role of the audiologist (Position statement). Available at http://www.asha.org/ members/deskref-journals/deskref/default. Bellis, T. J. (2003). Assessment and management of central auditory processing disorders in the educational setting: From science to practice (2nd ed.). Clifton Park, NY: Delmar Learning. Katz, J., & Tillery, K. L. (2005). Can central auditory processing tests resist supramodal influences? American Journal of Audiology, 14, 124–127. Masters, M., Stecker, N., & Katz, J. (1998). Central auditory processing disorders: Mostly management. Boston: Allyn and Bacon. Parthasarathy, T. K. (Ed.). (2006). An introduction to auditory processing disorders in children. Mahwah, NJ: Lawrence Erlbaum Associates. Richards, G. J. (2001). The source for processing disorders. East Moline, IL: LinguiSystems. Rosenberg, G. (2002). Classroom acoustics and personal FM technology in management of auditory processing disorders. Seminars in Hearing, 23(4), 309–318.

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Resources for Families American Academy of Audiology. 11730 Plaza America Drive, Suite 3000, Reston, VA 20190. Toll Free: (800) 222-2336 at www.audiology.org American Speech–Language–Hearing Association. 10801 Rockville Pike, Rockville, MD 20852. Toll free: (800) 638-8244 at www.asha.org National Institute of Deafness and Other Communication Disorders at www.nidcd.nih.gov/health/ voice/auditory NICHCY, National Dissemination Center for Children with Disabilities at www.nichcy.org

Chapter 31

A Hunt for the Elusive Neuropsychological Impairment: Conversion Disorder Michael J. Zaccariello

‘‘Some of Dean’s other doctors believe that psychological issues and our family situation is affecting his neurological problems. Are you in agreement, Doctor?’’ ‘‘In a word, yes.’’

Thus began the case of the elusive neuropsychological impairment. Approximately 2 years prior to his neuropsychological evaluation, Dean was jumped by a friend and hit several times in the head. He fell and reportedly hit the side of his head on a rock. According to his parents, there was evidence of blood on the rock. Dean’s recollection was consistent with his parents’ report. He could not recall what side of his head was hit. Upon falling, he got up, noticed blood on a rock and felt a lot of blood on his face. He was able to stumble home, and was subsequently taken to the local emergency room. Neuroimaging conducted at the hospital was unremarkable, and after several hours of observation Dean was discharged home. However, the next day, he reportedly lost his color vision and had significant fine motor difficulties to the point that he could not use utensils because his hand would shake so violently. In addition, Dean’s arms would spontaneously swing uncontrollably, knocking over plates and bowls. Magnetic resonance imaging (MRI) of the brain conducted about a week after this altercation showed no evidence of traumatic injury or intracranial hemorrhage. Prior to this event, Dean had no significant cognitive complaints, and he was considered an average student. Cognitively, since his injury, Dean was having extreme difficulty maintaining focus on school tasks and projects because of stamina issues. It was also Dean’s parents’ perception that his attention had become progressively worse. However, his parents noted that he had no problems focusing on video games for long periods of time. His memory was reportedly significantly poorer, and at times he was not oriented to time and place. His chronic fatigue was so debilitating that he could not attend school full-time. Consequently, he was taking courses through an Internet-based home school program. It was his father’s perception that Dean’s unorthodox schooling was affecting his overall academic achievement.

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Prior to his injury, Dean had multiple medical issues that hindered making sense of his current complaints. According to his father, approximately 2–3 days after birth Dean began to demonstrate rhythmic arm and leg twitching that was diagnosed as a seizure disorder. He was subsequently put on an antiepileptic medication, but he has not had a verifiable seizure since he was 3 months of age. He did undergo long-term EEG monitoring a year before neuropsychological testing, after an episode when he was unresponsive, but no clinical seizures were captured and a provisional diagnosis of nonepileptic seizures (commonly known as pseudoseizures) was made. When he was 11-years old, Dean began to complain of migraines characterized by bitemporal and frontal pain. These headaches increased in frequency and duration, resulting in Dean missing 6 months of school when he was in eighth grade. After this episode, his migraines decreased in frequency and intensity but then escalated to daily headaches since his injury. In terms of other medical history, Dean was diagnosed with hearing loss as a 12-year old. His hearing issues spontaneously resolved within the year, without any medical intervention. At the time of the assessment, Dean was taking amitriptyline and melatonin. He also carried a diagnosis of myofascial pain syndrome. Multiple work-ups through the neurology clinic, allergy clinic, and physical medicine and rehabilitation service found no specific cause for Dean’s chronic conditions. In Dean’s psychosocial history, his father reported numerous problems. Dean’s siblings had a myriad of mental health difficulties, learning disabilities, and suspected neurological disorders. His parents also had mental health issues, namely depression. However, Dean’s father did not think his son was struggling with depression or anxiety. There was a great deal of stress in the family because of financial strain and multiple failed businesses. They had no mode of transportation, and Dean’s parents and three siblings were living in a two-bedroom apartment. At the time Dean’s father requested the neuropsychological evaluation, both of Dean’s parents reported that he had diffuse and wide-ranging cognitive and academic difficulties. A broad approach was taken to ascertain his neuropsychological profile for two main reasons. First, in the absence of any significant previously identified neurological abnormality, it was important to screen all the major cognitive domains (e.g., intellectual functioning, attention, memory) to identify any potential subtle or underlying deficits not identified during multiple neurological and medical examinations. The second reason was more conceptual. Neuropsychologists often are called to ‘‘rule in’’ areas of cognitive weakness in patients, but sometimes (such as Dean’s case) what becomes equally important is ‘‘ruling out’’ cognitive dysfunction. The purpose of excluding dysfunction is to then hypothesize about other potential explanations for parent and patient complaints. The test protocol focused initially on intellectual functioning and academic achievement. Further testing addressed memory, attention and executive function, and screening of language and visual-spatial abilities. Given his reported fatigue, Dean was assessed over the course of two sessions.

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Test Results At his first appointment, Dean presented as a shy adolescent with minimal emotional expressiveness. He did not seem to be overtly depressed or anxious; rather, he was ‘‘flat.’’ Although he never initiated conversation, he readily engaged in spontaneous discussion about a myriad of topics. He had no overt fine motor difficulties, but he remained in a wheelchair throughout the testing session because he had great difficulty walking. In contrast, during his second appointment, Dean was able to walk to the testing area and did not complain of pain as he moved around. His father reported, ‘‘One day he just decided to get up and walk. He is also back to riding his bike.’’ Dean was also more conversational and emotionally expressive. In his approach to testing, there was no significant evidence of inattention, distractibility, motor restlessness, or impulsivity across both sessions. He was polite and his demeanor was pleasant, even on tasks where he had difficulty. He was cooperative and persistent for all tasks, and there was no evidence of waxing and waning of effort throughout the entire evaluation. In talking with Dean, he was not concerned at all about his reported cognitive decline since his fight. He did note that his cognitive issues made attending school difficult for him but that he felt he was making adequate progress in school. He did not report significant levels of depression or anxiety. Interestingly, and in stark contrast to his father’s report, he denied any chronic stress or difficulty at home. Dean’s overall intellectual ability was average with no significant discrepancy between his average intellectual indices (Table 31.1). A similar pattern was seen in Table 31.1 Intellectual Percentile Wechsler Adult Intelligence Scorea Scale – Third Edition Verbal comprehension 103 58th Information (11) Similarities (11) Vocabulary (10) Working memory 92 30th Letter-number sequencing (11) Arithmetic (8) Digit span (7) Full scale IQ 98 45th Perceptual organization 99 47th Picture completion (11) Block design (9) Matrix reasoning (10) Processing speed 93 32nd Coding (8) Symbol search (10) Verbal IQ 97 42nd Performance IQ 99 47th a Standard score, (scaled score), [T-score], {z-score}.

338 Table 31.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition Broad reading Letter–word identification Passage comprehension Reading fluency Basic reading skills Letter–word identification Word attack Broad math Calculation Applied problems Math fluency Broad written language Spelling Writing samples Writing fluency Academic skills Academic applications Academic fluency

M.J. Zaccariello

Score

Percentile

93 96 97 91 90 96 82 85 83 89 89 84 81 101 84 86 87 92

32nd 39th 43rd 27th 26th 39th 12th 16th 13th 23rd 23rd 14th 10th 53rd 15th 18th 19th 29th

terms of academic achievement. Reading was average. Mathematics and written language were relative weaknesses but still within broad normal limits (Table 31.2). His performance on other neuropsychological measures was generally consistent with that seen on measures of intelligence and academic achievement. Attention and executive function abilities were low average to solidly average. Language and visual-spatial screening was within normal limits. Memory testing was solidly average for immediate and delayed recall of paragraph-length stories and word lists. He demonstrated an extremely well-developed ability to organize and encode information to aid retention. Dean’s greatest weakness was in fine motor speed and dexterity, though his scores were still low average (Table 31.3). As previously mentioned, Dean did not seem to exaggerate his cognitive difficulties by purposefully performing poorly during testing. A commonly used quantitative measure of effort corroborated the clinical impression that Dean put forth adequate effort during the evaluation. Dean easily and quickly completed this specific task with no errors.

