Chorea - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References

CHOREA A M EDICAL D ICTIONARY , B IBLIOGRAPHY , AND A NNOTATED R ESEARCH G UIDE TO I NTERNET R E FERENCES

J AMES N. P ARKER , M.D. AND P HILIP M. P ARKER , P H .D., E DITORS

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ICON Health Publications ICON Group International, Inc. 4370 La Jolla Village Drive, 4th Floor San Diego, CA 92122 USA Copyright 2004 by ICON Group International, Inc. Copyright 2004 by ICON Group International, Inc. All rights reserved. This book is protected by copyright. No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without written permission from the publisher. Printed in the United States of America. Last digit indicates print number: 10 9 8 7 6 4 5 3 2 1

Publisher, Health Care: Philip Parker, Ph.D. Editor(s): James Parker, M.D., Philip Parker, Ph.D. Publisher's note: The ideas, procedures, and suggestions contained in this book are not intended for the diagnosis or treatment of a health problem. As new medical or scientific information becomes available from academic and clinical research, recommended treatments and drug therapies may undergo changes. The authors, editors, and publisher have attempted to make the information in this book up to date and accurate in accord with accepted standards at the time of publication. The authors, editors, and publisher are not responsible for errors or omissions or for consequences from application of the book, and make no warranty, expressed or implied, in regard to the contents of this book. Any practice described in this book should be applied by the reader in accordance with professional standards of care used in regard to the unique circumstances that may apply in each situation. The reader is advised to always check product information (package inserts) for changes and new information regarding dosage and contraindications before prescribing any drug or pharmacological product. Caution is especially urged when using new or infrequently ordered drugs, herbal remedies, vitamins and supplements, alternative therapies, complementary therapies and medicines, and integrative medical treatments. Cataloging-in-Publication Data Parker, James N., 1961Parker, Philip M., 1960Chorea: A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References / James N. Parker and Philip M. Parker, editors p. cm. Includes bibliographical references, glossary, and index. ISBN: 0-497-00242-6 1. Chorea-Popular works. I. Title.

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Disclaimer This publication is not intended to be used for the diagnosis or treatment of a health problem. It is sold with the understanding that the publisher, editors, and authors are not engaging in the rendering of medical, psychological, financial, legal, or other professional services. References to any entity, product, service, or source of information that may be contained in this publication should not be considered an endorsement, either direct or implied, by the publisher, editors, or authors. ICON Group International, Inc., the editors, and the authors are not responsible for the content of any Web pages or publications referenced in this publication.

Copyright Notice If a physician wishes to copy limited passages from this book for patient use, this right is automatically granted without written permission from ICON Group International, Inc. (ICON Group). However, all of ICON Group publications have copyrights. With exception to the above, copying our publications in whole or in part, for whatever reason, is a violation of copyright laws and can lead to penalties and fines. Should you want to copy tables, graphs, or other materials, please contact us to request permission (E-mail: [email protected]). ICON Group often grants permission for very limited reproduction of our publications for internal use, press releases, and academic research. Such reproduction requires confirmed permission from ICON Group International, Inc. The disclaimer above must accompany all reproductions, in whole or in part, of this book.

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Acknowledgements The collective knowledge generated from academic and applied research summarized in various references has been critical in the creation of this book which is best viewed as a comprehensive compilation and collection of information prepared by various official agencies which produce publications on chorea. Books in this series draw from various agencies and institutions associated with the United States Department of Health and Human Services, and in particular, the Office of the Secretary of Health and Human Services (OS), the Administration for Children and Families (ACF), the Administration on Aging (AOA), the Agency for Healthcare Research and Quality (AHRQ), the Agency for Toxic Substances and Disease Registry (ATSDR), the Centers for Disease Control and Prevention (CDC), the Food and Drug Administration (FDA), the Healthcare Financing Administration (HCFA), the Health Resources and Services Administration (HRSA), the Indian Health Service (IHS), the institutions of the National Institutes of Health (NIH), the Program Support Center (PSC), and the Substance Abuse and Mental Health Services Administration (SAMHSA). In addition to these sources, information gathered from the National Library of Medicine, the United States Patent Office, the European Union, and their related organizations has been invaluable in the creation of this book. Some of the work represented was financially supported by the Research and Development Committee at INSEAD. This support is gratefully acknowledged. Finally, special thanks are owed to Tiffany Freeman for her excellent editorial support.

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About the Editors James N. Parker, M.D. Dr. James N. Parker received his Bachelor of Science degree in Psychobiology from the University of California, Riverside and his M.D. from the University of California, San Diego. In addition to authoring numerous research publications, he has lectured at various academic institutions. Dr. Parker is the medical editor for health books by ICON Health Publications. Philip M. Parker, Ph.D. Philip M. Parker is the Eli Lilly Chair Professor of Innovation, Business and Society at INSEAD (Fontainebleau, France and Singapore). Dr. Parker has also been Professor at the University of California, San Diego and has taught courses at Harvard University, the Hong Kong University of Science and Technology, the Massachusetts Institute of Technology, Stanford University, and UCLA. Dr. Parker is the associate editor for ICON Health Publications.

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About ICON Health Publications To discover more about ICON Health Publications, simply check with your preferred online booksellers, including Barnes&Noble.com and Amazon.com which currently carry all of our titles. Or, feel free to contact us directly for bulk purchases or institutional discounts: ICON Group International, Inc. 4370 La Jolla Village Drive, Fourth Floor San Diego, CA 92122 USA Fax: 858-546-4341 Web site: www.icongrouponline.com/health

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Table of Contents FORWARD .......................................................................................................................................... 1 CHAPTER 1. STUDIES ON CHOREA .................................................................................................... 3 Overview........................................................................................................................................ 3 The Combined Health Information Database................................................................................. 3 Federally Funded Research on Chorea ........................................................................................... 5 The National Library of Medicine: PubMed ................................................................................ 25 CHAPTER 2. NUTRITION AND CHOREA .......................................................................................... 69 Overview...................................................................................................................................... 69 Finding Nutrition Studies on Chorea .......................................................................................... 69 Federal Resources on Nutrition ................................................................................................... 70 Additional Web Resources ........................................................................................................... 71 CHAPTER 3. ALTERNATIVE MEDICINE AND CHOREA .................................................................... 73 Overview...................................................................................................................................... 73 National Center for Complementary and Alternative Medicine.................................................. 73 Additional Web Resources ........................................................................................................... 78 General References ....................................................................................................................... 78 CHAPTER 4. PATENTS ON CHOREA ................................................................................................. 81 Overview...................................................................................................................................... 81 Patents on Chorea ........................................................................................................................ 81 Patent Applications on Chorea .................................................................................................... 84 Keeping Current .......................................................................................................................... 85 CHAPTER 5. BOOKS ON CHOREA .................................................................................................... 87 Overview...................................................................................................................................... 87 Chapters on Chorea ...................................................................................................................... 87 CHAPTER 6. PERIODICALS AND NEWS ON CHOREA....................................................................... 93 Overview...................................................................................................................................... 93 News Services and Press Releases................................................................................................ 93 Academic Periodicals covering Chorea ........................................................................................ 94 CHAPTER 7. RESEARCHING MEDICATIONS .................................................................................... 97 Overview...................................................................................................................................... 97 U.S. Pharmacopeia....................................................................................................................... 97 Commercial Databases ................................................................................................................. 98 APPENDIX A. PHYSICIAN RESOURCES .......................................................................................... 101 Overview.................................................................................................................................... 101 NIH Guidelines.......................................................................................................................... 101 NIH Databases........................................................................................................................... 103 Other Commercial Databases..................................................................................................... 105 APPENDIX B. PATIENT RESOURCES ............................................................................................... 107 Overview.................................................................................................................................... 107 Patient Guideline Sources.......................................................................................................... 107 Finding Associations.................................................................................................................. 110 APPENDIX C. FINDING MEDICAL LIBRARIES ................................................................................ 113 Overview.................................................................................................................................... 113 Preparation................................................................................................................................. 113 Finding a Local Medical Library................................................................................................ 113 Medical Libraries in the U.S. and Canada ................................................................................. 113 ONLINE GLOSSARIES................................................................................................................ 119 Online Dictionary Directories ................................................................................................... 119 CHOREA DICTIONARY ............................................................................................................. 121

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INDEX .............................................................................................................................................. 185

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FORWARD In March 2001, the National Institutes of Health issued the following warning: "The number of Web sites offering health-related resources grows every day. Many sites provide valuable information, while others may have information that is unreliable or misleading."1 Furthermore, because of the rapid increase in Internet-based information, many hours can be wasted searching, selecting, and printing. Since only the smallest fraction of information dealing with chorea is indexed in search engines, such as www.google.com or others, a nonsystematic approach to Internet research can be not only time consuming, but also incomplete. This book was created for medical professionals, students, and members of the general public who want to know as much as possible about chorea, using the most advanced research tools available and spending the least amount of time doing so. In addition to offering a structured and comprehensive bibliography, the pages that follow will tell you where and how to find reliable information covering virtually all topics related to chorea, from the essentials to the most advanced areas of research. Public, academic, government, and peer-reviewed research studies are emphasized. Various abstracts are reproduced to give you some of the latest official information available to date on chorea. Abundant guidance is given on how to obtain free-of-charge primary research results via the Internet. While this book focuses on the field of medicine, when some sources provide access to non-medical information relating to chorea, these are noted in the text. E-book and electronic versions of this book are fully interactive with each of the Internet sites mentioned (clicking on a hyperlink automatically opens your browser to the site indicated). If you are using the hard copy version of this book, you can access a cited Web site by typing the provided Web address directly into your Internet browser. You may find it useful to refer to synonyms or related terms when accessing these Internet databases. NOTE: At the time of publication, the Web addresses were functional. However, some links may fail due to URL address changes, which is a common occurrence on the Internet. For readers unfamiliar with the Internet, detailed instructions are offered on how to access electronic resources. For readers unfamiliar with medical terminology, a comprehensive glossary is provided. For readers without access to Internet resources, a directory of medical libraries, that have or can locate references cited here, is given. We hope these resources will prove useful to the widest possible audience seeking information on chorea. The Editors

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From the NIH, National Cancer Institute (NCI): http://www.cancer.gov/cancerinfo/ten-things-to-know.

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CHAPTER 1. STUDIES ON CHOREA Overview In this chapter, we will show you how to locate peer-reviewed references and studies on chorea.

The Combined Health Information Database The Combined Health Information Database summarizes studies across numerous federal agencies. To limit your investigation to research studies and chorea, you will need to use the advanced search options. First, go to http://chid.nih.gov/index.html. From there, select the “Detailed Search” option (or go directly to that page with the following hyperlink: http://chid.nih.gov/detail/detail.html). The trick in extracting studies is found in the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer, and the format option “Journal Article.” At the top of the search form, select the number of records you would like to see (we recommend 100) and check the box to display “whole records.” We recommend that you type “chorea” (or synonyms) into the “For these words:” box. Consider using the option “anywhere in record” to make your search as broad as possible. If you want to limit the search to only a particular field, such as the title of the journal, then select this option in the “Search in these fields” drop box. The following is what you can expect from this type of search: •

Alzheimer's Disease and Dementing Disorders Source: Physical Medicine and Rehabilitation: State of the Art Reviews. 4(1): 9-17. February 1990. Summary: Dementia is a major cause of disability in the elderly. Alzheimer's disease and multiple brain infarctions account for more than 80 percent of patents with dementia. The rest suffer from other disorders such as Pick's disease, Parkinson's disease, Creutzfeldt-Jakob disease, Huntington's chorea, multisystem atrophy, and progressive supranuclear palsy. Other causes of dementia are potentially reversible, such as subdural hematoma, brain tumors, or normal pressure hydrocephalus. Subacute and chronic infections of the central nervous system (CNS) can also cause dementia, such as syphilis, AIDS, and cryptococcal meningitis. Factors extrinsic to the CNS may cause dementia, which is often preventable, arrestable, or reversible. Examples include

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drug intoxication, liver disease, thyroid disorders, high or low blood sugar, and thiamine deficiency. Lastly, mental depression in the elderly can sometimes mimic dementia. The etiology of Alzheimer's disease remains unknown, although evidence suggests a genetic factor. Experimental treatment of Alzheimer's disease with cholinergic agents has met with limited success. Reversible disorders must be ruled out prior to the diagnosis of Alzheimer's dementia in the elderly patient. 42 references. •

Implant-Supported Denture in a Patient with Huntington's Disease: Interdisciplinary Aspects Source: SCD. Special Care in Dentistry. 21(1): 15-20. January-February 2001. Contact: Available from Special Care Dentistry. 211 East Chicago Avenue, Chicago, IL 60611. (312) 440-2660. Summary: Patients with Parkinson's disease or Huntington's chorea often cannot maintain independent, efficient oral hygiene due to restricted motor ability of the upper extremities and lack of coordination. The hermetic closure of the mouth and lips, and the associated ability to keep liquid and toothpaste in the mouth, can become so weak that effective oral hygiene cannot be maintained. Over a period of many years, this illness leads to loss of teeth and the need for complete prosthodontic care. Dyskinesia and hyperkinesia of the tongue and the peri oral musculature, combined with xerostomia (dry mouth) and pooling of saliva, make it impossible for the patient to wear a conventional complete denture, despite an anatomically adequate bearing area. In such cases, an implant supported prosthesis is a better therapeutic measure, although some aspects of oral hygiene must initially be disregarded. This article reports the case of a patient with Huntington's chorea, in whom two ITI implants were inserted into the anterior mandibular (lower jaw) region. The implants were used because a complete denture could not be retained on the alveolar ridge, despite adequate vestibule depth, due to tongue dyskinesia. A bar joint was used to anchor this mucosal borne denture. This implant supported complete denture led to a clear improvement in the patient's chewing function when observed over a period of a year. The authors conclude that, given interdisciplinary cooperation between the attending neurologist and the dentist or oral surgeon, treatment by means of an implant supported prosthesis should be possible, even for patients with the most severe neurological symptoms, on a case by case basis, taking all relevant modifying factors into account. 3 figures. 27 references.

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Dental Management of a Child with Huntington's Disease: Case Report Source: SCD. Special Care in Dentistry. 13(2): 71-73. March-April 1993. Summary: This article presents a case report of the dental management of a child with Huntington's disease (HD), a chronic, progressive hereditary disorder characterized by chorea, behavioral changes, dementia, and irregular gait. Dyslalia is a common feature due to the severe darting movements of the tongue and head. This case report features an 11 year old white male, diagnosed at age 5, who was brought to the University of Minnesota Hospital Dental Clinic for an oral examination. The findings are presented, and the management of patients with HD are discussed. The authors conclude that if any dental treatment is rendered to HD patients it should be basic, including exodontia, simple operative, non-surgical periodontal therapy. Endodontics, crowns, and bridges are not indicated. Dentures or appliances of any kind are contraindicated. 6 references. (AA-M).