Formulation and Recommendations Results from the assessment indicated average global intellectual ability with generally commensurate academic skills development. Dean’s performance on measures of attention, executive function, language, visual-spatial, and memory functions was consistent with his intelligence and academic achievement. Notably, there was no evidence of significant impairment in any tested domain.

31 A Hunt for the Elusive Neuropsychological Impairment: Conversion Disorder Table 31.3 Other cognitive functions Tests Grooved Pegboard Test Right hand Left hand Trailmaking Test Part A Part B Wisconsin Card Sorting Test Categories Total errors Perseverative responses Perseverative errors Nonperseverative errors Conceptual level responses Failure to maintain set Learning to learn Boston Naming Test Benton Judgment of Line Orientation Test Wechsler Memory Scale – Third Edition Logical Memory I II California Verbal Learning Test – Second Edition Short-delay free recall Short-delay cued recall Long-delay free recall Long-delay cued recall Semantic clustering Delayed recognition

Score

Percentile

81 80

10th 10th

97 82

42nd 12th

91 96

>16th 25th 23rd 21st 30th 30th >16th >16th 27th 40th

(12) (11)

75th 63rd

{1} {1} {1} {0.5} {2.5} {0.5}

84th 84th 84th 69th >99.99th 31st

90 89 88 92

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Clinical neuropsychology and related medical disciplines lack a consensus about what constitutes a mild brain injury. Such entities as the American Academy of Pediatrics (1999) and the American Congress of Rehabilitation Medicine (1993) have opined their respective algorithm of what constitutes mild injury. Diagnostic systems such as the International Classification of Diseases (World Health Organization, 1992) also provide criteria. There exist points of overlap and distinction among these various groups and agencies that complicate clinicians’ ability to understand and diagnose mild head injury. Nevertheless, in reviewing criteria, it would appear that Dean’s brain injury was mild. He did not have a skull fracture, and two different forms of neuroimaging (each appropriately selected in light of time since injury) conducted on the day of and a week after his injury were normal. It was also thought that Dean’s loss of consciousness was less than 20 min. His rapid reacquisition of place orientation and lack of amnesia for events at the time (i.e., he was able to remember his home address and walked home; he reported aspects of the fight) provided further

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preliminary evidence of a mild traumatic brain injury. Finally, there was no deficit in his neuropsychological profile. Given the nature and extent of this injury, one would not predict or expect long-standing cognitive or academic difficulties or decline in these areas. Dean’s reported cognitive and academic difficulties were not thought to be related to postconcussive syndrome (PCS); rather, more complex and, to some extent preexisting, psychiatric issues appeared involved. PCS is a somewhat controversial syndrome characterized by impairment in neuropsychological functioning accompanied by at least three physical complaints that have persisted for at least 3 months subsequent to a reported closed head injury (American Psychiatric Association, 2000). Dean endorsed some features of the syndrome, namely fatigue, headache, and sleep difficulties. However, the constellation of symptoms associated with PCS generally persists for a matter of weeks or months and are not typically observed years after a mild head injury. In addition, there was no consistent and compelling evidence of cognitive impairment. Finally, and arguably most importantly, Dean’s quick remission of ambulation difficulties, across testing sessions, in the absence of any medical or neurological intervention is not indicative of systematic and chronic neurological pathology. A brief clinical interview with Dean revealed no compelling evidence of depression, anxiety, psychotic thought process, or posttraumatic stress. In contrast to his father’s report of chronic stress in the family concerning health and finances, Dean reported no pressing family concerns. He displayed a rather nonchalant attitude about his reported cognitive and medical complaints. The combination of his ‘‘unawareness’’ of family strife coupled with his relative lack of concern about his reported symptoms was reminiscent of la belle indifference. In the absence of other medical or neurological etiologies, the most parsimonious explanation of Dean’s presentation was thought to be related to psychological factors. On the basis of his presentation and neuropsychological test results, Dean did appear to meet criteria for Conversion Disorder (American Psychiatric Association, 2000). In Conversion Disorder, individuals present deficits in voluntary or sensory functions that suggest a neurological or other general medical condition. The constellation of symptoms cannot, after appropriate investigation, be fully explained by a medical or neurological condition, substance abuse, or culturally sanctioned behavior. Psychological factors are judged to be associated with the condition because the initiation or exacerbation of symptoms coincides with the emergence of psychosocial stressors. Exclusion criteria include the ability to explain the individual’s presentation through medical conditions or symptoms not limited to pain or sexual dysfunction. One main recommendation came from the evaluation: Psychotherapy could provide Dean an environment to explore the relationship between psychosocial stress and physical functioning. Counseling could also provide a supportive and nurturing environment for him to identify his emotional state and learn effective coping strategies to use when he becomes distressed. In addition, counseling could provide Dean’s parents with a better understanding of the nature of his behavioral issues and an opportunity to learn strategies to facilitate his psychological and

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emotional development. Dean should be in psychotherapy at least weekly and treatment may be long-term. Was the full neuropsychological assessment needed? Probably not absolutely, but it did increase the clinician’s degree of confidence in ruling out alternative explanations to the Conversion Disorder diagnosis. This could be seen as the beginning of a psychotherapeutic process, in which a qualified professional carefully considered the cognitive complaints presented by an important member of the system (Dean’s father) whose ongoing support will certainly be needed to make treatment progress. The role of the neuropsychological assessment in cases such as this probably reduces to a post-hoc analysis of what elements in the overall medical and psychiatric care were necessary and sufficient in the end.

Additional Resources Key Concepts and Terms Conversion Disorder A condition that presents as an alteration or loss of a physical function suggestive of a physical disorder; however, no medical or neurological etiology can be identified. Consequently, such a presentation is presumed to be the expression of an underlying psychological conflict or need. La Belle Indifference A relative lack of concern about the nature or implications of symptoms manifested on the part of the patient. Myofascial Pain Syndrome Pain and inflammation in the body’s soft tissues. It is a chronic condition that affects the fascia (connective tissue that covers the muscles). Myofascial pain syndrome may involve either a single muscle or a muscle group. Postconcussive Syndrome Common, albeit controversial, sequelae of traumatic brain injury. It is a symptom constellation that includes headache, dizziness, psychological symptoms, and cognitive impairment. Nonepileptic Seizures Commonly known as pseudoseizures. A disorder where the patient presents with neurological signs of a seizure disorder without the consistent clinical behaviors of seizure or abnormal EEG correlates. The etiology of pseudoseizures is thought to be psychological and resulting from stressors in the individuals’ life.

References Resources for Clinicians and Families American Academy of Pediatrics (1999). The management of minor closed head injury in children. Pediatrics, 104, 1407–1415.

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American Psychiatric Association (2004). Diagnostic and statistical manual of mental disorders (4th ed., text revision). Washington DC: American Psychiatric Association. Coleman, R. D., Rapport, L. J., Millis, S. R., Ricker, J. H., & Farchione, T. J. (1998). Effects of coaching on detection of malingering on the California Verbal Learning Test. Journal of Clinical and Experimental Neuropsychology, 20, 201–210. Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (2000). California verbal learning test second edition, adult version. San Antonio, TX: Harcourt Assessment Company. Kirmayer, L. J., & Taillefer, S. (1997). Somatoform disorders. In S. M. Turner & M. Hersen (Eds.), Adult psychopathology and diagnosis (pp. 333–383). New York: Wiley. McCrea, M. A. (2008). Mild traumatic brain injury and postconcussion syndrome: The new evidence base for diagnosis and treatment. New York: Oxford University Press. Mild Traumatic Brain Injury Committee of the Head Injury Interdisciplinary Special Interest Group of the American Congress of Rehabilitation Medicine. Definition of mild traumatic brain injury. Journal of Head Trauma Rehabilitation, 8, 86–87. Millis, S. R., Putnam, S. H., Adams, K. M., & Ricker, J. H. (1995). The California Verbal Learning Test in the detection of incomplete effort in neuropsychological evaluation. Psychological Assessment, 7, 463–471. Sweet, J. J., Wolfe, P., Sattlberger, E., Numan, B., Rosenfeld, J. P., Clingerman, S., & Nies, K. J. (2000). Further investigation of traumatic brain injury versus insufficient effort with the California Verbal Learning Test. Archives of Clinical Neuropsychology, 15, 105–113. World Health Organization (1992). The ICD-10 classification of mental and behavioural disorders: Clinical descriptions and diagnostic guidelines. Geneva: World Health Organization.

Chapter 32

A Mystery of Perplexing Symptoms: Neuropsychological Assessment in a Case of Dysautonomia Robert F. Newby and Safwan S. Jaradeh

Poor misunderstood Laura! Preschool staff filled out referral forms about her struggle with ‘‘physical and verbal control when with others’’ and ‘‘swaying attention.’’ Her kindergarten teacher felt that she actually enjoyed disturbing others, although Laura’s parents suspected that this teacher’s status as the oldest and most dominant in the traditional culture at her small rural school might have been coloring her view of students who did not sit and do their work quietly. Were her periods of apparent fatigue really signs of becoming less interested in the work? Elementary grade staff debated if she might be a ‘‘tactile/visual learner’’ rather than an ‘‘auditory learner.’’ Things became particularly perplexing when she started showing quirky medical features, such as rapid weight shifts, losing her sense of smell at age 6, and being diagnosed with asthma and then unexpectedly never having asthma symptoms again after a 10-day course of steroids. Pediatric neuropsychologists occasionally see such confusing symptom patterns, often combining physical and behavioral features, often for year after year, that are eventually discovered to have a low-prevalence neurological disorder that helps knit things together. Laura’s break came at age 8, when careful neurological evaluation uncovered a diagnosis of dysautonomia. Laura’s main neurological issues at that time included headaches, dizziness, intermittent vomiting, chronic constipation, fatigue, and weight changes. The cause of these symptoms was not initially apparent, so the neurologist began with a detailed medical history. Laura was born at 29 weeks gestation and delivered via cesarean section because of transverse lie. Her birth weight was 3 pounds and 10 ounces, with Apgar scores of 5 at 1 min and 8 at 5 min. She also had breathing difficulties at birth and was on a ventilator for 3 days. During the first 2 years of her life, she developed reflux and was treated with cisapride. Her motor development was unremarkable, and she sat and walked at the expected stages. She was toilet trained before age 3. However, at age 5, she developed chronic constipation and intermittent fecal soiling. She also had several issues with carsickness, and then started having headaches followed by vomiting; these episodes typically resolved after sleep. Laura also sometimes felt

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dizzy and tight in the chest when she got up abruptly, which was worse when she was having a headache. She did not have loss of consciousness, but she felt almost faint when headache and vomiting occurred together. When Laura started physical education classes at school, she enjoyed many activities, but she showed excessive muscular fatigue even after 20 minutes of exercise. The family also noticed that during a vacation, Laura could keep up with her siblings only for the first hour or so, and her endurance declined further when the day was warmer. One of the most unique symptoms when she first presented for neurological evaluation consisted of rapid weight shifts, despite a well-monitored diet. Laura had a detailed metabolic workup, which was negative. Chromosomal studies to check for Prader-Willi and Angelman syndrome were normal. She had normal creatine kinase (CK), thyroid function tests, lactate and pyruvate, porphyrins, biotinidase, and carnitine profile. An endocrinologist did not find evidence for mastocytosis. An evaluation by an allergist at that time was also negative. An electromyography (EMG) study was normal, and did not reveal any neuropathy or myopathy. A sleep study was abnormal and showed a mild degree of obstructive sleep apnea and an excessive number of periodic leg movements during sleep. An abdominal and pelvic computed tomography (CT) scan showed mildly dilated small bowels and colon. Various endoscopic biopsies (from the esophagus, stomach, duodenum, ileum, and colon) were normal. Magnetic resonance imaging (MRI) of her brain, cervical, thoracic, and lumbosacral spine was normal. On neurological examination, she was alert and oriented and appeared quite happy as a child. However, her mood was quite labile. She was mildly overweight. She had no dysmorphic features. While the neurologist was interviewing her mother, she kept climbing to the exam table from the floor several times on her own. Her cranial nerve examination showed decreased smell but no other abnormalities. Her strength testing was intact. Her deep tendon reflexes were present and symmetric. Her sensory examination was normal. Her plantar response was flexor bilaterally. Her cerebellar examination was intact. She was able to stand up from the knee position without difficulties. Her gait was normal, without pelvic waddling. Laura had autonomic testing, which showed patchy decrease in the sweating over her trunk, fingertips, and toes. The sweating was normal over her forehead. Her tilt test showed significant orthostatic tachycardia (her heart rate increased by 57 beats in the first 10 minutes of tilt), which became associated with her symptoms of chest tightness and dizziness. The symptoms persisted for the entire duration of the tilt test (20 minutes), and then she developed a typical episode of headache after she was tilted back. The valsalva maneuver and heart rate variability to deep breathing were intact. The clinical impression was that of mild dysautonomia with orthostatic intolerance, migraine, and vomiting. The patient’s dysautonomia was also felt to explain her constipation and her significant fatigue. The fluid shifts could also account for her rapid fluctuations of weight. Treatment began at 8 years of age with hydration and low-dose pyridostigmine to improve her vasomotor tone. At her follow-up visit 3 months later, she was markedly improved. Her mother thought that her muscle tone and endurance were

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much better. Her constipation was also better, and the frequency of her headaches decreased significantly. Because her exercise tolerance became better, her physical conditioning improved, and her episodes of dizziness and chest pain decreased significantly. Of interest, Laura’s mother also commented on improvement in her attention span and emotional lability. The benefits were sustained at subsequent follow-ups at 6 months and 1 year later, although fluctuations in symptoms reemerged at times, requiring medication adjustment. Laura’s neurologist referred her for neuropsychological evaluation at age 11, during the spring of fifth grade, because teachers wondered about the possibility of Attention Deficit Hyperactivity Disorder (ADHD), and the neurologist also wanted to check for other cognitive deficits that might be episodically associated with her dysautonomia. She had been in psychotherapy off and on from the time of her neurological diagnosis, working on issues such as aggression turned inward in relation to her long period of insufficiently explained medical problems, passivity interacting with her parents’ anxieties and control messages, and often-critical teachers. The therapist did not have the impression that Laura showed classic ADHD, even though she could go so deep inside herself at times that she could neglect to attend to concrete things happening at the moment and thus not remember them later. Laura had shown strong growth in academic skills despite the disruption of fatigue and headaches, and the result from her standardized group-administered testing at school was average to above average, so a learning disability appeared unlikely. A brief neuropsychological assessment was planned to focus on overall cognitive abilities, attention, and executive mental functioning. Then a series of extraneous events that can delay these things came up, including the birth of a younger sibling, the wish to not have Laura miss school for a testing appointment during the last few weeks of school, and scheduling problems in the clinic. In the meantime, behavior rating questionnaires were collected at home and school, she finished the school year strongly after a medication adjustment helped her remain symptom free for a period of time, and she settled into a more relaxed routine for the summer. The neuropsychologist carefully reviewed report cards from school since kindergarten, finding no consistent or compelling history of ADHD symptoms. Careful history-taking and several months of the watching-and-waiting approach seemed to have paid off, as the clinical information now appeared to adequately account for Laura’s fluctuations in functioning over the years, which correlated with her medical condition. Particularly in light of questions about the specificity and sensitivity of neuropsychological testing in suspected ADHD, was testing to be considered optional, advised, medically necessary, or something else at this time? Laura’s parents, with characteristic thorough and detailed orientation to their children’s well-being, decided to proceed.