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Dementia Syndromes: Distinguishing Their Clinical Differences Source: Postgraduate Medicine. 95(5): 91-93, 97-101. April 1993. Summary: This article reviews the clinical differences in various dementia syndromes. It includes the primary dementias: Alzheimer's disease, Pick's disease, alcoholic dementia syndrome, and Marchiafava-Bignami disease; dementias associated with other neurologic deficits; and dementias that associate with other illnesses. Primary care physicians can help patients and their families by recognizing the differences in pathophysiology and clinical presentation among these forms of dementia. Other principal forms of dementia examined include multi-infarct dementia, Parkinson's disease, progressive supranuclear palsy, Creutzfeldt-Jakob disease, Huntington's chorea, acquired immunodeficiency syndrome dementia, neurosyphilis, and hypothyroidism. Final comments examine practical and important aspects of laboratory workup in patients with dementia to find treatable causes of reversible syndromes. 15 references.

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Rheumatic Fever: No Cause for Complacence Source: Patient Care. 34(14): 40-42,45-46,53,57,61. July 30, 2000. Summary: This journal article provides health professionals with information on the epidemiology, clinical characteristics, diagnosis, treatment, and prevention of rheumatic fever. This inflammatory disorder of the connective tissue can affect the heart, joints, brain, and cutaneous and subcutaneous tissues. Cardiac damage is the only potentially chronic debilitating effect. Factors with a role in the etiology of this disease include socioeconomic status, environmental factors, and heredity. The epidemiology of rheumatic fever is linked to that of streptococcal pharyngitis because it occurs most frequently in the spring following the peak period of streptococcal pharyngitis. Clinical manifestations, which usually occur 1 to 3 weeks after the onset of pharyngitis, include arthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules. Diagnosis is based on the presence of two major criteria or one major criteria and two minor criteria. Conclusive evidence of a preceding streptococcal infection must be present to confirm the diagnosis of rheumatic fever. Treatment consists of an intramuscular injection of 1.2 million U of long acting bezathine penicillin G. Aspirin should be started as soon as the diagnosis is suspected. Treatment lasts for 3 to 4 weeks, but when carditis is present, treatment may be required for 6 to 8 weeks. Corticosteroids are used primarily when severe carditis with congestive heart failure occurs. Rheumatic fever may recur with subsequent streptococcal infections, so penicillin may be administered. Sulfadiazine and erythromycin may be substituted when penicillin cannot be used. Sequelae include mild mitral insufficiency and aortic insufficiency. 3 figures, 3 tables, and 4 references.

Federally Funded Research on Chorea The U.S. Government supports a variety of research studies relating to chorea. These studies are tracked by the Office of Extramural Research at the National Institutes of Health.2 CRISP (Computerized Retrieval of Information on Scientific Projects) is a searchable database of 2

Healthcare projects are funded by the National Institutes of Health (NIH), Substance Abuse and Mental Health Services (SAMHSA), Health Resources and Services Administration (HRSA), Food and Drug Administration (FDA), Centers for Disease Control and Prevention (CDCP), Agency for Healthcare Research and Quality (AHRQ), and Office of Assistant Secretary of Health (OASH).

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federally funded biomedical research projects conducted at universities, hospitals, and other institutions. Search the CRISP Web site at http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen. You will have the option to perform targeted searches by various criteria, including geography, date, and topics related to chorea. For most of the studies, the agencies reporting into CRISP provide summaries or abstracts. As opposed to clinical trial research using patients, many federally funded studies use animals or simulated models to explore chorea. The following is typical of the type of information found when searching the CRISP database for chorea: •

Project Title: A NATIONAL RESOURCE FOR POSTMORTEM BRAIN RESEARCH Principal Investigator & Institution: Benes, Francine M.; Professor of Psychiatry (Neuroscience); Mc Lean Hospital (Belmont, Ma) Belmont, Ma 02478 Timing: Fiscal Year 2003; Project Start 17-SEP-2003; Project End 31-JUL-2008 Summary: (provided by applicant): This is a competitive renewal application for the Harvard Brain Tissue Resource Center, a national resource for the acquisition, processing, storage and distribution of high quality postmortem (PM) brain to the neuroscience community. This facility collects brains from normal controls (CON), as well as individuals with a variety of movement disorders (Huntington's chorea and Parkinson's disease), dementias (Alzheimer's disease, frontotemporal dementias) and major psychoses (schizophrenia and bipolar disorders). The HBTRC distributes tissue specimens to neuroanatomists, neuropathologists, neuropharmacologists, neurochemists and molecular biologists throughout the U.S. Over the past 5 years, this tissue has contributed to more than 170 scientific publications. During the current funding period, the HBTRC has undergone a major restructuring that has resulted in an increase in the overall quality and efficiency of its operation. An important outcome of this re-organization has been an increase in the number of fresh, frozen cases and a marked decrease in the postmortem interval (PMI). As a result of this effort, abundant, high quality tissue is now available for a broad array of state-of-the-art research applications. The number of CONs received by the HBTRC has more than doubled during the current funding period and the HBTRC is now able to provide investigators with sets of CONs and diseased brains matched for age, PMI, gender and hemisphere. For the major psychoses, where the acquisition of postmortem brains is actually challenging, the number of schizophrenic and bipolar cases has also increased. Since these are largely from community-based referrals, the potential confounding effects of institutionalization, inanition and substance abuse are relatively lower than in medical examiner cases. To facilitate the donation process, the HBTRC has established a website with a) Informed Consent forms for potential donors and their families, b) instructions for handling brains by pathologists or dieners involved in brain removal, and c) application forms for investigators seeking tissue for their research. The HBTRC has also developed a User-Interactive Database, so that investigators who receive tissue from the "bank" can obtain demographics, neuropathology reports and images of gross dissections and histological slides for their cases. With supplemental funding from NIH, the HBTRC is now obtaining gene expression profiling and SNP analyses using the standard Affymetrix microarray system and this information will be immediately placed in our public domain National Databank as it is obtained. All investigators receiving tissue from the HBTRC will also be encouraged to "deposit" their findings in the Databank so that they will be available to the neuroscience community for complex

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relational analyses. Over the next funding period, these new initiatives will enhance the functioning of the "brain bank" in its role as a vital national resource. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: AUTOIMMUNE NEUROPSYCHIATRIC DISORDERS

MECHANISMS

IN

PEDIATRIC

Principal Investigator & Institution: Trifiletti, Rosario R.; Assistant Professor; Weill Medical College of Cornell Univ New York, Ny 10021 Timing: Fiscal Year 2002; Project Start 01-DEC-2001; Project End 30-NOV-2002 Summary: Recent evidence suggests that Sydenham chorea, obsessive/compulsive disorder and Tourette syndrome may have a similar autoimmune basis. Dr. Zabriskie and co-workers have shown that a specific B-cell D8/17 clonal marker is greatly expanded in all of these groups. The goal of the present study is to correlate brain autoantigens, B-cell clonal status and neurologic, psychaitric and cognitive outcome in all of these groups. If correlations are found, efforts will be made to isolate the respective autoantigens. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: BASAL GANGLIA CIRCUITRY IMMEDIATE EARLY GENE EXPRESSION Principal Investigator & Institution: Wirtshafter, Robert D.; Psychology; University of Illinois at Chicago 1737 West Polk Street Chicago, Il 60612 Timing: Fiscal Year 2002; Project Start 01-DEC-1998; Project End 31-DEC-2003 Summary: (from Abstract) The long term objective of the current proposal is the attainment of a greater understanding of the functional organization of the basal ganglia. Substantial evidence suggests that many neurological and psychiatric disorders, including Parkinson's disease, Huntington's disease, Sydenham's chorea, torsion dystonia, Tourette's syndrome and schizophrenia, may result from abnormalities in the activity of these nuclei. Progress in understanding the etiology of these disorders, and in developing treatments for them, is largely dependent on advances in understanding the basic nature of basal ganglia functioning. The proposed experiments are designed to examine basal ganglia by using the immunocytochemical detection of immediate early genes (IEGs) as markers for neurons affected by various behavioral or pharmacological manipulations. The striatum is the largest nucleus within the basal ganglia and is the primary terminus of input into this system. The first set of studies will examine the pharmacological and behavioral control of IEG expression within this structure with emphasis on the patterning of IEG expression with respect to the striosome/matrix compartmentation of the striatum. The striatum contains a number of neuroactive compounds including dopamine, serotonin, adenosine, acetylcholine and substance P and the role played by these substances in controlling striatal IEG expression will be examined. The applicants will also examine the role of dopamine in IEG expression induced by shuttling behavior and characterize the cells that express IEGs under these conditions. Events occurring within the striatum can only influence behavior by affecting the activity of neurons within other parts of the brain. The second group of studies will therefore use IEG expression as a tool to investigate the basic organization of extrastriatal circuitry related to the basal ganglia. Experiments will examine the ability drugs microinjected directly into the striatum, or other basal ganglia nuclei, to influence IEG expression at extrastriatal sites. Other studies will examine the effects of

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lesions within the basal ganglia on the IEG expression induced by systemic administration of dopaminergic drugs. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: BRAIN IMAGING OF CHILDHOOD ONSET PSYCHIATRIC DISORDERS, ENDOCRINE DISORDERS Principal Investigator & Institution: Giedd, Jay N.; University of California Los Angeles 10920 Wilshire Blvd., Suite 1200 Los Angeles, Ca 90024 Timing: Fiscal Year 2002 Summary: Cerebral MRI will be used to assess brain anatomy and function in normal volunteers and subjects with a variety of childhood onset psychiatric disorders including attention deficit disorder, autism, congenital adrenal U hyperplasia. childhood-onset schizophrenia. dyslexia, multidimensional impairment syndrome. obsessive compulsive disorder, Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infection (PANDAS), stuttering, Sydenham's chorea, and Tourette's Syndrome. Quantitative measures of brain structure and function will be compared across age, gender. and diagnostic groups. Correlations between brain and behavioral measures will be examined for normal and clinical populations. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: CHOLINERGIC REGULATION OF THE CIRCADIAN CLOCK Principal Investigator & Institution: Gillette, Martha U.; Professor; Cell and Structural Biology; University of Illinois Urbana-Champaign Henry Administration Bldg Champaign, Il 61820 Timing: Fiscal Year 2002; Project Start 01-AUG-1997; Project End 31-MAY-2005 Summary: (provided by applicant): Acetylcholine (ACh) has diverse regulatory roles in the central nervous system. Cholinergic neurons in brainstem and basal forebrain structures send ascending fibers throughout the brain where they may participate in a wide range of functions, including modulation of learning, memory retrieval, mood states, central autonomic control, and the processes regulating sleep/arousal. Mechanisms by which cholinergic signals induce long-lasting changes in neuronal state, and their consequences for behavior, are areas of intense research interest with great importance for human health. We propose to investigate mechanisms of cholinergic signaling via M1 mAChRs (M1.Rs) to behaviorally relevant hypothalamic neurons and immortalized cells from rat, as well as in transgenic mouse models. Imaging of M1.R distribution within the suprachiasmatic nucleus (SCN) reveals substantial receptor concentrations. We have found that M1 subtype-like pharmacological reagents selectively alter the cholinergic response. Preliminary data using transgenic mice and immortalized cell lines support the pharmacology and point to this brain site as an important model in which to evaluate M1.R signaling. We propose to employ techniques ranging from behavioral analyses to extracellular and patch-clamp recordings in brain slices, accompanied by immunocytochemical, biochemical and cell biological approaches, together aimed at uncovering fundamental mechanisms of M1.R signaling. Our specific aims include: 1) To fully evaluate the effect of genetic deletion of the M1.R on the cholinergic response, 2) To assess actions of downstream diffusible messengers, and 3) To define the role of specific PKG isoforms in this M1.R signaling cascade. In addition to making fundamental contributions to understanding muscarinic neuromodulation of CNS neurons, these studies will permit us to evaluate the roles of M1.R-mediated neurotransmission within a defined behavioral axis. This

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multidisciplinary approach will provide new insights into central cholinergic neurotransmission, muscarinic signal transduction mechanisms, and decision-making processes that form the neural substrates of behavioral change. Signal transduction is a cellular process, and by identifying the roles of specific receptors, second messenger systems and targets, we will be able to understand the causal mechanisms that mediate long-term adjustments in neuronal state. This research has applied relevance for strategies in developing rationally-based therapies for cholinergic disorders, including those altering sleep/arousal, autonomic function, senile dementia, Alzheimer's type (SDAT), Parkinson's disease, Huntingtons chorea and other neuropsychiatric and movement disorders. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: PROCESSING

CORTICOSTRIATAL

INFLUENCES

ON

NEOSTRIATAL

Principal Investigator & Institution: Alloway, Kevin D.; Professor; Neuroscience and Anatomy; Pennsylvania State Univ Hershey Med Ctr 500 University Drive Hershey, Pa 170332390 Timing: Fiscal Year 2002; Project Start 01-JUL-1999; Project End 30-JUN-2007 Summary: (provided by applicant): The neostriatum and related parts of the basal ganglia contain functional channels that separately process prefrontal, limbic, oculomotor, and sensorimotor information received from the cerebral cortex. Although the specific cortical areas that activate each functional channel are different, each channel contains the same basic input-output pattern of connections. By using cutaneous stimuli to activate corticostriatal projections from somatosensory cortex, we can characterize the pattern recognition properties of neostriatal neurons and determine how neural activity in the sensorimotor channel is coordinated during sensory stimulation. Hence, this paradigm presents a unique opportunity for understanding the rules that govern the dynamic operation of corticostriatal circuits across all functional channels because there are no accepted methods for directly activating limbic or prefrontal channels in a controlled, naturalistic manner. The anatomic and physiologic properties of the neostriatum represent a significant issue in contemporary neuroscience because this brain region has been implicated in Parkinson's disease, Huntington's chorea, Tourette's syndrome, and schizophrenia. In this project we will use anterograde tracing methods to test the hypothesis that corresponding representations in the primary and secondary somatosensory cortical areas send convergent projections to the neostriatum. We will also use retrograde tracing techniques to verify previous results indicating that corticostriatal projections from somatosensory cortex have an anisostropic organization. We will also compare corticostriatal and corticopontine projections to determine if the corticopontine projections follow the same principles of organization that have been identified in the corticostriatal system (ie. Principles of Cortical Proximity, Somatotopic Homology, and Behavioral Cooperativity). Finally, we will record neuronal activity in multiple parts of the cerebral cortex and neostriatum so that we may analyze neuronal interactions between the cortex and neostriatum. These physiology studies will determine whether a primary function of neostriatal neurons is to detect synchronized activity among functionally-related cortical areas. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: DEVELOPMENT OF PET RADIOLIGANDS FOR NMDA RECEPTORS Principal Investigator & Institution: Waterhouse, Rikki N.; Assistant Professor of Neuroscience; Psychiatry; Columbia University Health Sciences Po Box 49 New York, Ny 10032 Timing: Fiscal Year 2003; Project Start 15-DEC-2002; Project End 30-NOV-2004 Summary: (provided by applicant): Alterations in normal NMDA channel subunit composition and function are implicated in the pathophysiology of certain neurological and neuropsychiatric disorders such as Parkinson's Disease, Huntington's Chorea, schizophrenia, alcoholism and stroke. Binding of NMDA ligands to succinct modulatory sites located on the outside of or within the ion channel serves to modulate behavioral and neurochemical responses. For this proposal, the NMDA/PCP site has been specifically chosen as an imaging target since the involvement of this site in cognitive and neurodegenerative processes and psychotic behaviors is well documented. The development of effective PET tracers for the study of NMDA receptors is critical to providing much needed tools for understanding of the etiology and improving the clinical management of disorders thought to involve the associated ion channel. The PCP site is located inside the ion channel. Effective PCP site tracers will bind their target selectively during the active and "open" state of the ion channel. Therefore, an effective PCP site tracer would serve as a non-invasive in vivo tool to report on the functional state of the channel. In addition to providing information regarding channel activation, these tracers would also be able to monitor the density of these sites. To identity effective PCP site tracers, novel substituted N, N'-alkyl-diphenyl guanidines will be synthesized and evaluated. Candidate ligands possessing high affinity, selectivity, and appropriate lipophilicity will be radiolabeled and further characterized in in vitro assays and in vivo models. In addition to aspects of radiotracer design synthesis, specific issues regarding appropriate methods to evaluate the new tracers for effective labeling of the ion channel in vivo will be addressed. To supplement the more classical methods of PET radiotracer evaluation, we will adopt an ex vivo method for determining the degree of in vivo saturable binding that occurs with these ligands. While well-established cell membrane assays will be used, an additional modified in vitro assay will allow us to probe the nature of binding of tracers to closed and open states of the channel. The use of these models will provide additional information to assist in the development of clinically useful PCP site radioligands. The data obtained will be pivotal for the design of studies to non-invasively assess NMDA receptor function and will identify suitable radiotracers and provide preliminary data to support future grant submissions. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: DOPAMINE TRANSPORTER IMAGING WITH FLUORINE-18 PET Principal Investigator & Institution: Neumeyer, John L.; Professor ( Emeritis), Director of Medic; Natural Pharmacia International, Inc. 115 Mill St Belmont, Ma 02478 Timing: Fiscal Year 2002; Project Start 01-JUN-2000; Project End 30-APR-2004 Summary: (provided by applicant):Positron emission tomography (PET) is sensitive and specific non-invasive imaging technology that can provide information about the functional status of neurotransmitter systems in-vivo. Recent efforts have focused on the development of PET-based radiotracers for use in studies of the dopamine transporter (DAT) abundance and pharmacology Changes in the density and function of DAT have been implicated in neurodegenerative and neuropsychiatric diseases such as Parkinson?s disease, major depression, Huntington's chorea, schizophrenia, and attention deficit-hyperactivity disorders (ADHD). Our earlier studies have focused on