Test Results Laura began testing on a day when she had been generally symptom free for several months, and she showed her normal level of energy. However, her mother reported at the feedback session the following week that Laura was already showing gradual

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reappearance of symptoms such as swelling at the time of testing. In part because Laura might be in a period of change in her general balance of well-being vs. malaise, and in part because the psychosocial and educational history had not suggested consistent ongoing impairment in behavioral or cognitive function (except in periods of increased dysautonomic symptoms, which were treatable with adjustments in medication), a limited sampling of neuropsychological functions was assessed for baseline purposes. Laura’s effort and cooperation throughout testing were adequate to ensure that the reported results were valid and reliable. She was talkative and pleasant, smiling frequently, and sometimes singing or humming while completing tasks. She did not become frustrated during the testing, but she did state when items were becoming more difficult for her. Most of the time, she thought carefully before choosing an answer. On one executive task, however, she began each problem very quickly, seemingly without thinking ahead. She often stood up during tasks or sat on her knees, leaning over the table. She frequently fidgeted and moved around throughout the testing. She also played with pencils, cups, and wrappers. Although she appeared to become slightly fatigued toward the end of the testing session, neither this nor her signs of restlessness seemed to affect her performance, and she continued to think about and put forth a great deal of effort on all tasks. Laura’s overall intelligence was in the average range, with equivalent verbal reasoning skills and visual-spatial problem-solving skills (Table 32.1). Among core verbal subskills, she showed high average vocabulary knowledge, with average commonsense social reasoning and word comparison skills or abstract verbal conceptualization. She was average on a cluster of subtests sensitive to verbal working memory. Among core visual-spatial subskills, she was average in abstract Table 32.1 Intellectual Wechsler Intelligence Scale for Children – Scorea Fourth Edition Full Scale IQ 102 General Ability Index 108 Verbal Comprehension Index 106 Similarities (11) Vocabulary (13) Comprehension (10) Perceptual Reasoning Index 108 Block Design (10) Picture Concepts (11) Matrix Reasoning (13) Working Memory Index 104 Digit Span (11) Letter-Number Sequencing (11) Processing Speed Index 80 Coding (5) Symbol Search (8) a Standard score, (scaled score), [T-score], {z-score}.

Percentile 55th 70th 66th 63rd 84th 50th 70th 50th 63rd 84th 61st 63rd 63rd 9th 5th 25th

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visual construction when copying block design patterns and discerning concepts in pictures and high average in visual analogies. She was mildly impaired on a cluster of subtests that relied on visual-perceptual processing speed. Assessment of attention, impulse control, and related executive mental functions suggested mild inconsistencies (Table 32.2). When Laura was asked to respond to a specified number sequence flashing on a small computer screen over a several minute period, her sustained attention was in the normal range, as was her number of incorrect impulsive responses. When asked to work on math problems alone while being observed through a one-way window for 10 min, she showed modestly excessive off-task time and fidgeting. Her planning and sequential problem-solving were mildly impaired, but she showed high average basic concept formation and adaptive switching of mental sets. She made excessive slips in maintenance of mental sets, but this appeared related to her unique behavior of humming throughout this particular test. Her visual searching speed was average both with a simple number sequence and with an alternating number and letter sequence. Behavior-rating questionnaires were completed by her parents and teachers with separate reference to a flare-up of dysautonomic symptoms several months before vs. her more recent stable period (Table 32.2). The Child Behavior Checklist (CBCL) during the symptomatic time showed clinically significant withdrawn/depressed symptoms, physical problems, attention problems, and unusual behavior patterns (e.g., extra fantasy talk and unexpected eye movements) and clinically borderline social problems. The CBCL during the relatively symptom-free period was within the normal range. The Teacher Report Forms for the two periods were virtually identical, with clinically borderline withdrawn/depressed symptoms.

Formulation and Recommendations Laura showed modest fluctuations in executive measures and mental processing speed on testing, but these were not sufficient to diagnose any of the three types of ADHD in light of her history. During periods of symptom exacerbation, her concentration and work persistence suffered, and then subsequently improved with treatment modifications. Subtle (and sometimes not so subtle) contextual issues in family and school interactions contributed to Laura’s level of anxiety, frustration, and initiative-taking. Her overall cognitive functioning and strong academic skills rendered other, more detailed neuropsychological assessment not needed at the time of the evaluation reported here. The neurologic determination of dysautonomia is distinct from, but perhaps relevant to, psychophysiological studies of the relationship between autonomic reactivity and psychopathology. For instance, Boyce et al. (2001, p. 148) found that ‘‘children with behavior suggestive of developmental psychopathology revealed profiles of upregulated or downregulated physiological arousal derived from one or both branches of the autonomic nervous system,’’ including differences between early elementary aged children with internalizing vs. externalizing symptoms. The authors suggested that these patterns might contribute to estimating

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R.F. Newby, S.S. Jaradeh Table 32.2 Other functions Tests Gordon Diagnostic System Vigilance task (9 min) Correct Commission errors Restricted Academic Task Off-task Fidgeting Vocalizing Playing with objects Out of seat Trailmaking Test Part A Part B Wisconsin Card Sorting Test Categories Total errors Perseverative responses Perseverative errors Nonperseverative errors Conceptual level responses Failure to maintain set The Tower of London Achenbach Child Behavior Checklist Parent 1 Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior Parent 2 Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior Teacher’s Report Form 1 Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems

Raw score

Score

Percentile

44 4 50th 45th 0 35th 0 99 91

117 116 116 113 120 72

[53] [70] [74] [65] [70] [69] [50] [51]

62nd >97th >97th 93rd >97th 97th 50th 54th

[50] [54] [50] [51] [50] [51] [50] [50]

50th 65th 50th 54th 50th 54th 50th 50th

[52] [67] [62] [53] [60]

58th 96th 89th 62nd 84th

47th 27th >16th 87th 86th 86th 81st 91st 6th–10th 3rd

(continued)

32 A Mystery of Perplexing Symptoms Table 32.2 (continued) Tests Attention problems Rule-breaking behavior Aggressive behavior Teacher’s Report Form 2 Anxious/depressed Withdrawn/depressed Somatic complaints Social problems Thought problems Attention problems Rule-breaking behavior Aggressive behavior

349

Raw score [54] [50] [53]

Score 65th 50th 62nd

[51] [67] [62] [53] [64] [56] [50] [53]

54th 96th 89th 62nd 92nd 73rd 50th 62nd

Percentile

children’s risk for psychopathology. It is feasible that Laura’s case illustrated a connection between abnormal autonomic functions and her development of internalizing symptoms in particular. On the one hand, when Laura has future exacerbations of dysautonomic symptoms, it might contribute to educational programming or behavior management to selectively repeat some of the attention and executive function measures in the neuropsychological evaluation. On the other hand, the main intervention at such times would involve medication adjustment based on relevant physical symptoms, and everyday life observation could be quite sufficient for pragmatic, short-term psychosocial treatment planning and adjustment of school requirements (as outlined in some of the previous chapters on ADHD). Laura’s continuing psychotherapy provides ongoing support through more difficult periods and is probably relevant for preventative work on what could be an autonomic-based increased risk for psychopathology in her case.

Additional Resources Key Concepts and Terms Dysautonomia Any dysfunction of the autonomic nervous system (ANS). Often the result of failure of the sympathetic or parasympathetic components of the ANS and rarely the result of excessive ANS activity. May be focal, as in complex regional pain syndrome, but is more often generalized; its course can be acute and reversible or chronic and progressive. Electromyography (EMG) Test for motor neuron and muscle function in which an electrode that is inserted into muscle records the action potentials generated by muscle activity.

350

R.F. Newby, S.S. Jaradeh

Neuropathy Disorder affecting a segment of the nervous system, particularly cranial or peripheral nerves. Myopathy Abnormal condition or disease of muscular tissues; most commonly refers to skeletal muscle.

References Resources for Clinicians Boyce, W. T., Quas, J., Smider, N. A., Essex, M. J., Kupfer, D. J., & the MacArthur Assessment Battery Working Group of the MacArthur Foundation Research Network on Psychopathology and Development. (2001). Autonomic reactivity and psychopathology in middle childhood. British Journal of Psychiatry, 179, 144–150. Menkes, J. H., Sarnat, H. B., & Maria, B. L. (Eds.) (2006). Child Neurology (7th ed.). Philadelphia: Lippincott, Williams and Wilkins.

Resources for Families Dysautonomia Foundation, 315 W. 39th Street, Suite 701, New York, NY 10018. Dysautonomia Youth Network of America, Inc., 1301 Greengate Court, Waldorf, MD 20601, [email protected], http://www.dynakids.org, Tel: 301-705-6995, Fax: 301-638-DYNA [email protected], http://www.familialdysautonomia.org, Tel: 212-279-1066, Fax: 212-279-2066. Familial Dysautonomia Hope Foundation, Inc. (FD Hope), 110 Deerwood Court, Chapel Hill, NC 27517, [email protected], http://www.fdhope.org, Tel: 828-238-3149. National Dysautonomia Research Foundation, http://www.ndrf.org/

Appendix

Description of Tests and Reported Scores

Test Achenbach Child Behavior Checklist

Adaptive Behavior Assessment System (Second Edition) Attention Deficit Hyperactivity Disorder Rating Scale (Fourth Edition)

Animal Fluency/Animal Naming

Assessment of Basic Language and Learning Skills Beery–Buktenica Developmental Test of Visual–Motor Integration Behavior Assessment System for Children

Behavior Assessment System for Children (Second Edition) Behavior Rating Inventory of Executive Function Benton Facial Recognition Test Benton Judgment of Line Orientation Test Boston Naming Test

Source information Achenbach, T. M. (1991). Integrative guide to the 1991 CBCL/4–18, YSR, and TRF profiles. Burlington, VT: Department of Psychology, University of Vermont. Harrison, P., & Oakland, T. (2003). San Antonio, TX: Harcourt Assessment. DuPaul, G. J., Power, T. J., Anastopoulos, A. D., & Reid, R. (1998). ADHD rating scales-IV: Checklists, norms, and clinical interpretation. New York: Guilford. Bousfield, W. A., & Sedgewick, H. W. (1944). An analysis of sequences of restricted associative responses. Journal of General Psychology, 30, 149–165. Gruenewald, P. J., & Lockhead, G. R. (1980). The free recall of category examples. Journal of Experimental Psychology: Human Learning and Memory, 6, 225–240. Partington, J. W. Pleasant Hill, CA: Behavior Analysts. Beery, K. E., Buktenica, N. A., & Beery, N. A. (2004). Minneapolis, MN: Pearson. Reynolds, C. R., & Kamphaus, R. W. (1992). Behavior assessment system for children: Manual. Circle Pines, MN: AGS. Reynolds, C. R., & Kamphaus, R. W. (2004). Circle Pines, MN: AGS. Gioia, G. A., Isquith, P. K., Guy, S. C., & Kenworthy, L. (2000). Lutz, FL: Psychological Assessment Resources. Benton, A. L. Lutz, FL: Psychological Assessment Resources. Benton, A. L. Lutz, FL: Psychological Assessment Resources. Kaplan, E., Goodglass, H., & Weintraub, S. Baltimore: Lippincott Williams & Wilkins. 351

352 Bracken Basic Concept Scale – Revised Buschke Selective Reminding Test

California Verbal Learning Test – Children’s Version California Verbal Learning Test (Second Edition) Children’s Depression Inventory Children’s Memory Scale Clinical Evaluation of Language Fundamentals – Preschool (Second Edition) Clinical Evaluation of Language Fundamentals (Fourth Edition) Comprehensive Test of Phonological Processing Conners’ Continuous Performance Test Conners’ Continuous Performance Test (Second Edition) Controlled Oral Word Association Test

Delayed Alternation Task

Delis–Kaplan Executive Function System Dichotic Digits Test

Differential Abilities Scale

Appendix Bracken, B. A. San Antonio, TX: Harcourt Assessment. Buschke, H. (1991). In O. Spreen, & E. Strauss (Eds.), A compendium of neuropsychological tests: Administration, norms, and commentary (pp. 125–138). New York: Oxford University Press. Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (1994). San Antonio, TX: The Psychological Corporation. Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. Lutz, FL: Psychological Assessment Resources. Kovacs, M. (1992). North Tonawanda, NY: MultiHealth Systems. Cohen, M. J. (1997). San Antonio, TX: The Psychological Corporation. Semel, E., Wiig, E. H., & Secord, W. A. Minneapolis, MN: Pearson. Semel, E., Wiig, E. H., & Secord, W. A. San Antonio, TX: The Psychological Corporation. Wagner, R. K., Torgeson, J. K., & Rashotte, C. A. (1999). Austin, TX: Pro-Ed. Conners, C. K. (1992, 1995). North Tonawanda, NY: Multi-Health Systems. Conners, C. K. (2000). North Tonawanda, NY: Multi-Health Systems. Benton, A. L., Hamsher, K. D., & Sivan, A. B. (1994). Multilingual aphasia examination: Manual of instructions (3rd ed.). Iowa City, IA: AJA Associates. Schum, R. L., Sivan, A. B., & Benton, A. L. (1989). Multilanguage aphasia examination: Norms for children. The Clinical Neuropsychologist, 3, 375–383. Levin, H. S., Culhane, K. A., Hartmann, J., Evankovich, K., et al. (1991). Developmental changes in performance on tests of purported frontal lobe functioning. Developmental Neuropsychology, 7, 377–395. Delis, D. C., Kaplan, E., & Kramer, J. H. San Antonio, TX: The Psychological Corporation. Moncrieff, D., & Musiek, F. (2002). Interaural asymmetries revealed by dichotic listening tests in normal and dyslexic children. Journal of the American Academy of Audiology, 13, 428–437. Musiek, F. E. (1983). Assessment of central auditory dysfunction: The dichotic digit test revisited. Ear and Hearing, 4, 79–83. Elliott, C. D. San Antonio, TX: The Psychological Corporation.

Appendix Expressive Vocabulary Test Finger Agnosia/Finger Gnosis

Finger Graphesthesis

Finger Tapping Test

Goldman–Fristoe–Woodcock Test of Auditory Discrimination Gordon Diagnostic System

Graphesthesia (Numbers)

Gray Oral Reading Test (Fourth Edition) Grip Strength

Grooved Pegboard Test Hopkins Verbal Learning Test Intermediate Booklet Category Test Kaufman Assessment Battery for Children Kaufman Brief Intelligence Test Luria’s Tapping Test

Multilingual Aphasia Examination

353 Williams, K. T. (1997). Circle Pines, MN: AGS. Critchley, M. (1953). The parietal lobes. New York: Hafner. Neilsen, J. (1938). Gerstmann’s syndrome: finger agnosia, agraphia, comparison of right and left and acalculia. Archives of Neurology and Psychopathology, 39, 536–560. Reitan, R. M., & Wolfson, D. (1993). The Halstead– Reitan neuropsychological test battery: Theory and clinical interpretation. Tucson, AZ: Neuropsychology. Christianson, M. K., & Leathem, J. M. (2004). Development and standardization of the computerized finger tapping test: Comparison with other finger tapping instruments. New Zealand Journal of Psychology, 33(2), 44–49. Minneapolis, MN: Pearson. Gordon, M. (1987). How is a computerized attention test used in the diagnosis of Attention Deficit Disorder? In: Loney, J. (Ed.), The young hyperactive child: Answers to questions about diagnosis, prognosis, and treatment. New York: Haworth. Reitan, R. M., & Wolfson, D. (1993). The Halstead– Reitan neuropsychological test battery: Theory and clinical interpretation. Tucson, AZ: Neuropsychology. Wiederholt, J. L., & Bryant, B. R. San Antonio, TX: Harcourt Assessment. Reitan, R. M., & Davison, L. A. (1974). Clinical neuropsychology: Current status and applications. Washington, DC: Winston. Lafayette, IN: Lafayette Instrument, 1989. Brandt, J., & Benedict, R. H. B. Lutz, FL: Psychological Assessment Resources. Byrd, P. B. Lutz, FL: Psychological Assessment Resources. Kaufman, A. S., & Kaufman, N. L. Minneapolis, MN: Pearson. Kaufman, A. S., & Kaufman, N. L. Circle Pines, MN: AGS. Diamond, A., & Taylor, C. (1996). Development of an aspect of executive control: Development of the abilities to remember what I said and to “do as I say, not as I do”. Developmental Psychobiology, 29, 315–334. Benton, A. L., Hamsher, K. D., & Sivan, A. B. (1994). Multilingual aphasia examination: Manual of instructions (3rd ed.). Iowa City, IA: AJA Associates.

354 Name Writing

Nelson–Denny Reading Test NEPSY: A Developmental Neuropsychological Assessment Paced Auditory Serial Addition Test

Peabody Picture Vocabulary Test (Fourth Edition) Pitch Pattern Sequence Progressive Figures Purdue Pegboard Qualitative Reading Inventory Random Gap Detection Test Rapid Automatized Naming/Rapid Alternating Stimuli Reitan-Indiana Aphasia Screening Test

Restricted Academic Task

Rey–Osterrieth Complex Figure Test

Rosner Test of Auditory Awareness Skills

Scales of Independent Behavior – Revised

Scan-C: Test for Auditory Processing Disorders in Children – Revised Sensory Processing Patterns