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fluorine-18 labeled tropane derivatives, such as FP-C1T, in which the N-methyl group of the tropane was replaced by a [S18FJ fluoropropyl group. During the Phase I project, we characterized the cerebral monoamine transporter binding affinity of a series of novel fluoralkyl-containing tropane derivatives, which showed higher DAT affinity and selectivity than FP-C1T. These new ligands are attractive candidates for development of ?8F-labeled PET radiotracers for clinical imaging DAT in human brain. Our objectives on this Phase II project are to further synthesize and pharmacologically evaluate novel N- or 0-fluoroalkyl tropane derivatives with a view toward one-step simplified 18Fradiolabeling. The most promising compounds' physiochemical properties (lipophilicity) will be evaluated. A facile and rapid method of synthesis of 18F labeled tropane derivatives will be developed for the most promising compound(s). The lead compound in this series (presently BRL-308) will be evaluated by PET imaging and pharmacokinetics, in non-human primates. PROPOSED COMMERCIAL APPLICATION: NOT AVAILABLE Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: FUNCTIONAL NEUROANATOMY OF MOVEMENT DISORDERS Principal Investigator & Institution: Brown, Lucy L.; Associate Professor; Neurology; Yeshiva University 500 W 185Th St New York, Ny 10033 Timing: Fiscal Year 2002; Project Start 01-APR-1998; Project End 31-MAY-2007 Summary: (provided by applicant): Although the gene for Huntington's disease (HD) has been identified, the processes that lead to cell pathology and degeneration are still unclear. The proposed studies examine early pathology and pathophysiology in two transgenic mouse models of Huntington's disease. Our preliminary data in one mutant model, the reversible HD94 model, suggest that the chemoarchitecture of the striatum is altered in early symptomatic stages of the disease: there are more mu opioid receptorrich striosomes in mutants than in controls, which may lead to an imbalance of activity between the striosome and matrix compartments and produce the symptoms of chorea and involuntary activity. The studies will determine whether there are more striosomes in mutants by using immunocytochemical methods to identify striosomes. The working hypothesis states that the early stages of the disease are associated with abnormal developmental processes. The specific hypothesis is that a critical part of the pathology underlying the symptoms and final degenerative process of Huntington's disease is abnormal neurogenesis prenatally and postnatally, and that the number of striosomes in mutants reflects neurogenesis abnormalities. In addition, our model of the behavioral functions of striosomes predicts that mutants will be more sensitive to dopamine agonists. Finally, the studies will investigate prenatal and adult cell proliferation and neurogenesis in mutants and their controls by using a thymidine analogue, bromodeoxyuridine (BrdU). The studies may provide clues to the function of the gene huntingtin, and define a target for therapeutic strategies. In addition, these studies address the plastic and proliferative capacity of the adult brain. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: K OPIOIDS AND DOPAMINE--MECHANISMS OF STIMULANT ABUSE Principal Investigator & Institution: Izenwasser, Sari; Research Professor; Neurology; University of Miami-Medical Box 248293 Coral Gables, Fl 33124 Timing: Fiscal Year 2001; Project Start 30-SEP-1998; Project End 31-JUL-2004

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Summary: (Applicant's Abstract) Psychostimulant abuse is a considerable problem in society. Both cocaine and amphetamine are widely used, and the use of methamphetamine on the rise. These psychostimulant drugs produce their reinforcing effects via interactions with the dopamine transporter, cocaine by inhibiting dopamine uptake (the inward flow of dopamine into the neuron), and amphetamines by stimulating dopamine release via reversal of the transporter. These actions result in an increase in extracellular dopamine, which then interacts with dopamine receptors to increase dopaminergic transmission. In order to develop a treatment for the abuse of these substances, it is necessary to find a mechanism by which the activity of the dopamine transporter and dopamine receptors can be regulated. Repeated administration of a selective kappa-opioid agonist produces a down-regulation of the dopamine transporter, and of dopamine D: receptors, suggesting that the kappa-opioid regulation of dopamine neurotransmission might provide a mechanism by which to alter the effects of psychostimulants such as cocaine and amphetamine. The specific hypothesis of this proposal is that the dopamine transporter can be regulated via manipulations of kappa-opioid receptors, and that this will alter the neurochemical, and hence the behavioral effects of psychostimulant drugs such as cocaine and amphetamine. Specifically, the anatomical and pharmacological alterations in the dopamine transporter and dopamine receptors, as well as the associated behavioral changes, will be measured following chronic administration of kappa-opioid receptor agonists. We will measure receptor binding using in vitro autoradiography and homogenate binding. Dopamine transporter function will be measured using dopamine uptake and release assays, and dopamine receptor function will be evaluated using adenylyl cyclase and GTPgammaS binding assays. Behavior will be measured using locomotor activity studies. Understanding of the regulation of dopamine neurotransmission by kappa-opioid receptor agonists will provide leads to the development of a therapeutic agent for treating abuse of psychostimulant drugs. Altered dopamine receptor function has been implicated in numerous neurological disorders including schizophrenia, Parkinson's disease, Tourette's syndrome, and Huntington's chorea, in addition to psychostimulant drug abuse. Treatments for most of these syndromes involve use of agonists and antagonists at particular receptors, a state that often leads to marked side effects. Hence, the ability to regulate dopaminergic transmission via alterations of the dopamine transporter would be useful in the treatment of such syndromes. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: LONGITUDINAL STUDIES AMONG AT-RISK HD GENE CARRIERS Principal Investigator & Institution: Foroud, Tatiana M.; Associate Professor; Medical and Molecular Genetics; Indiana Univ-Purdue Univ at Indianapolis 620 Union Drive, Room 618 Indianapolis, in 462025167 Timing: Fiscal Year 2002; Project Start 01-APR-2002; Project End 31-MAR-2007 Summary: (provided by applicant) To identify and quantify changes among presymptomatic Huntington disease gene carriers who had not yet developed definite chorea, we performed the largest, study of individuals at-risk for HD (n=657) Subtle abnormalities in oculomotor, extrapyramidal and pyramidal motor, and cognitive measures were identified. We propose to reexamine this unique sample of at-risk individuals using an expanded test battery that includes more sensitive and specific quantitative measures for each subset of variables for which significant differences between presymptomatic gene carriers and nongene carriers were initially observed. These new measures increase the power of our proposed longitudinal studies of the rate

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of change among presymptomatic gene carriers as they approach the manifestation of clinically diagnosable HD. These novel studies are designed to: 1) Further delineate the deficits observed in the subclinical and early symptomatic phase of disease; 2) measure the rate of increasing abnormality among presymptomatic gene carriers; 3) investigate the interrelationships among the variables so as to identify measures with similar rates of deterioration, which might suggest common pathways affected early in the disease process; 4) quantify the relationship of CAG repeat number with disease onset and progression. The results of these studies will improve the understanding of the presymptomatic and early symptomatic phase of HD allowing for earlier diagnosis and identify subclinical biomarkers that can be utilized in clinical trials to evaluate therapeutic agents designed to slow progression and delay the onset of clinical HD. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MECHANISMS NEURODEGENERATION

OF

HUNTINGTIN-INDUCED

Principal Investigator & Institution: Finkbeiner, Steven M.; Assistant Professor; J. David Gladstone Institutes Box 419100, 365 Vermont St San Francisco, Ca 94103 Timing: Fiscal Year 2002; Project Start 01-JUL-2000; Project End 30-JUN-2004 Summary: (adapted from applicant's abstract): Huntington disease (HD) is a devastating inherited neurodegenerative disease characterized by chorea, dementia, and premature death. It is caused by a mutation within the gene IT15 that encodes an abnormally long stretch of polyglutamines (polyGlns) within the protein huntingtin (htt). The overall goal of our research is to elucidate the molecular mechanisms by which mutant htt induces neurodegeneration. We have developed a cellular model that recapitulates two key features of Huntington's disease: cultured neurons transfected with mutant htt die in a mutation (polyGln expansion)-dependent fashion and the death is cell-specific, affecting only the enkephalin-positive striatal neurons in culture. Htt is expressed widely in the nervous system, so the basis for neuron-specific degeneration in Huntington's disease is unknown. Using the model, we found evidence that mutant htt must translocate from the cytoplasm into the nucleus to trigger neurodegeneration, but the mechanisms that regulate the translocation are not known. How htt acts in the nucleus to trigger neurodegeneration is also unclear. Preliminary experiments with our model suggest that expanded polyGln tracts may alter htt to promote gene expression in a mutation-dependent and transcription factor-specific manner. However, the molecular basis for the effect of mutant htt on gene expression is unknown. To begin to address these unanswered questions, we propose to use our cellular model to accomplish the following Specific Aims: 1) Characterize the mechanisms of neuron-specific degeneration in Huntington's disease by varying independently the type of neuron in which mutant htt is expressed, the expression level of mutant htt, and the protein context in which the polyGln tract is expressed; 2) Characterize the critical processes by which htt is translocated to the nucleus by characterizing the factors that normally regulate htt localization and by mapping within the gene that encodes it, sequences that function to localize it to the cytoplasm, translocate it to the nucleus, or restrict it to sub domains of the nucleus and; 3) Identify mechanisms by which mutant htt, acting in the nucleus, could trigger neurodegeneration by elucidating the molecular mechanisms by which mutant htt potentiates gene expression mediated by the transcription factor Sp1, and by using microarray technology and inducible expression to identify the earliest changes in endogenous gene expression triggered by mutant htt. Completion of these aims should reveal new insights into general mechanisms of neurodgeneration and to the identification of potential molecular targets for new HD therapies.

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Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MECHANISMS OF STREPTOCOCCAL/HOST INTERACTION Principal Investigator & Institution: Zabriskie, John; Rockefeller University New York, Ny 100216399 Timing: Fiscal Year 2002 Summary: We have now completed our studies on the nature of the nephritogenic protein using the sera and renal biopsies from patients with acute post streptococcal glomerulonephritis (APSGN). All nephritogenic strains produce a protein called nephritis plasmin binding protein (NPBP) which was isolated, sequenced and found to be identical with streptococcal proteinase or streptococcal pyrogenic exotoxin B (SPEB). Using antibodies prepared against recombinant SPEB, 65% of 20 APSGN biopsies were found to contain the antigen in the glomerulus while only 4% of 25 non APSGN biopsies were positive. None of the biopsies were positive for streptokinase, another streptococcal antigen possibly associated with APSGN. APSGN sera reacted preferentially with this antigen when compared to sera from patients with either rheumatic fever or uncomplicated streptococcal infections and normal controls. Furthermore, serial serum studies of APSGN patients revealed that while the titers decreased over one year they never returned to baseline values suggesting a possible protective effect against the known fact that recurrences of APSGN are extremely rare. A manuscript detailing these findings has been submitted to Kidney International. Studies involving the rheumatic fever marker D8/17 have proceeded along two areas of investigation. The first is concerned with the predictive role of the marker for disease susceptibility. Approximately 3,000 children ages 5-10 years who come from high risk areas of rheumatic fever in Mexico City have been tested for the marker. Seven percent of these unaffected children are positive for the marker. All children are being followed over time for the appearance of rheumatic fever. If our hypothesis is correct, only those positive for the D8/17 marker will be susceptible. The second area of investigation of the marker was unexpected. In collaboration with a group from Child Psychiatry at NIH under the direction of Dr. Sue Swedo, we have examined the presence or absence of this marker in a group of 23 children (and appropriate controls) with obsessive-compulsive disorders (OCD). In a double blind test the marker correctly identified ninety percent of the OCD patients compared to the expected 7% controls. In view of the known crossreactions between streptococcal antigens and caudate nucleus cells, these studies suggest that other brain-streptococcal cross reactive antigens may be involved in the OCD syndrome. A manuscript detailing these findings has been sent to the J. Child Psychaitry. Our more basic approach to the exact nature of the D8/17 antigen and caudate binding antigen in the sera of Sydenham's chorea are being pursued along two main lines of investigation. Concerning the D8/17 antigen our main problem in identifying this antigen has been the fact that the antibody is the IgM class. Thus nonspecific binding of other proteins has resulted in identification of a number of bands. Secondly, this antibody does recognize other antigens expressing a coil-coiled structure. We have recently isolated a IgG clone of the D8/17 antibody and hope this will have the same specificity as the IgM molecule. We are also using the chemiluminescence technique (quite sensitive) to further identify the putative antigen. Concerning the Sydenham's caudate antigen we are screening sera from these patients both on a cDNA library of human caudate as well as immunoblots of human and mouse caudate specimens. In this respect we have identified two bands of 82 and 66 Kda that appear promising. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: APOPTOSIS