Appendix Yeudall, L. T., Reddon, J. R., Gill, D. M., & Stefanyk, W. O. (1987). Normative data for the Halstead–Reitan neuropsychological tests stratified by age and sex. Journal of Clinical Psychology, 43, 346–367. Brown, J. I., Fishco, V. V., & Hanna, G. S. Itasca, IL: Riverside Publishing. Korkman, M., Kirk, U., & Kemp, S. (1998). San Antonio, TX: The Psychological Corporation. Gronwall, D. M. A. (1977). Paced auditory serialaddition task: A measure of recovery from concussion. Perceptual and Motor Skills, 44, 367–373. Dunn, L. M., & Dunn, L. M. Circle Pines, MN: AGS. Auditec, http://www.auditec.com Halstead–Reitan Neuropsychology Laboratory, http://reitanlabs.com Lafayette, IN: Lafayette Instrument. Leslie, L., & Caldwell, J. A. (2006). Boston: Allyn & Bacon. Keith, R. W. Tartan Products, http://www.capdtest. com Wolf, M., & Denckla, M. B. Greenville, SC: Super Duper Publications. Williams, J. M., & Shane, B. (1986). The ReitanIndiana aphasia screening test: Scoring and factor analysis. Journal of Clinical Psychology, 42, 156–160. Fischer, M., & Newby, R. F. (1998). Use of the restricted academic task in ADHD dose–response relationships. Journal of Learning Disabilities, 31(6), 8–12. Osterrieth, P. A. (1944). Le test de copie d’une figure complexe. Archives de Psychologie, 30, 206–356. Meyers, J. E., & Meyers, K. R. (1995). Rey complex figure test and recognition trial: Professional manual. Odessa, FL: Psychological Assessment Resources. Rosner, J. (1979). Test of auditory analysis (TAAS). In Rosner, J. (Ed.), Helping children overcome learning difficulties: A step-by-step guide for parents and teachers (pp. 77–80). New York: Academic Therapy. Bruininks, R. H., Woodcock, R. W., Weatherman, R. F., & Hill, B. K. (1996). Itasca, IL: Riverside Publishing. Keith, R. W. Tartan Products, http://www.capdtest. com Dunn, W. (2007). Sensory profile supplement. San Antonio, TX: Harcourt Assessment.

Appendix Sensory Profile Symbol Digit Modalities Test Test of Auditory Processing Skills (Third Edition) Test of Early Mathematics Ability (Second Edition) Test of Early Reading Ability (Third Edition) Test of Early Written Language (Second Edition) Test of Language Development – Primary (Third Edition) Test of Problem Solving Test of Variables of Attention Test of Word Reading Efficiency Test of Written Language (Third Edition) The Token Test for Children The Tower of London

Time Compressed Words

Trailmaking Test Verbal Fluency Test (FAS)

Vineland Adaptive Behavior Scales (Second Edition) Visual Suppression Test Wechsler Abbreviated Scale of Intelligence Wechsler Adult Intelligence Scale (Third Edition) Wechsler Individual Achievement Test (Second Edition) Wechsler Intelligence Scale for Children – Revised Wechsler Intelligence Scale for Children (Third Edition) Wechsler Intelligence Scale for Children (Fourth Edition) Wechsler Intelligence Scale for Children (Fourth Edition, Integrated)

355 Dunn, W. San Antonio, TX: Harcourt Assessment. Smith, A. Western Psychological Services, http:// portal.wpspublish.com Martin, N., & Brownell, R. LinguiSystems, http:// www.linguisystems.com Ginsburg, H. P., & Baroody, A. J. Austin, TX: Pro-Ed. Reid, D. K., Hresko, W. P., & Hammill, D. D. Austin, TX: Pro-Ed. Hresko, W., Herron, S., & Peak, P. Austin, TX: Pro-Ed. Hammill, D. D., & Newcomer, P. L. Minneapolis, MN: Pearson. Bowers, L., Huisingh, R., & LoGiudice, C. East Moline, IL: LinguiSystems. The TOVA Company, http://www.hometova.com/ Torgesen, J., Wagner, R., & Rashotte, C. Minneapolis, MN: Pearson. Hammill, D. D., & Larsen, S. C. (1996). Minneapolis, MN: Pearson. DiSimoni, F. (1978). Austin, TX: Pro-Ed. Krikorian, R., Bartok, J., & Gay, N. (1994). Journal of Clinical and Experimental Neuropsychology, 16, 840–850. Sticht, T. G., & Gray, B. B. (1969). The intelligibility of time compressed words as a function of age and hearing loss. Journal of Speech and Hearing Research, 12, 443–448. Halstead–Reitan Neuropsychology Laboratory, http://reitanlabs.com Benton, A. L., & Hamsher, K. (1977). Multilingual aphasia examination. Iowa City: University of Iowa. Sparrow, S. S., Cicchetti, D. V., & Balla, D. A. (2005). Circle Pines, MN: AGS. Takemori, S. (1978). Visual suppression test. Clinical Otolaryngology, 3, 145–153. Wechsler, D. San Antonio, TX: Harcourt Assessment. Wechsler, D. (1997). San Antonio, TX: The Psychological Corporation. Wechsler, D. (2002). San Antonio, TX: The Psychological Corporation. Wechsler, D. San Antonio, TX: The Psychological Corporation. Wechsler, D. San Antonio, TX: The Psychological Corporation. Wechsler, D. San Antonio, TX: The Psychological Corporation. Wechsler, D. San Antonio, TX: The Psychological Corporation.

356 Wechsler Memory Scale (Third Edition) Wide Range Achievement Test – Third Revision Wide Range Achievement Test (Fourth Edition) Wide Range Assessment of Memory and Learning (Second Edition) Wisconsin Card Sorting Test

Woodcock Johnson III Tests of Cognitive Abilities Woodcock Johnson Psychoeducational Battery – Revised Woodcock Johnson Psychoeducational Battery (Third Edition) Woodcock Johnson Tests of Achievement (Third Edition) Woodcock Reading Mastery Test – Revised

Appendix Wechsler, D. San Antonio, TX: The Psychological Corporation. Wilkinson, G. S. (1993). Wilmington, DE: Wide Range. Wilkinson, G. S., & Robertson, G. J. (2006). Lutz, FL: Psychological Assessment Resources. Sheslow, D., & Adams, W. (2003). Wilmington, DE: Wide Range. Heaton, R. K., Chelune, G. J., Talley, J. L., Kay, G. G., & Curtiss, G. Lutz, FL: Psychological Assessment Resources. Woodcock, R. W., McGrew, K. S., & Mather, N. Itasca, IL: Riverside Publishing. Allen, TX: DLM Teaching Resources. Woodcock, R. W., McGrew, K. S., & Mather, N. Itasca, IL: Riverside Publishing. Mather, N., & Woodcock, R. W. (2001). Itasca, IL: Riverside Publishing. Woodcock, R. W. (1989). Minneapolis, MN: Pearson.

Scores indicate performance compared to other same aged children: standard scores have a mean of 100 and a standard deviation of 15 and an average range of 85– 115; scaled scores have a mean of 10 and a standard deviation of 3 and an average range of 7–13; T-scores have a mean of 50 and a standard deviation of 10 and an average range of 40–60; Z-scores have a mean of 0 and a standard deviation of 1 and an average range of 1 to +1 Percentile ranks show how well a child performed compared to a group of children the same age from across the United States. For example, if the percentile is 45, it means that he/she ranked at or above 45 out of 100 students his/her age, which would be considered solidly average Age equivalents, where reported, are in years–months Grade equivalents, where reported, are in the grade’s year and month

Index

Acoustic reflexes, 319, 322 Adaptive behavior, in autism, 240 Adaptive behavior, in seizure, 81, 83 Adolescent traumatic brain injury (TBI) behavioral changes in, 34, 35 cognitive functions evaluation, 37 computerized tomography (CT) scans, 34 IQ measurement, 38 neuropsychological testing and results, 35, 36 recovery, 40 speech and language evaluation, 39–40 American Speech-Language-Hearing Association (ASHA), 308 Analytic phonics-based program. See Phonological decoding Anoxic brain injury analytic phonics-based program, 134 articulation problems and language impairments, 130 encephalopathy and adjustment disorder, 126 immediate memory skills, 133 left-sided neglect, 129 pathological left-handedness syndrome, 92–93 pharmacotherapy trial, 135–136 post traumatic stress disorder, 126 processing speed in, 130 psychometric intelligence measures, 131–132 recommendations for, 126–127 slow attention and nocturnal enuresis, 133–134

slow information processing, 129 Antiepileptic drug (AED), 69 Antiepileptic medication, seizure, 79, 98 Anxious/depressed and thought problems scales, 210 Aphasia, 109 Applied behavioral analysis (ABA) therapy, 219, 242 Applied verbal behavior (AVB), 220–221 Asperger’s disorder behavior patterns, 297 sensory processing, 302–304 Asperger’s disorder, 241 Assessment of Basic Language and Learning Skills (ABLLS) Child’s Mastery Level Assessment labeling, 225 receptive language, 223 social interaction, 226 vocal imitation and request, 224 Attention deficit hyperactivity disorder (ADHD) abstract assignments tracking, 156 CAPD, 330 and CAPD overlap, 311, 318 combined type (ADHD-C) abstract reasoning in, 148 emotional functioning in, 146 organizational checklists and hyperactive tendencies, 147 double-deficit dyslexia, 176 dysautonomia, 345, 347, 349 environmental complications, 151–152 executive functions in, 155 357