MECHANISMS

OF

TRANSLATION

CONTROL

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DURING

Principal Investigator & Institution: Lloyd, Richard; Associate Professor; Molecular Virology & Microbiol; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2002; Project Start 01-AUG-1999; Project End 31-JUL-2004 Summary: Apoptosis or programmed cell death is a highly controlled, universal cellular process of cells. Dysregulation of apoptosis has been linked to several diseases such as cancer. Alzheimers disease and Huntinton's chorea. The process of apoptosis induces translation shutoff which is likely to be mediated by cleavage of certain initiation factors and phosphorylation of other regulatory factors, and which is also likely to be crucial for the completion of cellular killing. Studies will be performed in vitro in cell extracts or with purified recombinant proteins or in vitro in tissue culture cells to elucidate the various modifications which occur in translation regulatory factors and their specific effects on translation activity during apoptosis. Efforts will focus on eukaryotic translation initiation factor 4G (eIF4G) and poly(A)binding protein (PABP) which are crucial factors which control translation initiation rates and modulate 5'-3' interactions between mRNA ends. Preliminary evidence provided shows that both eIF4G and PABP are cleaved at the time during development of apoptosis when protein synthesis is being shutoff in the cells. Experiments will determine if cleavage destroys or modulates the ability of eIF4G and PABP to function in all or only some translation reactions and/or if 5'-3' interactions are disrupted. Further, we hypothesize that cap- independent translation of crucial regulatory proteins whose mRNAs contain internal ribosomal entry sites (IRES) will be capable of continued translation after cleavage of eIF4G. IRES control elements found on certain viral and cellular genes will be subcloned into various expression vectors and tested for translational efficiencies in vitro and in vivo under various conditions of apoptosis or factor-cleavage. The long term goal is to understand how events during apoptosis lead to cell death and further our basic understanding of how protein synthesis is regulated in human cells. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: METABOTROPIC RECEPTORS IN GLOBUS PALLIDUS Principal Investigator & Institution: Poisik, Olga; Division of Neuroscience; Emory University 1784 North Decatur Road Atlanta, Ga 30322 Timing: Fiscal Year 2003; Project Start 14-DEC-2002 Summary: (provided by applicant): The primary objective of this project is to understand how metabotropic glutamate receptors (mGluRs) shape the output of the globus pallidus (GP), an intrinsic component of the basal ganglia, a group of subcortical nuclei that control motor behavior and also have cognitive functions. The activity of the GP is disrupted in some disorders of the basal ganglia such as Parkinson's disease, Hungtington's chorea, and depression. Activation of mGluRs results in important and long-lasting effects in many brain regions, which makes them attractive pharmacological targets in treating neurological disorders. The ultimate goal of this proposal is to collect the necessary information that is required to establish whether mGluRs in the GP can serve as therapeutic targets in treatment or management of neurological diseases that affect the GP output. More specifically, this work will examine the roles of mGluRs on: 1) the excitability of GP neurons, 2) the inhibitory synaptic transmission in the GP, and 3) on the desensitization mechanisms of some postsynaptic mGluRs in the GP. The functional roles of mGluRs and their desensitization mechanisms will be studied using in vitro electrophysiological

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recordings in living rat brain slices. Commercially available agonists and antagonists to all groups of mGluRs will be utilized. We intend to study the desensitization mechanisms of mGluRs in the GP because some of these receptors are known to desensitize, which limits their usefulness as therapeutic targets in treatment or management of diseased states. Overall, this work will disclose the functions of mGluRs in the GP and their useful potential in treating basal ganglia disorders. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR AND CELLULAR PHYSIOLOGY OF EPISODIC ATAXIA Principal Investigator & Institution: Maylie, James G.; Obstetrics and Gynecology; Oregon Health & Science University Portland, or 972393098 Timing: Fiscal Year 2003; Project Start 30-SEP-1998; Project End 30-NOV-2006 Summary: (provided by applicant): Episodic Ataxia / Myokymia Syndrome Type 1 (EA1) is an inherited autosomal dominant human neurological disorder. The principal symptoms of EA1 are myokymia and episodic attacks of generalized ataxia that are diminished by carbonic anhydrase inhibitors such as acetazolamide. EA1 is due to missense point mutations in KCNA1, the gene encoding the voltage-gated delayed rectifier potassium channel, Kv1.1; most mutations alter the biophysical properties of Kv1.1 channels. In the cerebellum, Kv1.1 is localized to axonal branch points and the pinceau synaptic structure of GABAergic interneurons that innervate the Purkinje cells. To understand the cellular consequences of EA1 mutations in Kv1.1 that result in ataxia, and the mechanism by which acetazolamide reduces the ataxia, ES cell-mediated homologous recombination was used to produce a transgenic mouse model of EA1 harboring the EA1 mutation V408A. In the EA1 mice, cerebellar Purkinje neurons show increased frequency and amplitude of spontaneous inhibitory postsynaptic currents (IPSCs). Two overall aspects will be investigated. First, the mouse model will be evaluated for EA1 using a battery of behavioral tests for motor coordination and the efficacy of acetazolamide. Second, the cellular basis of ataxia in EA1 mice will be examined using paired recordings from presynaptic basket cells and postsynaptic Purkinje cells in cerebellar slices, coupled with Ca2+ imaging. Four specific hypotheses will be tested: 1) Transgenic mice harboring the V408A allele provide an animal mode/for human EA1. 2) The increased frequency of spontaneous Purkinje cell IPSCs in EA1 mice results from reduced failure of action potential propagation at branch points along the basket cell axon. 3) The increased amplitude of Purkinje cell IPSCs in EA1 mice results from reduced failure of action potential propagation within the synaptic terminals of the basket cell, the pinceaus. 4) The higher GABAergic activity in EA1 basket cells predisposes the Purkinje cells to a transformation from a hyperpolarizing to depolarizing postsynaptic potential. Acetazolamide, by minimizing repair of the HCO3 gradient, reduces the depolarizing GABAA signal transformation, thus maintaining an inhibitory postsynaptic response to GABAA. These experiments will establish a link between altered electrophysiology and behavior in EA1 and demonstrate a precise connection between altered Kvl.1 channel biophysical function and the consequence of the altered channel function in neurons in rive. The use of transgenic technology to develop mouse models of disease will permit an understanding of EA1 and the possibility of new pharmacological treatments for this disease. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MOLECULAR STUDIES OF BRAIN NMDA RECEPTORS Principal Investigator & Institution: Zukin, R Suzanne.; Professor; Neuroscience; Yeshiva University 500 W 185Th St New York, Ny 10033 Timing: Fiscal Year 2002; Project Start 01-JUL-1984; Project End 30-JUN-2004 Summary: The NMDA-type glutamate receptor is implicated in long-term potentiation, memory formation, brain development, and the neurodegeneration associated with epilepsy, ischemia, Huntington's chorea, Alzheimer's disease and AIDS encephalopathy. The proposed research aims to study molecular diversity in neuronal NMDA receptors. Molecular diversity will be addressed at the following levels of resolution; amino acid residues, receptor domains, receptor variants, individual cells, and neural circuits. Experiments outlined in this proposal are directed at the development of a detailed molecular/functional/anatomic profile of NMDA receptor variants. This laboratory has cloned two new NMDA receptor splice variants from rat brain and has characterized their functional properties. The receptor variants differ in their agonist affinity, current amplitudes, and regulation by polyamines, zinc and protein kinase C(PKC). In one project, we will use site-directed mutagenesis to identify functionally important amino acid residues in the NR1 receptor protein. Recombinant NMDA receptor channels will be analyzed in Xenopus oocytes and human embryonic kidney 293 cells by whole cell recording and by patch clamp. Studies will focus on the receptor domains involved in polyamine and zinc potentiation, regulation by PKC and in binding of glycine and glutamate. Working hypotheses are as follows: 1) Positively charged residues within the N terminal insert N1 govern zinc and spermine potentiation, agonist potency, and current amplitude, thereby generating receptors with altered normal responsiveness and sensitivity to glutamate pathogenicity; 2) In NR1 receptors lacking the C1 insert, serine residues within the cytoplasmic loops play a critical role in regulation by PKC; 3) A glycine receptor-like motif within the N terminal domain forms part of the glycine binding site. 4) A glutamate binding protein (QBP)-like domain within the N terminal domain of NR1, just preceding TMI, is involved in the glutamate binding site. In a second project we will characterize NR1 heteromers with normal, mutationally altered and chimeric NR2 receptors for comparison in a situ receptors and to determine structure/activity relationships for this receptor subunit. In a third project we will define cell-specific and circuit-specific expression of NMDA receptor splice variants in the hippocampus, a brain region known to be vulnerable to glutamate toxicity. Individual hippocampal neurons grown in dissociated culture on coverslips will be analyzed electrophysiologically at the whole cell and single channel level for sensitivity to polyamines and to PKC. Subpopulations of NMDA splice variants and subtypes will be identified by in situ hybridization using exon-specific and splice junction-specific oligonucleotide probes. As a future direction, individual neurons will be analyzed for expression of splice variants by patch clamp methods and by the polymerase chain reaction. Findings from these studies are expected to aid in the development of new strategies for intervention in neurodegenerative disorders. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MYOSIN--A LINK BETWEEN STREPTOCOCCI AND HEART Principal Investigator & Institution: Cunningham, Madeleine W.; George Lynn Cross Research Professor; Microbiology and Immunology; University of Oklahoma Hlth Sciences Ctr Health Sciences Center Oklahoma City, Ok 73126 Timing: Fiscal Year 2002; Project Start 01-AUG-1989; Project End 29-FEB-2004

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Summary: Rheumatic fever is a sequela of group A streptococcal infection primarily in children. Manifestations of the disease include carditis, arthritis and chorea. Our hypothesis is that autoimmune mechanisms due to molecular mimicry between the group A streptococcus and human tissues are responsible for the disease. Our data support this hypothesis. We have identified host and streptococcal antigens which react with anti-strep/heart antibodies and T cells, and we have identified streptococcal and human cardiac myosin epitopes which produce carditis and valvulitis in animal models of disease. Despite our progress, we do not know how these crossreactive autoantibodies function in the pathogenesis of acute rheumatic fever(ARF) or the exact nature and antigenic specificities of the T cells in rheumatic carditis. Therefore, the goal and objectives propose to answer questions about the potential role of antibody in disease and to investigate the nature of the T cells which are crossreactive and appear to be responsible for valvulitis. The objectives are 1) to produce a panel of cytotoxic/crossreactive monoclonal antibodies (mAbs) from humans and transgenic mice and passively transfer IgM and IgG mAbs to test for tissue deposition in vivo; 2) to determine the nucleotide sequences of crossreactive antibody V, D, and J region genes; 3) to produce transgenic mice containing the VDJ genes(H and L) of human and mouse crossreactive and/or cytotoxic mAbs; 4) to investigate the Lewis rat model of valvulitis by producing and characterizing T cell clones from rats immunized with rM6 protein and cardiac myosin and in passive transfer experiments determine if these T cells produce disease; 5) to compare valves immunohistochemically from rheumatic carditis and Lewis rat valvulitis to identify similarities. These studies will attempt to define the steps in the pathogenesis of rheumatic carditis and will continue to support the growing body of evidence that infectious agents play a role in the development of autoimmunity in man. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: NEUROBIOLOGICAL LONGITUDINAL STUDY

MECHANISMS--PROSPECTIVE

Principal Investigator & Institution: Leckman, James F.; Nieson Harris Professor; Yale University 47 College Street, Suite 203 New Haven, Ct 065208047 Timing: Fiscal Year 2002 Summary: (Adapted from applicant's abstract): The long-term goal of this Project is to establish whether or not immunological alterations accompany an increase in clinical severity in patients with Tourette's syndrome (TS), obsessive-compulsive disorder (OCD), and attention deficit hyperactivity disorder (ADHD). It has been hypothesized that susceptible individuals develop symptoms of these disorders as a result of postinfectious autoimmune processes. Infections with group A beta-hemolytic streptococci are thought to initiate these processes. Two mechanisms have been proposed to explain the putative post-infectious autoimmunity: antigenic mimicry and superantigens (SAGs). We plan to explore each of these mechanisms by performing a prospective longitudinal study of children and adolescents at risk of symptom exacerbation. Based on pilot data reported in a Project, we plan to examine in an exploratory fashion the predictive value of rises in anticytoskeletal, antinuclear, and antineuronal antibodies for ensuing symptom exacerbations. Based on pilot data, we postulate that symptom exacerbations in susceptible individuals are associated with skewing of the T-cell receptor (TCR) V beta repertoire and changes in cytokine expression. In order to evaluate the SAG hypothesis, we will make repeated measures to determine the TCR V beta repertoire of CD4+ T cells and CD8+ T cells using flow cytometry. Patients with Sydenham's chorea are reported to have increased volumes of their basal ganglia. We

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propose to collect serial magnetic resonance imaging scans to monitor basal ganglia volumes over the course of symptom exacerbation. Patients with autoimmune disorders often share specific HLA alleles within the histocompatability complex. As in a Project, we propose to now test whether a similar sharing of alleles exists. The DR and DQ alleles for all patients will be determined. In contrast to a Project, the HLA genotypes can be examined in relation to clinical and immunological phenotypes defined over the period of the prospective longitudinal study. Phenotypic data will be obtained by direct structured psychiatric interviews of all pertinent family members with respect to the occurrence of symptom exacerbations using expert interviewers trained by the Clinical Core. If specific immunological alterations are consistently associated with acute clinical exacerbations, then the exact nature of these alterations should provide insight as to the immunologic mechanisms involved. Most importantly, this knowledge may provide a basis for the rational design of therapeutic and preventative interventions, primarily in the form of antimicrobial prophylaxis, but possibly including plasmapheresis, IVIG therapy, and future vaccination. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: DISORDERS