358

inattention or hyperactive symptoms, 143 intellectual abilities and executive function skills, 153, 155 and nonverbal learning disability (NLD), 8 pediatric bipolar disorder, 245 section 504 evaluation plan, 157, 158 and shaken baby syndrome (SBS), 19 symptoms of, 146 types of, 142 Williams syndrome, 259, 260 Attention deficits, CAPD, 327 Auditory figure ground subtest, 309, 319 Auditory processing disorder (APD). See Central auditory processing disorder (CAPD) Autism diagnosis, 240–241 neuropsychological profile cognitive ability assessment, 234–235 developmental language test, 237–238 fine motor dexterity tests, 235 formal and visual attention test, 235–237 preacademic skills, 238 social interaction skills, 238–240 Autism spectrum disorders (ASD). See also Autism ABA therapy behavioral language classification, 220–221 model of, 220 ABLLS child’s mastery level assessment, 221–222 Steven’s skill development, 223–226 Child Development inVentory results, 219 consequences, 218, 240 neuropsychological assessment, 218–219 recommendations, 227 Beery-Buktenica Developmental Test reading disorder, 269 sensory processing, 298 Bracken School Readiness Assessment, 238

Index

California Verbal Learning Test for Children (CVLT-C), 208–209 Central auditory nervous system, 322 Central auditory processing disorder (CAPD) acoustic stimuli, 308and ADHD overlap, 311–317 assessment of diagnosis, 318 auditory skills, 309 cognitive function test, 311–317 diagnosis, 309, 310 formulation and recommendations, 329 mixed receptive and expressive language disorder, 330 organization and planning skills, 332 phonics skills, 330–331 problem solving skills, 332 reading strategies, 331 written language skills, 331–332 Goldman-Fristoe-Woodcock test, 319 hearing mechanics, 309 IEP evaluation, 310, 311, 321, 322, 325 intervention for, 320, 321 modifications for patients, 321 psychometric testing, 309, 310 symptoms, 308 test result in diagnosis, 319 Wechsler Intelligence Scale, 326 Woodcock-Johnson tests, 327 Cerebral vascular accident (CVA). See Intrauterine stroke Chemotherapy, neurological problems cognitive behavioral therapy techniques, 116–117 evaluation results emotional concerns, 115 intellectual ability, 111–112 motor dexterity and memory functions, 114 language difficulties, 110 leukemia, 109 trouble focusing, 111 Child Behavior Checklist (CBCL), dysautonomia, 347 Child life specialist, 67

Index

Clinical Evaluation of Language Fundamentals-fourth Edition (CELF-IV), Williams syndrome, 258 Cognitive ability assessment, 234–235 Cognitive behavioral therapy (CBT), 101, 116. See also Residential treatment center (RTC) Cognitive-behavioral treatment (CBT), Tourette’s syndrome, 274 Complex partial seizures, 69 Comprehensive Test of Phonological Processing (CTOPP) lingering dyslexia, 170 reading disorder, 268, 270, 271, 273 Concussion and mild traumatic brain injury, 43, 44 neuropsychological recommendations, 46, 48 test on persistent effect, 45–47 Wechsler adult intelligence scale, 47 Conversion disorder, 335 formulation and recommendations, 338, 339 postconcussive syndrome and la belle indifference, 340 psychotherapy and counseling, 340–341 nonepileptic seizures and myofascial pain syndrome, 336 Wechsler Adult Intelligence Scale, 337 Woodcock-Johnson tests, 338 Dactylitis, 51 Depression, 116 Developmental speech apraxia, 267 Diagnostic delineation, 241 Differential Abilities Scale (DAS) mild autism, 234 posthemorrhagic hydrocephalus, 5 Williams syndrome, 256–257 Double-deficit dyslexia. See also Naming-speed deficits assessment construct clusters, 177 double-deficit factors, 180 naming-speed deficits, 176–177 phonological deficits, 176 processing and achievement tests and criteria, 178

359

RAVE-O goals, 182 secondary learning difficulties, 181 skills pattern assessement, 181–182 WRMT-Passage Comprehension subtest, 179–180 WRMT-R Word Attack subtest, 179 Dysautonomia Child Behavior Checklist, 347 clinical impression, 344 formulation and recommendations behavior management, 349 psychopathology risk, 348–349 neurological evaluation, 343–345 Wechsler Intelligence Scale, 346 Dyslexia. See also Reading disability; Reading disorder eighth grader test results cognitive functions, 171 math word problems, 171–172 phonological awareness, 170 recommendation, 172 eleventh grader test results cognitive functions, 172 recommendation, 173 third grader test results cognitive functions, 169 recommendations, 169–170 Wechsler scales factor analysis, 167–168 Electroencephalogram (EEG), 87–88 Electromyography (EMG), dysautonomia, 344 Embolic stroke, 121 Emergency medical services (EMS), 33 Encephalomalacia, 122 Epilepsy, 77. See also Seizure Executive functions ADHD, 155 CAPD, 330 prefrontal-subcortical brain systems, 157 Expressive language disorder, 74 Fine motor dexterity tests, 235 Floor time therapy, 242–243 Fluorescence in situ hybridization (FISH) test, 255 FM system, 321, 322

360

Index

Glasgow Coma Scale (GCS), 24, 44 Goldman-Fristoe-Woodcock test, 319 Graphomotor difficulties, Williams syndrome, 261 Grooved Pegboard test, 209

Jaundice, 87, 130

Habit reversal training (HRT), Tourette’s syndrome, 275 Hemiparesis brain injury and physical trauma, 122, 123, 127 epilepsy, 79 pathological left-handedness, 87, 88 viral encephalitis, 98 Hemizygous microdeletion, 255 Hemorrhages adolescent traumatic brain injury, 34, 41 conversion disorder, 335 intraventricular definition, 10 Papile grading, 4 physical trauma and brain injury, 122 retinal, 14, 20, 21 subdural, 14, 20, 21 Higher order language skills, 74 Hydrocephalus, 4, 5 Hyperactive symptoms motor restlessness, 143, 144 test session behavior, 145

La belle indifference, conversion disorder, 340 Language-based academic functions. See Chemotherapy, neurological problems Left-hemisphere stroke, 92 Leukemia chemotherapy treatment, 115–116 classification, 110 stroke and aphasia, 109 Lingering dyslexia CTOPP, 170 Pennington’s scheme, 168–169 Lovaas therapy. See Applied behavioral analysis (ABA) therapy

Immediate memory skills, 133 Individualized education program (IEP) auditory processing disorder, 310 autism spectrum disorders, 222 brain injury and, 127 CAPD, 325 epilepsy and seizures, 77–79 leukemia and, 117 medulloblastoma and, 65 nonverbal learning disability, 211–212 pediatric bipolar disorder, 246 premature birth, 9 reading disability, 159 sickle-cell disease, 56 and TBI, 25 Intrauterine stroke, 87–88 Intraventricular hemorrhage (IVH), premature birth, 3, 4

Kaufman Brief Intelligence Test (K-BIT), 256–257

Medulloblastoma cognitive functions, 64 neuropsychological evaluation, 61 posterior fossa in, 59 radiation and chemotherapy, 60 recommendations implications of, 66 individualized education program (IEP), 65 test results Wechsler Intelligence Scale, 62 Woodcock-Johnson tests, 63 white matter damage, 63, 64 Memory functions, psychosocial factors, 114 Mild traumatic brain injury (TBI), 43. See also Concussion Mixed receptive and expressive language disorder CAPD, 330 reading disorder, 267 Mood disorder. See Pediatric bipolar disorder Myofascial pain syndrome, 336 Myopathy, dysautonomia, 344

Index

Naming-speed deficits analytic and synthetic phonic instruction, 183 multistep learning technique and ROAR, 184–185 tapes listening and independent reading, 185 word pattern review cards, 183–184 Nelson-Denny Reading Test, 198 Neuropathy, dysautonomia, 344 Nocturnal enuresis, 134 Nonepileptic seizures, 336 Nonverbal learning disability (NLD), 8 anxious/depressed and thought problems scales, 210 cognitive functions, 205–206 CVLT-C, 208–209 diagnostic principles, 211 Grooved Pegboard test, 209 group therapy and direct skill instruction, 213 Individualized education program (IEP), 211–212 intellectual functions, 204–205 perceptual reasoning and working memory scale, 207–208 similarities and picture completion subtest, 208 tactile perception and reading assessment, 209 verbal script development, 212 Obsessive-compulsive (OC) disorder, 274 Oppositional defiant disorder (ODD) diagnosis, 302–303 formulation and recommendations behavioral and sensory control, 304 medical evaluation, 305–306 psychotherapy, 305 structured social interaction, 304–305 sensory profile scores, 301–303 Paraphasia, 318 Pathological left-handedness syndrome, 92–93 Pediatric bipolar disorder cognitive functions, 249