NEUROIMMUNOLOGY

OF

CHILDHOOD

PSYCHIATRIC

Principal Investigator & Institution: Murphy, Tanya K.; Associate Professor; Psychiatry; University of Florida Gainesville, Fl 32611 Timing: Fiscal Year 2002; Project Start 15-FEB-2001; Project End 31-JAN-2006 Summary: This is a request for a Mentored Patient-Oriented Research Career Development Award. The purpose of this award is to support the supervised research experience coupled with critical didactic training to develop the skills necessary to become an independent investigator in the area of child neuroimmunology research. An example of a neuropsychiatric illness believed to have an autoimmune etiology is Sydenham's chorea (SC). An interplay of genetic, developmental, and environmental factors determines vulnerability to this complication of a streptococcal infection. It has been hypothesized that SC may be a medical model for some forms of tic and obsessive compulsive disorders (OCD). Evidence to support an autoimmune etiology in these and other child neuropsychiatric disorders has been increasing. It is an exciting area to explore as future developments may lead to additional diagnostic and treatment approaches to these disorders. The aim of the award is to promote career development in neuroimmunology of child neuropsychiatric disorders via didactic training in these areas and conducting a research plan under the mentorship of experts in OCD and Tourette's syndrome (TS) patient-oriented research, ileuroimmunology and related laboratory techniques, and pediatric infectious disease/immunology. This multidisciplinary training is necessary to foster skills in patient-oriented research in child neuropsychiatry and the translation from basic science skills needed to study mechanisms of disease. The proposed research study will test the following hypotheses: 1) Is there, evidence of immune dysfunction in children with OCD and/or chronic tic disorders (CTD)?;and 2) Is immune dysfunction more evident during symptom exacerbations? Exploring the role of immune responses in pediatric neuropsychiatry would provide the opportunity to fully develop skills in research design and methodology, patient characterization and neuroimmunology laboratory techniques. This award, along with support from the University Florida Brain Institute and the Department of Psychiatry, will enable the candidate to build a strong foundation of research skills needed to become an independent investigator. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: NEUROPROTECTION BY GROWTH FACTORS DURING NITRIC OXIDE TO Principal Investigator & Institution: Wagner, John A.; Professor; Neurology and Neuroscience; Weill Medical College of Cornell Univ New York, Ny 10021 Timing: Fiscal Year 2002; Project Start 01-MAR-1993; Project End 31-MAR-2004 Summary: Neurodegenerative disorders, whether caused by genetic defects, trauma, or other insults, result in long term deficits that seriously compromise the quality of life. The mechanisms that lead to neurodegeneration are complex, but recurrent themes suggest different types of neurodegenerative disorders may share common mechanisms. During the previous grant period, we made significant progress in developing two ideas: 1) that NO (nitric oxide) could be a major contributor to neuron death during ischemia, and 2) that growth factors and second messenger systems could modify the sensitivity of neurons to NO and ischemia. It is becoming increasingly clear that NO may play a central role in a number of neurodegenerative diseases, including Huntington's Chorea, Parkinson's Disease, and Amyotrophic Lateral Sclerosis. Signalling systems that minimize the damage caused by NO have the potential are potential therapeutic agents. This application focuses both on the mechanism of NOtoxicity and on neuroprotective mechanisms. It builds on several observations from the previous grant period. We want to determine signaling pathways controlled by growth factors are protective and which gene products make neurons more resistant to NOtoxicity. We are also interested in determining the toxic events that are controlled by NO that lead to cell death. The specific aims of this grant are: Specific Aim 1. To determine the roles of the ras/MAP kinase and the rac pathways in protection against NO toxicity. Specific Aim 2(a). To understand the toxic effects of cGMP on hippocampal neurons. Specific Aim 2(b). To determine the importance of a cascade of events, including the production of cGMP, the activation of cGMP-gated non-selective cation channels (cGGCC), and the activation of the cGMP-dependent protein kinase in the sensitivity of hippocampal neurons to NO-toxicity and ischemia. Specific Aim 3. To isolate genes that encode proteins which confer resistance to NO toxicity by using an expression-cloning approach. Our long range goals are to understand the role of NO in ischemia and neurodegenerative disorders, to understand how growth factors and other signaling molecules can protect neurons from the toxic effects of NO, and to translate these discoveries into therapy. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: NEW CYCLIZATION METHODS AND MULTICOMPONENT COUPLINGS Principal Investigator & Institution: Montgomery, John; Professor; Chemistry; Wayne State University 656 W. Kirby Detroit, Mi 48202 Timing: Fiscal Year 2002; Project Start 01-JAN-1998; Project End 31-DEC-2002 Summary: The primary significance of the proposed research is that potentiallypowerful new synthetic methods will be developed. The specific aims include the development of several new nickel-catalyzed procedures involving cyclizations and multicomponent couplings. Considerable attention will be devoted to the development of asymmetric and solid- phase procedures. The efforts in reaction discovery are designed to develop processes that rapidly construct complex organic substructures from simple, readily-available precursors in a predictable and efficient manner. The research will focus exclusively on the development of catalytic processes that should be amenable to scaleup in an environmentally-benign fashion. A new approach to alpha-

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amino acid synthesis will be pursued which is unique that the amino acid side chain is derived from up to four components in a simple three-step operation. The rapid and flexible preparation of amino acids in the solid phase should provide a powerful combinatorial tool for small molecule drug discovery and polypeptide synthesis employing nonproteinogenic amino acids. A general synthetic approach to the allopumiliotoxin alkaloids will be developed, and a formal total synthesis of a representative member, allopumiliotoxin 339B, will be pursued. The pharmacological activity of the pumiliotoxins in linked to mechanisms involving facilitation of calcium influx into the muscle fiber and/or a facilitation of calcium release from the sarcoplasmic reticulum. The proposed synthetic route to the allopumiliotoxins should allow rapid construction of the central bicyclic skeleton with complete stereocontrol. A general approach to the kainoid amino acids will be developed, and an enantioselective total synthesis of (-)-kainic acid, the simplest member, will be pursued. Their potent neuroexcitatory activity has allowed the kainoids to serve as effective models for neuronal diseases such as epilepsy and Huntington's chorea and for neuronal cell loss in senile dementia. The proposed synthetic route to the kainoids will allow facile introduction of various C-4 side chains, which is the position at which all kainoids differ structurally. The concise and flexible routes proposed for the synthesis of allopumiliotoxin alkaloids and kainoid amino acids should allow the facile preparation of preparative quantities of the natural products as well as new derivatives for further pharmacological investigations. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: PANDAS AND STREP INFECTION: ARE THEY LINKED? Principal Investigator & Institution: Kurlan, Roger M.; Professor; Neurology; University of Rochester Orpa - Rc Box 270140 Rochester, Ny 14627 Timing: Fiscal Year 2002; Project Start 25-SEP-2001; Project End 31-JUL-2005 Summary: Sydenham's chorea is the only accepted immune-mediated central nervous system manifestation of group A beta hemolytic streptococcal (GABHS) infection. Several lines of evidence now suggest that there may be a spectrum of post-GABHS immune- mediated neurobehavioral sequelae termed "Pediatric Autoimmune Neuropsychiatric Disorders after Streptococcal Infection (PANDAS)". Tics, including Tourette's syndrome (TS), and obsessive-compulsive disorder (OCD) have been reported as the characteristic features of PANDAS. Proposed is a multicenter prospective case control cohort study involving 40 cases of PANDAS and 40 matched controls with TS and/or OCD but without evidence of PANDAS. All subjects will undergo intensive clinical and laboratory prospective observation for 24 months to determine whether antecedent GABHS infection is: 1) temporally associated with exacerbations of PANDAS, 2) specifically associated with exacerbations of tics and OCD, and 3) a specific trigger for exacerbations of PANDAS. All determinations of case/control status, GABHS infection and clinical exacerbation will be determined independently by blinded review in order to limit selection and clinical biases. Establishing a post-infectious etiology for PANDAS would dramatically change our understanding of the causes of TS and OCD, alter our therapeutic approach and may have a critical public health implication of preventing potentially fatal rheumatic cardiac sequelae in affected children. Disproving the PANDAS hypothesis would prevent the use of expensive and potentially dangerous therapies (e.g., plasma exchange, immune globulin, antibiotics) that have been proposed for PANDAS patients. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: PATHOPHYSIOLOGY OF CHOREA Principal Investigator & Institution: Mink, Jonathan W.; Associate Professor; Neurology; University of Rochester Orpa - Rc Box 270140 Rochester, Ny 14627 Timing: Fiscal Year 2002; Project Start 19-JUN-2000; Project End 31-MAY-2005 Summary: Chorea is a movement disorder that results from basal ganglia injury due to a variety of causes in children. Chorea is characterized by sudden, brief, involuntary muscle contractions causing movements that appear to flow from body part to body part in an unpredictable manner. Disorders associated with chorea include CNS infections, post-infectious and other autoimmune diseases, ischemia during cardiopulmonary bypass, 'extrapyramidal' cerebral palsy, a variety of toxic and acute metabolic processes, degenerative conditions, and inborn errors of metabolism. In many cases the cause is unknown. In diseases with well defined neuropathology, chorea has been associated with abnormalities in the striatum (caudate and putamen) and the subthalamic nucleus (STN). However, the fundamental pathophysiology of chorea is not known. This is due in part to the lack of non-primate models and in part to the difficulty of measuring involuntary movements in the primate models that do exist. Current medical treatment options for chorea are few, may have significant side effects, and are often ineffective. The proposed experiments will develop quantitative 3dimensional kinematic measures of chorea and use them measure spatial and temporal properties of chorea in adults and children with different disorders. Focal pharmacologic manipulation of basal ganglia nuclei will be used in monkeys to produce chorea. The resulting chorea will be measured in the monkeys and compared to human chorea in order to validate the monkey models, especially with respect to childhood chorea. The monkey models will then be used to investigate the fundamental pathophysiology of chorea by recording the activity of individual globus pallidus internal segment neurons before and during chorea. Through a combination of neurophysiologic and kinematic techniques to study experimentally produced chorea, the prevailing hypotheses of chorea pathophysiology can be tested rigorously. There is strong potential to identify the fundamental mechanisms of chorea. Development of a non-invasive method to quantify chorea in children and in monkeys will be an important advance toward better characterization of the pathophysiology of involuntary movements and development of more effective medical therapies. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: REGION SPECIFIC PHOSPHOPROTEINS IN THE BASAL GANGLIA Principal Investigator & Institution: Greengard, Paul; Vincent Astor Professor; Lab/Molec/Cellular Neurosci; Rockefeller University New York, Ny 100216399 Timing: Fiscal Year 2002; Project Start 01-JUL-1985; Project End 30-JUN-2005 Summary: There are major medical reasons for understanding the nature of dopaminergic neurotransmission in the neostriatum. Parkinson's disease, Huntington's chorea, schizophrenia and attention deficit hyperactivity disorder all reflect disorders of dopamine signaling pathways. This grant application proposes a multi-disciplinary study of signal Project (Biochemical Analysis of Basal Ganglia Phosphoproteins) will include: biochemical characterization of the role of phosphorylation of Th475 and DARPP-32; elucidation of the molecular basis of the interaction of neurabin. Project (Pharmacological Regulation of Basal Ganglia- enriched Phosphoproteins) will include: characterization of the phosphorylation of DARPP-32 at Th473 in intact cells; characterization of the roles of Th475 or Th434 in the regulation of glutamate and GABA receptor phosphorylation using genetically modified mice in which the Th434 and

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Th475 phosphorylation sites are substituted with alanine; characterization of the role of phosphorylation of spinophilin and neurabin in intact cells; characterization of gene knockout mice to evaluate the roles of PP1 isoforms, and neurabin in the phosphorylation of glutamate and GABA receptors. Project (Physiological and Cell Biological Studies of Phosphoproteins in the Basal Ganglia) will include: electrophysiological analysis of mice lacking either PP1 isoforms, spinophilin, neurabin, or in which the Thr34 and Thr75 phosphorylation sites in DARPP-32 will have been substituted with alanine; characterization of the regional and subcellular localization of spinophilin and neurabin; examination of the mechanism of targeting and the dynamic regulation of the spinophilin-PP1 and neurabin-PP1 complex in neurons. Project (Molecular Biology of Basal Ganglia and Phosphoproteins) will include: analysis of the behavioral and biochemical responses to dopamine in mice lacking striatal PP1 isoforms, mice lacking spinophilin and neurabin, and mice with mutated DARPP-32 genes, in which the Thr34 and Thr 75 phosphorylation sites are substituted with alanine; identification of proteins that interact with spinophilin and neurabin in yeast 2-hybrid systems. Project (Scientific Core) will produce key materials and perform routine required for the other projects. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: ROLES OF GSH1 & 2 GENES IN TELENCEPHALIC NEUROGENESIS Principal Investigator & Institution: Campbell, Kenneth J.; Assistant Professor; Children's Hospital Med Ctr (Cincinnati) 3333 Burnet Ave Cincinnati, Oh 452293039 Timing: Fiscal Year 2002; Project Start 01-JUL-2002; Project End 30-APR-2007 Summary: (provided by applicant): The telencephalon represents the region of the brain most concerned with cognition and voluntary movement. Two of the major telencephalic regions controlling these processes are the cerebral cortex and the striatum (also known as the caudate-putamen). Malfunction of these regions occurs in a number neurodegenerative disorders such as Parkinson's disease and Huntington's chorea, leading to abnormal movements and in some cases dementia. Moreover, certain neurodevelopmental disorders, such as Schizophrenia, Tourette's syndrome and autism are believed to result from malfunction and/or altered development of the cerebral cortex and striatum. Thus understanding the development of these telencephalic brain regions is likely to be of considerable importance to the diagnosis and/or treatment of these disorders.Regional development along both the anterior/posterior and dorsal/ventral axes of the developing telencephalon is known to be controlled by the spatially and temporally restricted expression of developmental control genes. Many of these developmental control genes belong to the homeobox transcription factor subclass. Two such examples are the related genes Gsh1 and Gsh2, which encode for homeobox proteins and are required for normal development of distinct subregions in the telencephalon, diencephalon and hindbrain. Recent studies have demonstrated that Gsh2 is required for the normal development of the striatum and olfactory bulb. Homozygous loss-of-function mutants for Gsh2 possess a significantly smaller striatum and reduced numbers of olfactory bulb interneurons as compared to wild-type controls. These defects are caused by a misspecification of the striatal and olfactory bulb precursors in the Gsh2 mutants at early stages of neurogenesis. The striatal and olfactory bulb neurogenesis that does occur the Gsh2 mutants is likely due to the fact that the molecular specification of striatal projection neuron and olfactory bulb interneuron precursors normalizes at late stages of neurogenesis. Our recent results have shown that this delayed normalization depends on the function of Gsh1. Although Gsh1 homozygous mutants do not display obvious defects in striatal and olfactory bulb

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neurogenesis, this gene is essential for the development of these telencephalic regions in the Gsh2 mutant telencephalon.The experiments outlined in this proposal are aimed at better understanding the specific roles of Gshl and Gsh2 in the development of the striatum and olfactory bulb interneurons. Firstly, the role that Gsh genes play in retinoid metabolism and signaling will be studied using retinoid reporter assays as well as transgenic mice in which retinoid signaling has been blocked. Secondly, the functional equivalence of the GshI and Gsh2 will be tested in knock-in experiments where Gsh2 will be replaced with GshI using gene targeting as well as in embryonic transplantation studies using mutant cells. Finally, we will determine the spatial and temporal requirements for Gsh2 in striatal and olfactory bulb development using gene targeting and conditional mutagenesis. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: STREP THROAT, SYDENHAM CHOREA, AND TOURETTE SYNDROME Principal Investigator & Institution: Mcmahon, William M.; Associate Professor of Psychiatry; Psychiatry; University of Utah Salt Lake City, Ut 84102 Timing: Fiscal Year 2002; Project Start 01-MAR-2000; Project End 28-FEB-2005 Summary: This abstract is not available. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: STRUCTURAL BASIS OF PP1 REGULATION BY SPINOPHILIN Principal Investigator & Institution: Page, Rebecca; Scripps Research Institute Tpc7 La Jolla, Ca 92037 Timing: Fiscal Year 2002; Project Start 01-SEP-2000 Summary: The production of smooth, voluntary motions is a consequence of the rapid and precise processing of incoming sensory signals into outgoing motor responses. Much of this processing occurs within specialized cells of the striatum, medium spiny neurons (MSN), whose activity is regulated by the opposing actions of glutamate (excitatory), g-aminobutyric acid (inhibitory; GABA) and dopamine (modulatory) neurotransmitters. It is the integration of these signals in MSNs which leads to precise muscle control, and imbalances in these signaling pathways are characteristic of numerous motor disorders, including Huntington's chorea and Parkinson's disease. Studies have implicated a central role for the protein serine/threonine phosphatase (PSP) protein phosphatase-l (PP1) in MSN signal integration, as it is a target of both the glutamatergic and dopaminergic signaling pathways. A novel PP1 targeting/scaffolding protein, spinophilin, which localizes PP1 to dendritic spines, has recently been identified. In addition to binding PP1, spinophilin also binds actin filaments and D2 dopamine receptors. The presence of these independent domains within a single protein makes spinophilin uniquely structured to link the PP1 phosphatase signaling cascades to the proteins of the cytoskeleton and the ion channels of the synapse, thus providing a molecular basis for postsynaptic signal integration. Crystallographic studies of the individual spinophilin domains, both alone and complexed to their binding partners, will be carried out to elucidate the structural basis of PP1 localization and activity modulation by phosphatase targeting proteins. Structural information obtained from such a study could then be used to design drugs which function to disrupt the PP1:PP1targeting subunit interactions-drugs which may be potential therapies for the motor disorders of Huntington's chorea and Parkinson's disease. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: THE NEUROPHYSIOLOGY OF SHAPE AND SPATIAL WORKING MEMORY Principal Investigator & Institution: Sereno, Anne B.; Neurobiology and Anatomy; University of Texas Hlth Sci Ctr Houston Box 20036 Houston, Tx 77225 Timing: Fiscal Year 2002; Project Start 01-APR-2001; Project End 31-MAR-2006 Summary: adapted from applicant's abstract): This proposal examines two kinds of working memory-memory for shape and space- at the cellular level using a single unit recording technique in awake behaving rhesus monkeys. Much work suggests that perceptual processing proceeds along two cortical paths in temporal and parietal cortices, often characterized as the 'what' and where pathways, respectively. Both pathways project to regions of prefrontal cortex. Previous work has not been careful in the manipulation of featural and spatial working memory requirements and no study has compared cell response to both kinds of working memory within the same cortical area with identical visual conditions. Using the proposed paradigms, we have just finished recording from posterior parietal and inferior temporal cortices of animals performing these tasks where we found quite different patterns of findings. This study will provide valuable information as to whether working memory of shape and space are physiologically segregated in prefrontal cortex. We will also be able to report exactly how these types of working memory modulate cell response and how this relates to the sensory and motor response of the cell. Further, we will be able to compare the findings directly to data we have obtained from posterior parietal and inferior temporal cortices. Prefrontal cortex has been implicated in a variety of human neurological and mental diseases, including Parkinson's disease. Huntington's chorea, Korsakoff's disease, and schizophrenia. Little work in the neuropsvchology of memory has focused on shortterm or working memory. Characterization of mechanisms related to working memory in prefrontal cortex may prove useful for better understanding what role short-term or working memory deficits play in the cognitive and psychiatric abnormalities that are associated with these conditions. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

The National Library of Medicine: PubMed One of the quickest and most comprehensive ways to find academic studies in both English and other languages is to use PubMed, maintained by the National Library of Medicine.3 The advantage of PubMed over previously mentioned sources is that it covers a greater number of domestic and foreign references. It is also free to use. If the publisher has a Web site that offers full text of its journals, PubMed will provide links to that site, as well as to sites offering other related data. User registration, a subscription fee, or some other type of fee may be required to access the full text of articles in some journals. To generate your own bibliography of studies dealing with chorea, simply go to the PubMed Web site at http://www.ncbi.nlm.nih.gov/pubmed. Type “chorea” (or synonyms) into the search box, and click “Go.” The following is the type of output you can expect from PubMed for chorea (hyperlinks lead to article summaries): 3

PubMed was developed by the National Center for Biotechnology Information (NCBI) at the National Library of Medicine (NLM) at the National Institutes of Health (NIH). The PubMed database was developed in conjunction with publishers of biomedical literature as a search tool for accessing literature citations and linking to full-text journal articles at Web sites of participating publishers. Publishers that participate in PubMed supply NLM with their citations electronically prior to or at the time of publication.

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A case of chorea-acanthocytosis with dysautonomia; quantitative autonomic deficits using CASS. Author(s): Kihara M, Nakashima H, Taki M, Takahashi M, Kawamura Y. Source: Autonomic Neuroscience : Basic & Clinical. 2002 April 18; 97(1): 42-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12036185

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A Dutch family with benign hereditary chorea of early onset: differentiation from Huntington's disease. Author(s): Hageman G, Ippel PF, van Hout MS, Rozeboom AR. Source: Clinical Neurology and Neurosurgery. 1996 May; 98(2): 165-70. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8836592

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A family of McLeod syndrome, masquerading as chorea-acanthocytosis. Author(s): Takashima H, Sakai T, Iwashita H, Matsuda Y, Tanaka K, Oda K, Okubo Y, Reid ME. Source: Journal of the Neurological Sciences. 1994 June; 124(1): 56-60. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7931422

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A neuropathological study of a case of lupus erythematosus with chorea. Author(s): Kuroe K, Kurahashi K, Nakano I, Morimatsu Y, Takemori H. Source: Journal of the Neurological Sciences. 1994 May; 123(1-2): 59-63. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8064322

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A reproducible assay of polymerase chain reaction to detect trinucleotide repeat expansion of Huntington's disease and senile chorea. Author(s): Watanabe M, Abe K, Aoki M, Kameya T, Itoyama Y, Shoji M, Ikeda M, Iizuka T, Hirai S. Source: Neurological Research. 1996 February; 18(1): 16-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8714530

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A woman who trembled, then had chorea. Author(s): Nagaoka T, Matsushita S, Nagai Y, Kobayashi K. Source: Lancet. 1998 May 2; 351(9112): 1326. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9643796

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Abnormal accumulation of tTGase products in muscle and erythrocytes of choreaacanthocytosis patients. Author(s): Melone MA, Di Fede G, Peluso G, Lus G, Di Iorio G, Sampaolo S, Capasso A, Gentile V, Cotrufo R. Source: Journal of Neuropathology and Experimental Neurology. 2002 October; 61(10): 841-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12387450

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Allele distribution of a highly polymorphic repeat on chromosome 12 in patients with symptoms of chorea and ataxia. Author(s): Zuhlke C, Knobloch O, Wagner S, Hilgers R. Source: Journal of Medical Genetics. 1995 July; 32(7): 577-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7562978

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An unusual case of chorea gravidarum. Author(s): Qasim A. Source: Postgraduate Medical Journal. 2000 June; 76(896): 374, 378-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10824060

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Anti-basal ganglia antibodies in acute and persistent Sydenham's chorea. Author(s): Church AJ, Cardoso F, Dale RC, Lees AJ, Thompson EJ, Giovannoni G. Source: Neurology. 2002 July 23; 59(2): 227-31. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12136062

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Anti-basal ganglia antibody abnormalities in Sydenham chorea. Author(s): Singer HS, Loiselle CR, Lee O, Garvey MA, Grus FH. Source: Journal of Neuroimmunology. 2003 March; 136(1-2): 154-61. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12620655

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Antibodies against neural, nuclear, cytoskeletal, and streptococcal epitopes in children and adults with Tourette's syndrome, Sydenham's chorea, and autoimmune disorders. Author(s): Morshed SA, Parveen S, Leckman JF, Mercadante MT, Bittencourt Kiss MH, Miguel EC, Arman A, Yazgan Y, Fujii T, Paul S, Peterson BS, Zhang H, King RA, Scahill L, Lombroso PJ. Source: Biological Psychiatry. 2001 October 15; 50(8): 566-77. Erratum In: Biol Psychiatry 2001 December 15; 50(12): Following 1009. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11690591

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Antibodies to AChR, synapse-organizing proteins, titin, and other muscle proteins in Morvan's fibrillary chorea. Author(s): Agius MA, Zhu S, Lee EK, Aarli JA, Kirvan C, Fairclough RH, Maselli R. Source: Annals of the New York Academy of Sciences. 1998 May 13; 841: 522-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9668285

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Anticonvulsants-induced chorea: a role for pharmacodynamic drug interaction? Author(s): Zaatreh M, Tennison M, D'Cruz O, Beach RL. Source: Seizure : the Journal of the British Epilepsy Association. 2001 December; 10(8): 596-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11792164

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Anti-nuclear antibodies in Sydenham's chorea. Author(s): Abraham S, O'Gorman M, Shulman ST. Source: Advances in Experimental Medicine and Biology. 1997; 418: 153-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9331621

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Arm chorea secondary to an unruptured giant aneurysm. Author(s): Barreiro de Madariaga LM, Sian JE, Parera IC, Micheli F. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2003 November; 18(11): 1397-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14639693

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Association of chorea and motor neuron disease. Author(s): Pradat PF, Salachas F, Lacomblez L, Patte N, Leforestier N, Gaura V, Meininger V. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2002 March; 17(2): 419-20. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11921138

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Ataxia without telangiectasia masquerading as benign hereditary chorea. Author(s): Klein C, Wenning GK, Quinn NP, Marsden CD. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1996 March; 11(2): 217-20. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8684395

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Atypical McLeod syndrome manifested as X-linked chorea-acanthocytosis, neuromyopathy and dilated cardiomyopathy: report of a family. Author(s): Malandrini A, Fabrizi GM, Truschi F, Di Pietro G, Moschini F, Bartalucci P, Berti G, Salvadori C, Bucalossi A, Guazzi G. Source: Journal of the Neurological Sciences. 1994 June; 124(1): 89-94. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7931427

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Autosomal dominant chorea-acanthocytosis with polyglutamine-containing neuronal inclusions. Author(s): Walker RH, Morgello S, Davidoff-Feldman B, Melnick A, Walsh MJ, Shashidharan P, Brin MF. Source: Neurology. 2002 April 9; 58(7): 1031-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11940688

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Baclofen therapy may be associated with chorea in Alzheimer's disease. Author(s): Crystal HA. Source: Annals of Neurology. 1990 December; 28(6): 839. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2285270

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Benefits of monitoring plasma levodopa in Parkinson's disease patients with druginduced chorea. Author(s): Sage JI, Mark MH, McHale DM, Sonsalla PK, Vitagliano D. Source: Annals of Neurology. 1991 June; 29(6): 623-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1892365

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Benign (nonparoxysmal) familial chorea of early onset: an electroneurophysiological examination of two families. Author(s): Stapert JL, Busard BL, Gabreels FJ, Renier WO, Colon EJ, Verhey FH. Source: Brain & Development. 1985; 7(1): 38-42. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3159307

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Benign hereditary chorea improved on stimulant therapy. Author(s): Friederich RL. Source: Pediatric Neurology. 1996 May; 14(4): 326-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8962590

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Benign hereditary chorea of early onset maps to chromosome 14q. Author(s): de Vries BB, Arts WF, Breedveld GJ, Hoogeboom JJ, Niermeijer MF, Heutink P. Source: American Journal of Human Genetics. 2000 January; 66(1): 136-42. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10631144

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Benign hereditary chorea or hereditary idiopathic dystonia? Author(s): Quinn N. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1993 July; 8(3): 401-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8341316

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Benign hereditary chorea. Author(s): Wheeler PG, Weaver DD, Dobyns WB. Source: Pediatric Neurology. 1993 September-October; 9(5): 337-40. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8292207

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Benign hereditary chorea. A case report. Author(s): Loosmore SJ, Wood K. Source: The British Journal of Psychiatry; the Journal of Mental Science. 1988 January; 152: 131-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3167322

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Benign hereditary chorea. Clinical and genetic aspects. Author(s): Harper PS. Source: Clinical Genetics. 1978 January; 13(1): 85-95. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=624192

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Benign hereditary chorea: clinical, genetic, and pathological findings. Author(s): Kleiner-Fisman G, Rogaeva E, Halliday W, Houle S, Kawarai T, Sato C, Medeiros H, St George-Hyslop PH, Lang AE. Source: Annals of Neurology. 2003 August; 54(2): 244-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12891678

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Benign hereditary chorea--entity or syndrome? Author(s): Schrag A, Quinn NP, Bhatia KP, Marsden CD. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2000 March; 15(2): 280-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10752577

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Benign hereditary chorea--response to steroids. Author(s): Robinson RO, Thornett CE. Source: Developmental Medicine and Child Neurology. 1985 December; 27(6): 814-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4092854

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Beta-adrenergic receptor subtypes in the basal ganglia of patients with Huntington's chorea and Parkinson's disease. Author(s): Waeber C, Rigo M, Chinaglia G, Probst A, Palacios JM. Source: Synapse (New York, N.Y.). 1991 August; 8(4): 270-80. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1656540

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Bilateral chorea associated with caudate nuclei lacunar infarcts. A case report. Author(s): Goldblatt J, White NW, Wright MG. Source: South African Medical Journal. Suid-Afrikaanse Tydskrif Vir Geneeskunde. 1989 May 6; 75(9): 443-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2718072

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Biogenic amines in the hypothalamus: effect of L-DOPA on human growth hormone levels in patients with Huntington's chorea. Author(s): Podolsky S, Leopold NA. Source: Prog Brain Res. 1973; 39: 225-35. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4275115

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Blood brain barrier destruction in hyperglycemic chorea in a patient with poorly controlled diabetes. Author(s): Iwata A, Koike F, Arasaki K, Tamaki M. Source: Journal of the Neurological Sciences. 1999 February 1; 163(1): 90-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10223418

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Brain MRI in patients with past lupus-associated chorea. Author(s): Galanaud D, Dormont D, Marsault C, Wechsler B, Piette JC. Source: Stroke; a Journal of Cerebral Circulation. 2000 December; 31(12): 3079-83. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11108776

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Brain SPECT imaging in Sydenham's chorea. Author(s): Barsottini OG, Ferraz HB, Seviliano MM, Barbieri A. Source: Brazilian Journal of Medical and Biological Research = Revista Brasileira De Pesquisas Medicas E Biologicas / Sociedade Brasileira De Biofisica. [et Al.]. 2002 April; 35(4): 431-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11960191

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Brainstem reflexes and brainstem auditory evoked responses in Huntington's chorea. Author(s): Bollen E, Arts RJ, Roos RA, van der Velde EA, Buruma OJ. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1986 March; 49(3): 313-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2937885

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Bromocriptine and Huntington's chorea. Author(s): Frattola L, Albizzatir MG, Trabucchi M. Source: Archives of Neurology. 1978 January; 35(1): 60. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=145841

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Cause and course in a series of patients with sporadic chorea. Author(s): Piccolo I, Defanti CA, Soliveri P, Volonte MA, Cislaghi G, Girotti F. Source: Journal of Neurology. 2003 April; 250(4): 429-35. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12700907

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Chorea after cardiopulmonary bypass. Author(s): Thobois S, Bozio A, Ninet J, Akhavi A, Broussolle E. Source: European Neurology. 2004; 51(1): 46-7. Epub 2003 November 21. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14639031

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Chorea and community in a nineteenth-century town. Author(s): Wexler AR. Source: Bulletin of the History of Medicine. 2002 Fall; 76(3): 495-527. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12486915

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Chorea and jaw-opening dystonia as a manifestation of NeuroBehcet's syndrome. Author(s): Revilla FJ, Racette BA, Perlmutter JS. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2000 July; 15(4): 741-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10928591

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Chorea as a presenting feature of variant Creutzfeldt-Jakob disease. Author(s): McKee D, Talbot P. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2003 July; 18(7): 837-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12815669

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Chorea as a symptom of neuroborreliosis: a case study. Author(s): Piccolo I, Thiella G, Sterzi R, Colombo N, Defanti CA. Source: Italian Journal of Neurological Sciences. 1998 August; 19(4): 235-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10933464