361

depression symptoms and mental illness, 250 IEP and medication, 251 intellectual functions, 247 long-term treatment, 251–252 and psychosis, 249–250 rapid naming tasks, 248 Pennington’s scheme, lingering dyslexia, 168–169 Peripheral hearing system, 309 Peritoneal shunt, 21 Perseveration, seizure, 80 Pervasive developmental disorder, 241 See also Autism spectrum disorders (ASD) Phoneme aware deficit, 182 Phonemic decoding efficiency subtest, 179 Phonological awareness, 162–163 Phonological decoding abacus teaching method, 135 reading difficulties, 135 Phonological deficits, 176 Phonological memory CTOPP performance, 268, 269 definition, 194 Phonological processing method components of, 192 comprehensive test, 194, 197 RAVE-O method, 194–195 Physical trauma, brain injury embolic stroke, 121 encephalomalacia, 122 facial deformities, 123 hemiparesis and neglect, 122 intellectual and memory abilities, 123–124 Postconcussive syndrome (PCS), 340 Posttraumatic amnesia, 44 Pragmatic skills, 316, 318 Premature birth ADHD and NLD in, 8 and cognitive functions, 6, 7 complications of, 4 fine motor and visual-spatial weaknesses, 7–9 hydrocephalus, 4, 5 intellectual capabilities, 5

362

Index

intraventricular hemorrhages, 3, 4 neuropsychological testing in, 9, 10 Primitive neuroectodermal tumor (PNET), 59 Process assessment of the learner (PAL), 271, 272 Pseudoseizures. See Nonepileptic seizures Psychoeducation, 98–99 Ptosis, TBI, 24

psychiatric care, 97 psychoeducation, 101 Retinal hemorrhage, 21 Retrieval, automaticity, vocabulary elaboration, orthography (RAVE-O) method dyslexia, 170 reading fluency, 194 Right hemiparesis, 87

Reading disability cognitive and intellectual functions, 161 individualized education program (IEP), 159 learning difficulty and, 162–163 neuropsychological profile, 160–161 phonics training, 163 phonological awareness deficits, 163 wordwall concept, 164 Reading disorder initial evaluation cognitive testing, WIAT-II and CTOPP, 268–269 formulation and recommendations, 269–270 second evaluation formulation and recommendations, 273–275 TAPS-R and TOWL-3, 271–272 Wechsler Intelligence Scale, 270–271 Reading fluency neuropsychological evaluation, age 9 cognitive functions, 194 intellectual functions, 193 neuropsychological evaluation, age 17 cognitive functions, 197 intellectual functions, 196 phonological processing, 192, 194 psychotherapy, 197 RAVE-O method, 194–195 Repeated oral assisted reading (ROAR), 184 Residential treatment center (RTC) behavioral interventions, 102 care and medications, 104 cognitive behavior therapy (CBT), 102, 103 neuropsychological evaluation in, 105 primary treatment goals, 98–99

Schizophrenia, psychotic disorder, 250 Seeking patterns, sensory processing, 302–304 Seizures adaptive behavior, 81, 83 and behavioral problems, 70 cognitive and motor testing, 90–92 control of antiepileptic medication, 79, 98 neurosurgery and ketogenic diet, 77 vagus nerve stimulator (VNS), 106 electroencephalogram (EEG) monitoring, 88 formulation and recommendations, 74–75 hemispherotomy, 78 neuropsychological assessment intellectual ability testing, 88–90 language skills and eye movements, 79–80 visually-based reasoning skills disability, 80–81 perseveration, 80 role of antiepileptic drug, 69 source memory, 100 status epilepticus, 98 test results cognitive functions in, 71 emotional assessement, 72 intellectual screening, 73 visual field deficits, 79 Sensory processing behavioral and sensory control, 304 Dunn’s model and behavioral outcomes of, 302 neuropsychological testing, 297–301 sensory profile scores, 301–303 and structured social interaction, 304–305

Index

Wechsler Intelligence Scale, 295–296 Shaken baby syndrome (SBS) assessment in, 16 and attention deficit hyperactivity disorder, 19 cognitive functions, 17 intellectual functioning, 16, 17 neuropsychological impairments, 15 recommendations behavioral management, 20 verbally-based step-by-step approach, 21 traumatic brain injury and, 14 verbal list-learning task, 18, 19 visual and auditory attention, 18 Sickle-cell disease cognitive functions, 55, 56 dactylitis and acute chest syndrome, 51 executive functions, 54 focal silent ischemic infarct, 52 follow-up evaluation, 53, 54 individualized education plan, 56 initial evaluation, 52, 53 occupational therapy, 56–57 and stroke, 51, 56 Signal to noise ratio, 321 Somnolence, 38 Specific language impairment, 316 Speech and language pathologist (SLP), 267, 268, 271 Speech and language therapy, 242 Status epilepticus, in seizure, 98 Stroke. See Intrauterine strokeSubdural hematoma, 34 Test of Auditory Processing-Revised (TAPS-R), 267, 272 Test of Written Language-3 (TOWL-3), 271, 272 Time Compressed Words test, 319 Tourette’s syndrome (TS), 274–275 Traumatic brain injury (TBI) adolescence behavioral changes in, 34, 35 cognitive functions evaluation of, 37 formulations and recommendations, 38–40

363

IQ measurement of, 38 neuropsychological testing and results, 35, 36 recovery, 40 and ptosis, 24 school-aged children academic skills screening, 26, 27 behavior and token economy plan, 29 cognitive functions, 28 eyelid drooping, 24 neuropsychological testing, 27 neurostimulant for, 30 perseveration, 26 practice effects, 25 seizures, 30 and shaken baby syndrome, 14–20 Ventriculoperitoneal shunt, premature birth, 3 Visual attention test, 235–237 Visual field deficits, 79 Visual perception test, 238 Visuospatial construction, Williams syndrome, 256, 261, 263 Von Willebrand disease, 44 Wechsler Abbreviated Scale, 73 Wechsler Individual Achievement Test-II (WIAT-II), 268, 269 Wechsler Intelligence Scale for Children (WISC) ADHD, 143–145 adolescent TBI, 36 anoxic brain damage, 131–133 atypical ADHD, 153–154 CAPD, 326 conversion disorder, 337 double-deficit dyslexia, 177–178 dysautonomia, 346 dyslexia, 168 medulloblastoma, 62 pathological left-handedness, 89–90 pediatric bipolar disorder, 247 physical trauma, brain injury, 123–124 psychosocial factors, 112–113 psychotherapy, brain injury, 99 reading disorder, 346

364

reading fluency, 193 seizures, 82 sensory processing, 296 shaken baby syndrome, 17 sickle-cell disease and stroke, 53, 54 TBI, school-aged children, 26 Williams syndrome, 255 attention deficit hyperactivity disorder, 259–260 clinical assessment, 256 clinical evaluation of language fundamentals, 258 DAS and K-BIT, 256–257 formulation and recommendations adaptive self-statements, 262–263 reading comprehension skills, 261 social interaction and anticipatory anxiety, 262

Index

vocational skills, 261–262 written language skills, 261 visuospatial constructive abilities, 256, 261, 263 Woodcock-Johnson tests adolescent TBI, 36 CAPD, 327 conversion disorder, 338 medulloblastoma, 63 seizure disorder, 73 sensory processing, 287 sickle-cell disease, 53 WRMT-Passage Comprehension subtest, 179–180 WRMT-R Word Attack subtest, 179

Erratum

FM (Page No. ix, Contents) 10 Pathological Left-Handedness: Stroke and Seizures . . . . . . 87 Elizabeth N. Adams and Mariellen Fischer FM (Page No. xiii, Contributors Page) Mariellen Fischer, Ph.D. Professor, Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Chapter 10 (Page No. 87) Pathological Left-Handedness: Stroke and Seizures Elizabeth N. Adams and Mariellen Fischer In the print and online version of the original chapter, the second author’s name was omitted. The correct authors line is Elizabeth N. Adams and Mariellen Fischer. DOI 10.1007/978-0-387-78965-1