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Chorea as the presenting clinical feature of primary antiphospholipid syndrome in childhood. Author(s): Al-Matar M, Jaimes J, Malleson P. Source: Neuropediatrics. 2000 April; 31(2): 107-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10832588

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Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases. Author(s): Oh SH, Lee KY, Im JH, Lee MS. Source: Journal of the Neurological Sciences. 2002 August 15; 200(1-2): 57-62. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12127677

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Chorea due to nonketotic hyperglycemia. Author(s): Hamide A, Kumarsamy R, Srimannarayana J, Mathew J, Das AK. Source: Neurology India. 2002 June; 50(2): 213-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12134194

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Chorea gravidarum: a case report. Author(s): Karageyim AY, Kars B, Dansuk R, Aygun E, Unal O, Turan MC. Source: J Matern Fetal Neonatal Med. 2002 November;12(5):353-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12607770

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Chorea Huntington: a rare case with childhood onset. Author(s): Gencik M, Hammans C, Strehl H, Wagner N, Epplen JT. Source: Neuropediatrics. 2002 April; 33(2): 90-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12075490

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Chorea in a pregnant woman with rheumatic mitral stenosis. Author(s): Fam NP, Chisholm RJ. Source: The Canadian Journal of Cardiology. 2003 May; 19(6): 719-21. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12772024

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Chorea in an octogenarian. Author(s): Gilstad J, Reich SG. Source: The Neurologist. 2003 May; 9(3): 165-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12808413

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Chorea in motor neuron disease. Author(s): Miwa H, Kajimoto M, Kondo T. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2002 November; 17(6): 1397. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12465097

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Chorea Sancti Viti in Lexicon medicum anno 1696. Author(s): Rektor I. Source: Journal of Neurology. 2003 January; 250(1): 7-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12527985

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Chorea-acanthocytosis: clinical and genetic findings in three families from the Arabian peninsula. Author(s): Bohlega S, Al-Jishi A, Dobson-Stone C, Rampoldi L, Saha P, Murad H, Kareem A, Roberts G, Monaco AP. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2003 April; 18(4): 403-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12671946

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Clinical and genetic heterogeneity in benign hereditary chorea. Author(s): Breedveld GJ, Percy AK, MacDonald ME, de Vries BB, Yapijakis C, Dure LS, Ippel EF, Sandkuijl LA, Heutink P, Arts WF. Source: Neurology. 2002 August 27; 59(4): 579-84. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12196653

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Clinical, laboratory, psychiatric and magnetic resonance findings in patients with Sydenham chorea. Author(s): Faustino PC, Terreri MT, da Rocha AJ, Zappitelli MC, Lederman HM, Hilario MO. Source: Neuroradiology. 2003 July; 45(7): 456-62. Epub 2003 June 17. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12811441

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Comparison of the efficacy of carbamazepine, haloperidol and valproic acid in the treatment of children with Sydenham's chorea: clinical follow-up of 18 patients. Author(s): Pena J, Mora E, Cardozo J, Molina O, Montiel C. Source: Arquivos De Neuro-Psiquiatria. 2002 June; 60(2-B): 374-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12131934

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CSF and serum immune parameters in Sydenham's chorea: evidence of an autoimmune syndrome? Author(s): Church AJ, Dale RC, Cardoso F, Candler PM, Chapman MD, Allen ML, Klein NJ, Lees AJ, Giovannoni G. Source: Journal of Neuroimmunology. 2003 March; 136(1-2): 149-53. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12620654

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Decline in the predicted incidence of Huntington's chorea associated with systematic genetic counselling and family support. Author(s): Harper PS, Tyler A, Smith S, Jones P, Newcombe RG, McBroom V. Source: Lancet. 1981 August 22; 2(8243): 411-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6115171

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Deep brain stimulation of the internal pallidum did not improve chorea in a patient with neuro-acanthocytosis. Author(s): Wihl G, Volkmann J, Allert N, Lehrke R, Sturm V, Freund HJ. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2001 May; 16(3): 572-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11391763

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Delayed immune response in chorea-amyotrophy with spherocytosis. Author(s): Lagreze HL, Kornguth SE, Brooks BR, Levine RL. Source: Neurology. 1988 October; 38(10): 1642-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3419611

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Delayed recovery of diabetic chorea following correction of hyperglycemia. Author(s): Saleh MM, Zacks ES, Katz JS. Source: Journal of Neurology. 2002 September; 249(9): 1323-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12242567

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Delayed-onset hemidystonia and chorea following contralateral infarction of the posterolateral thalamus. A case report. Author(s): Gille M, Van den Bergh P, Ghariani S, Guettat L, Delbecq J, Depre A. Source: Acta Neurol Belg. 1996 December; 96(4): 307-11. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9008780

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Dental treatment considerations for patients with Huntington's chorea: a literature review and case report. Author(s): Feeney AW. Source: J Conn State Dent Assoc. 1985 October; 59(4): 118-23. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2934446

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Detection of Epstein-Barr virus genome in cerebrospinal fluid from a patient with acquired chorea by the polymerase chain reaction. Author(s): Tachi N, Nagata N, Chiba S, Imai S, Osato T. Source: Journal of Child Neurology. 1993 October; 8(4): 424-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8228044

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Development of chorea with lupus anticoagulant after interferon therapy. Author(s): Neau JP, Guilhot F, Boinot C, Dumas P, Tantot AM, Gil R. Source: European Neurology. 1996; 36(4): 235-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8814429

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Diabetic non-ketotic hyperglycaemia presenting as chorea--a case report. Author(s): Lim TO, Ngah BC. Source: Med J Malaysia. 1990 September; 45(3): 260-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2152091

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Diagnostic use of B-cell alloantigen D8/17 in rheumatic chorea. Author(s): Feldman BM, Zabriskie JB, Silverman ED, Laxer RM. Source: The Journal of Pediatrics. 1993 July; 123(1): 84-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8320631

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Different cerebral metabolism between parkinsonian rigidity and hyperkinesia (DID, chorea, dystonia). A PET study. Author(s): Hirato M, Horikoshi S, Kawashima Y, Satake K, Shibazaki T, Ohye C. Source: Adv Neurol. 1993; 60: 511-4. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8420180

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Differential diagnosis of facial chorea. Author(s): Kurlan R, Shoulson I. Source: Adv Neurol. 1988; 49: 225-37. Review. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2894125

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Differential labelling of UDP-N-acetylglucosamine in Huntington's-chorea fibroblasts. Author(s): Hung WY, Tourian A. Source: The Biochemical Journal. 1981 May 15; 196(2): 495-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6459085

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Digoxin-induced chorea in a child. Author(s): Sekul EA, Kaminer S, Sethi KD. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1999 September; 14(5): 877-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10495059

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Disturbance of eye movements in Huntington's chorea. Author(s): Oepen G, Clarenbach P, Thoden U. Source: Arch Psychiatr Nervenkr. 1981; 229(3): 205-13. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6452105

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DNA and Huntington's chorea. Author(s): Watt DC, Edwards J. Source: Psychological Medicine. 1984 November; 14(4): 729-32. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6242494

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Dopamine receptor properties in Parkinson's disease and Huntington's chorea evaluated by positron emission tomography using 11C-N-methyl-spiperone. Author(s): Hagglund J, Aquilonius SM, Eckernas SA, Hartvig P, Lundquist H, Gullberg P, Langstrom B. Source: Acta Neurologica Scandinavica. 1987 February; 75(2): 87-94. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2953165

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Dopamine uptake capacity of platelets from people at risk for Huntington's chorea. Author(s): McLean DR, Nihei T. Source: The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques. 1981 May; 8(2): 167-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6457680

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Dopa-resistant parkinsonism, oculomotor disturbances, chorea, mirror movements, dyspraxia, and dementia: the expanding clinical spectrum of hypoparathyroidism. A case report. Author(s): Galvez-Jimenez N, Hanson MR, Cabral J. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2000 November; 15(6): 1273-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11104223

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Dystonia and chorea in acquired systemic disorders. Author(s): Janavs JL, Aminoff MJ. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1998 October; 65(4): 43645. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9771763

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Early-onset Huntington chorea. Author(s): Karagol U, Deda G, Kukner S, Ince E. Source: European Journal of Pediatrics. 1995 September; 154(9): 752-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8582428

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Early-onset Huntington's chorea. Diagnostic clues. Author(s): Brooks DS, Murphy D, Janota I, Lishman WA. Source: The British Journal of Psychiatry; the Journal of Mental Science. 1987 December; 151: 850-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2971414

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Effect of piracetam in Huntington's chorea. Author(s): Destee A, Petit H, Warot P. Source: European Neurology. 1984; 23(2): 89-91. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6233148

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Effectiveness of risperidone in Huntington chorea patients. Author(s): Dallocchio C, Buffa C, Tinelli C, Mazzarello P. Source: Journal of Clinical Psychopharmacology. 1999 February; 19(1): 101-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9934953

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Effectiveness of sodium valproate in the treatment of Sydenham's chorea. Author(s): Daoud AS, Zaki M, Shakir R, al-Saleh Q. Source: Neurology. 1990 July; 40(7): 1140-1. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2113207

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Effects of bromocriptine in Huntington chorea. Case report. Author(s): Tsuneizumi T, Anai K, Aoba A, Yamaguchi N, Takagi H, Chishima T, Sakai T, Negishi K, Kamimura M, Takeshita T, et al. Source: Progress in Neuro-Psychopharmacology & Biological Psychiatry. 1994 July; 18(4): 823-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7938569

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Efficacy of valproic acid in the treatment of Sydenham's chorea. Author(s): Appleton RE, Jan JE. Source: Journal of Child Neurology. 1988 April; 3(2): 147. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3131415

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Efficacy of valproic acid in the treatment of Sydenham's chorea. Author(s): Steinberg A, Reifen RM, Leifer M. Source: Journal of Child Neurology. 1987 July; 2(3): 233-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3112212

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Eighty-nine-year-old man with generalised chorea and basal ganglia mineralization. Author(s): Warren JD, Kimber TE, Blumbergs PC, Thompson PD. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2001 March; 16(2): 362-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11295798

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Electron spin resonance studies of erythrocyte ghost cells in Huntington's chorea. Author(s): Beverstock GC, Pearson PL. Source: Acta Neurologica Scandinavica. 1982 May; 65(5): 413-23. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6287793

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EMD 23,448: effects of a putative dopamine autoreceptor agonist in chorea. Author(s): Newman RP, Tamminga CA, Chase TN, LeWitt PA. Source: Journal of Neural Transmission (Vienna, Austria : 1996). 1985; 61(1-2): 125-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3156964

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Erythrocyte anion transporter and antibrain immunoreactivity in choreaacanthocytosis. A contribution to etiology, genetics, and diagnosis. Author(s): Bosman GJ, Bartholomeus IG, De Grip WJ, Horstink MW. Source: Brain Research Bulletin. 1994; 33(5): 523-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8186997

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Erythrocyte membrane abnormalities in patients with amyotrophic chorea with acanthocythosis. Part 2. Abnormal degradation of membrane proteins. Author(s): Asano K, Osawa Y, Yanagisawa N, Takahashi Y, Oshima M. Source: Journal of the Neurological Sciences. 1985 May; 68(2-3): 161-73. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3159851

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Erythrocyte membrane abnormalities in patients with amyotrophic chorea with acanthocytosis. Part 1. Spin labeling studies and lipid analyses. Author(s): Oshima M, Osawa Y, Asano K, Saito T. Source: Journal of the Neurological Sciences. 1985 May; 68(2-3): 147-60. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3159850

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Ethics of a predictive test for Huntington's chorea. Author(s): Thomas S. Source: British Medical Journal (Clinical Research Ed.). 1982 May 8; 284(6326): 1383-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6462188

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Ethics of predictive testing for Huntington's chorea. Author(s): Kearns A. Source: British Medical Journal (Clinical Research Ed.). 1986 September 13; 293(6548): 695-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3092982

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Ethics of predictive testing for Huntington's chorea: the need for more information. Author(s): Craufurd DI, Harris R. Source: British Medical Journal (Clinical Research Ed.). 1986 July 26; 293(6541): 249-51. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2942216

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Evidence of genetic heterogeneity in Huntington's chorea. Author(s): Wallace DC, Hall AC. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1972 December; 35(6): 789800. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4265114

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Excess of folates in Huntington's chorea: a possible etiologic link. Author(s): Prakash R. Source: Southern Medical Journal. 1985 October; 78(10): 1268. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2931805

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Exclusion mapping of the benign hereditary chorea gene from the Huntington's disease locus: report of a family. Author(s): Yapijakis C, Kapaki E, Zournas C, Rentzos M, Loukopoulos D, Papageorgiou C. Source: Clinical Genetics. 1995 March; 47(3): 133-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7634535

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Factors influencing age at onset and duration of survival in Huntington's chorea. Author(s): Newcombe RG, Walker DA, Harper PS. Source: Annals of Human Genetics. 1981 October; 45(Pt 4): 387-96. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6211129

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Familial amyotrophic chorea with acanthocytosis. New clinical and laboratory investigations. Author(s): Gross KB, Skrivanek JA, Carlson KC, Kaufman DM. Source: Archives of Neurology. 1985 August; 42(8): 753-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4026606

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Familial benign chorea with intention tremor: a clinical entity. Author(s): Pincus JH, Chutorian A. Source: The Journal of Pediatrics. 1967 May; 70(5): 724-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4225654

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Familial chorea and myoclonus epilepsy. Author(s): Takahata N, Ito K, Yoshimura Y, Nishihori K, Suzuki H. Source: Neurology. 1978 September; 28(9 Pt 1): 913-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=99688

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Familial essential ("benign") chorea. Author(s): Bird TD, Carlson CB, Hall JG. Source: Journal of Medical Genetics. 1976 October; 13(5): 357-62. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1003446

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Familial remitting chorea, nystagmus, and cataracts. Author(s): Wheeler PG, Dobyns WB, Plager DA, Ellis FD. Source: American Journal of Medical Genetics. 1993 December 1; 47(8): 1215-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8291559

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Family break-down and stress in Huntington's chorea. Author(s): Tyler A, Harper PS, Davies K, Newcome RG. Source: Journal of Biosocial Science. 1983 April; 15(2): 127-38. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6221016

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Feeding difficulties in patients with Huntington's chorea. Author(s): Lavers A. Source: Nurs Times. 1982 June 2-8; 78(22): 920-1. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6211656

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Ferric chloride reaction in Huntington's chorea. Author(s): Ottosson JO. Source: Acta Psychiatrica Scandinavica. Supplementum. 1971; 221: 84-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4255684

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Fluoxetine-induced exacerbation of chorea in Huntington's disease? A case report. Author(s): Chari S, Quraishi SH, Jainer AK. Source: Pharmacopsychiatry. 2003 January; 36(1): 41-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12649776

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Fluphenazine decanoate in the treatment of chorea: a double-blind study. Author(s): Terrence CF. Source: Curr Ther Res Clin Exp. 1976 August; 20(2): 177-83. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=133787

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Free and conjugated CSF and plasma GABA in Huntington's chorea. Author(s): Uhlhaas S, Lange H, Wappenschmidt J, Olek K. Source: Acta Neurologica Scandinavica. 1986 October; 74(4): 261-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3028028

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Friedreich's ataxia with chorea and myoclonus caused by a compound heterozygosity for a novel deletion and the trinucleotide GAA expansion. Author(s): Zhu D, Burke C, Leslie A, Nicholson GA. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2002 May; 17(3): 585-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12112211

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From Morvan's fibrillary chorea to the "mal des ardents". Author(s): Schott B, Fischer-Perroudon C, Boucher M. Source: Journal of the History of the Neurosciences. 1996 December; 5(3): 265-73. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11618746

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From off-period dystonia to peak-dose chorea. The clinical spectrum of varying subthalamic nucleus activity. Author(s): Krack P, Pollak P, Limousin P, Benazzouz A, Deuschl G, Benabid AL. Source: Brain; a Journal of Neurology. 1999 June; 122 ( Pt 6): 1133-46. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10356065

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GABA in Huntington's chorea, Parkinsonism and schizophrenia. Author(s): Spokes EG. Source: Advances in Experimental Medicine and Biology. 1979; 123: 461-73. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=160193

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Ganglioside abnormality in amyotrophic chorea with acanthocytosis. Author(s): Gross KB, Skrivanek JA, Emeson EE. Source: Lancet. 1982 October 2; 2(8301): 772. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6125842

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Generalised chorea due to digoxin toxicity. Author(s): Mulder LJ, van der Mast RC, Meerwaldt JD. Source: British Medical Journal (Clinical Research Ed.). 1988 April 30; 296(6631): 1262. Erratum In: Br Med J (Clin Res Ed) 1988 August 20-27; 297(6647): 562. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3382897

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Generalized chorea associated with chronic subdural hematomas. Author(s): Bae SH, Vates TS Jr, Kenton EJ 3rd. Source: Annals of Neurology. 1980 October; 8(4): 449-50. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7436387

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Generalized chorea due to basal ganglia lacunar infarcts. Author(s): Sethi KD, Nichols FT, Yaghmai F. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1987; 2(1): 61-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3504261

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Generalized chorea due to bilateral small, deep cerebral infarcts. Author(s): Tabaton M, Mancardi G, Loeb C. Source: Neurology. 1985 April; 35(4): 588-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3982651

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Generalized chorea in an infant with semilobar holoprosencephaly. Author(s): Louis ED, Lynch T, Cargan AL, Fahn S. Source: Pediatric Neurology. 1995 November; 13(4): 355-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8771177

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Generalized chorea in two patients harboring the Friedreich's ataxia gene trinucleotide repeat expansion. Author(s): Hanna MG, Davis MB, Sweeney MG, Noursadeghi M, Ellis CJ, Elliot P, Wood NW, Marsden CD. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1998 March; 13(2): 339-40. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9539351

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Generalized chorea induced by nonketotic hyperglycemia. Author(s): Linazasoro G, Urtasun M, Poza JJ, Suarez JA, Marti Masso JF. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1993; 8(1): 119-20. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8419797

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Genetic aspects of Huntington's chorea: results of a national survey. Author(s): Hayden MR, Beighton P. Source: American Journal of Medical Genetics. 1982 February; 11(2): 135-41. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6461251

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Genetic counseling in Huntington's chorea. Author(s): Tyler A. Source: Birth Defects Orig Artic Ser. 1987; 23(2): 85-95. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2954595

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Genetic linkage studies in Huntington's chorea. Author(s): Volkers WS, Went LN, Vegter-Van Der Vlis M, Harper PS, Caro A. Source: Annals of Human Genetics. 1980 July; 44(Pt 1): 75-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6459755

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Genetic prediction and family structure in Huntington's chorea. Author(s): Harper PS, Sarfarazi M. Source: British Medical Journal (Clinical Research Ed.). 1985 June 29; 290(6486): 1929-31. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3159461

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Genetics of childhood disorders: XXXI. Autoimmune disorders, part 4: is Sydenham chorea an autoimmune disorder? Author(s): Loiselle CR, Singer HS. Source: Journal of the American Academy of Child and Adolescent Psychiatry. 2001 October; 40(10): 1234-6. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11589538

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Genomic imprinting: a possible mechanism for the parental origin effect in Huntington's chorea. Author(s): Reik W. Source: Journal of Medical Genetics. 1988 December; 25(12): 805-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2976840

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Genomic organization of the human galpha14 and Galphaq genes and mutation analysis in chorea-acanthocytosis (CHAC). Author(s): Rubio JP, Levy ER, Dobson-Stone C, Monaco AP. Source: Genomics. 1999 April 1; 57(1): 84-93. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10191087

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George Huntington (1850-1916) and hereditary chorea. Author(s): Lanska DJ. Source: Journal of the History of the Neurosciences. 2000 April; 9(1): 76-89. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11232352

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Glutamatergic therapy of Huntington's chorea. Author(s): Giuffra ME, Mouradian MM, Chase TN. Source: Clinical Neuropharmacology. 1992 April; 15(2): 148-51. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1350513

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Graphokinetic effect of Huntington's disease (chorea) in a blind study of handwriting. Author(s): Haydu GG, Korenyi C. Source: Act Nerv Super (Praha). 1977 July; 19 Suppl 2: 355-6. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=162166

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Growth hormone and prolactin response to bromocriptine in patients with Huntington's chorea. Author(s): Chalmers RJ, Johnson RH, Keogh HJ, Nanda RN. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1978 February; 41(2): 1359. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=24678

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Hair pulling in a patient with Sydenham's chorea. Author(s): Stein DJ, Wessels C, Carr J, Hawkridge S, Bouwer C, Kalis N. Source: The American Journal of Psychiatry. 1997 September; 154(9): 1320. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9286203

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Hemiballism and chorea in a patient with parkinsonism due to a multisystem degeneration. Author(s): Steiger MJ, Pires M, Scaravilli F, Quinn NP, Marsden CD. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1992; 7(1): 71-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1557069

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Hereditary causes of chorea in childhood. Author(s): Mathews KD. Source: Semin Pediatr Neurol. 2003 March; 10(1): 20-5. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12785744

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Hereditary late-onset chorea without significant dementia: genetic evidence for substantial phenotypic variation in Huntington's disease. Author(s): Britton JW, Uitti RJ, Ahlskog JE, Robinson RG, Kremer B, Hayden MR. Source: Neurology. 1995 March; 45(3 Pt 1): 443-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7898693

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HIV encephalitis presenting with severe generalized chorea. Author(s): Gallo BV, Shulman LM, Weiner WJ, Petito CK, Berger JR. Source: Neurology. 1996 April; 46(4): 1163-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8780114

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HLA class I and class II profiles of patients presenting with Sydenham's chorea. Author(s): Donadi EA, Smith AG, Louzada-Junior P, Voltarelli JC, Nepom GT. Source: Journal of Neurology. 2000 February; 247(2): 122-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10751115

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Hormone replacement therapy induced chorea. Author(s): Steiger MJ, Quinn NP. Source: Bmj (Clinical Research Ed.). 1991 March 30; 302(6779): 762. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1850638

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Huntington chorea is not associated with hyperactivity of nigrostriatal dopaminergic neurons: studies in postmortem tissues and in rats with kainic acid lesions. Author(s): Melamed E, Hefti F, Bird ED. Source: Neurology. 1982 June; 32(6): 640-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6211637

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Huntington's chorea and anaesthesia. Author(s): Holland R. Source: Anaesthesia and Intensive Care. 1992 May; 20(2): 256-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1534469

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Huntington's chorea. Author(s): Leng CP, Gupta K. Source: British Journal of Anaesthesia. 2001 January; 86(1): 154-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11575404

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Huntington's chorea: use of rocuronium. Author(s): Kulemeka G, Mendonca C. Source: Anaesthesia. 2001 October; 56(10): 1019. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11576133

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Huntington's disease--like 2 can present as chorea-acanthocytosis. Author(s): Walker RH, Rasmussen A, Rudnicki D, Holmes SE, Alonso E, Matsuura T, Ashizawa T, Davidoff-Feldman B, Margolis RL. Source: Neurology. 2003 October 14; 61(7): 1002-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14557581

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Hyperintense basal ganglia on T1-weighted MR images in a patient with central nervous system lupus and chorea. Author(s): Kashihara K, Nakashima S, Kohira I, Shohmori T, Fujiwara Y, Kuroda S. Source: Ajnr. American Journal of Neuroradiology. 1998 February; 19(2): 284-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9504479

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Hyperintense putamen on T1-weighted MR images in a case of chorea with hyperglycemia. Author(s): Nagai C, Kato T, Katagiri T, Sasaki H. Source: Ajnr. American Journal of Neuroradiology. 1995 June-July; 16(6): 1243-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7677016

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Hyperthyroid-induced chorea in an adolescent girl. Author(s): Pozzan GB, Battistella PA, Rigon F, Zancan L, Casara GL, Pellegrino PA, Zacchello F. Source: Brain & Development. 1992 March; 14(2): 126-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1621927

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Hyperthyroidism-associated chorea. Author(s): Sudo K, Tashiro K. Source: Lancet. 1998 July 18; 352(9123): 239. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9683244

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Hyperthyroidism-associated chorea. Author(s): Cretel E, Amoura Z, Piette JC. Source: Lancet. 1998 July 18; 352(9123): 239. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9683243

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Hyperventilation-enhanced chorea as a transient ischaemic phenomenon in a patient with moyamoya disease. Author(s): Spengos K, Tsivgoulis G, Toulas P, Vemmos K, Vassilopoulos D, Spengos M. Source: European Neurology. 2004; 51(3): 172-5. Epub 2004 April 01. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=15079090

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Hypocalcaemic chorea secondary to malabsorption. Author(s): Warren JD, Kimber TE, Thompson PD. Source: Aust N Z J Med. 1998 June; 28(3): 343. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9673747

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Iatrogenic parkinsonism in Huntington's chorea. Author(s): Moss JH, Stewart DE. Source: Canadian Journal of Psychiatry. Revue Canadienne De Psychiatrie. 1986 December; 31(9): 865-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2948633

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Identification of an expanded CAG repeat in the Huntington's disease gene (IT15) in a family reported to have benign hereditary chorea. Author(s): MacMillan JC, Morrison PJ, Nevin NC, Shaw DJ, Harper PS, Quarrell OW, Snell RG. Source: Journal of Medical Genetics. 1993 December; 30(12): 1012-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8133497

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Immune mediated chorea encephalopathy syndrome in childhood. Author(s): Hartley LM, Ng SY, Dale RC, Church AJ, Martinez A, de Sousa C. Source: Developmental Medicine and Child Neurology. 2002 April; 44(4): 273-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11995896

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Immunocytochemical studies on the basal ganglia and substantia nigra in Parkinson's disease and Huntington's chorea. Author(s): Waters CM, Peck R, Rossor M, Reynolds GP, Hunt SP. Source: Neuroscience. 1988 May; 25(2): 419-38. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2456487

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Improvement of severe trunk spasms by bilateral high-frequency stimulation of the motor thalamus in a patient with chorea-acanthocytosis. Author(s): Burbaud P, Rougier A, Ferrer X, Guehl D, Cuny E, Arne P, Gross Ch, Bioulac B. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2002 January; 17(1): 204-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11835468

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In vivo proton MR spectroscopy of chorea-ballismus in diabetes mellitus. Author(s): Lai PH, Chen PC, Chang MH, Pan HB, Yang CF, Wu MT, Li JY, Chen C, Liang HL, Chen WL. Source: Neuroradiology. 2001 July; 43(7): 525-31. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11512579

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Increase in incidence of Sydenham's chorea in Sao Paulo, Brazil. Author(s): Goldenberg J, Ferraz MB, Hilario MO, Fonseca AS, Bastos W, Sachetti S. Source: Journal of Tropical Pediatrics. 1993 June; 39(3): 192-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8326544

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Increased frequency of diabetes mellitus in patients with Huntington's chorea. Author(s): Podolsky S, Leopold NA, Sax DS. Source: Lancet. 1972 June 24; 1(7765): 1356-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4113563

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Increased membrane protein phosphorylation and anion transport activity in choreaacanthocytosis. Author(s): Olivieri O, De Franceschi L, Bordin L, Manfredi M, Miraglia del Giudice E, Perrotta S, De Vivo M, Guarini P, Corrocher R. Source: Haematologica. 1997 November-December; 82(6): 648-53. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9499662

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Increased striatal glucose consumption in Sydenham's chorea. Author(s): Weindl A, Kuwert T, Leenders KL, Poremba M, Grafin von Einsiedel H, Antonini A, Herzog H, Scholz D, Feinendegen LE, Conrad B. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1993 October; 8(4): 437-44. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8232353

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Infantile chorea following abrupt withdrawal of diazepam and pentobarbital therapy. Author(s): Patrick SJ, Snelling LK, Ment LR. Source: Journal of Toxicology. Clinical Toxicology. 1993; 31(1): 127-32. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8433409

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Infantile chorea in an infant with severe bronchopulmonary dysplasia: an EMG study. Author(s): Hadders-Algra M, Bos AF, Martijn A, Prechtl HF. Source: Developmental Medicine and Child Neurology. 1994 February; 36(2): 177-82. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8132128

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Insulin-induced hypoglycaemia does not abolish chorea. Author(s): Quinn NP, Lang AE, Marsden CD. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1982 December; 45(12): 1169-70. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6219188

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Insulin-induced hypoglycaemia does not abolish chorea. Author(s): Quinn NP, Lang AE, Marsden CD. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1982 October; 45(10): 9367. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6216326

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Intracerebral injections of kainic acid and tetanus toxin: possible models for the signs of chorea and dystonia. Author(s): McGeer PL, McGeer EG. Source: Adv Neurol. 1978; 21: 331-8. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=32748

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Intracortical inhibition of the motor cortex is normal in chorea. Author(s): Hanajima R, Ugawa Y, Terao Y, Furubayashi T, Machii K, Shiio Y, Enomoto H, Uesugi H, Mochizuki H, Kanazawa I. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1999 June; 66(6): 783-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10329756

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Involuntary movements in chorea-acanthocytosis: a comparison with Huntington's chorea. Author(s): Shibasaki H, Sakai T, Nishimura H, Sato Y, Goto I, Kuroiwa Y. Source: Annals of Neurology. 1982 September; 12(3): 311-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6215892

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Is a circulating neurotoxin involved in the pathogenesis of Huntington's chorea? Author(s): Perry TL, Yong VW, Hansen S, Foulks JG, Kish SJ. Source: Journal of the Neurological Sciences. 1985 March; 67(3): 351-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3157785

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Isoniazid and Huntington's chorea. Author(s): Roccatagliata G, Albano C. Source: The New England Journal of Medicine. 1978 February 23; 298(8): 457. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=146161

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IV amantadine improves chorea in Huntington's disease: an acute randomized, controlled study. Author(s): Lucetti C, Del Dotto P, Gambaccini G, Dell' Agnello G, Bernardini S, Rossi G, Murri L, Bonuccelli U. Source: Neurology. 2003 June 24; 60(12): 1995-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12821751

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Juvenile Huntington chorea: clinical, ultrastructural, and biochemical studies. Author(s): Goebel HH, Heipertz R, Scholz W, Iqbal K, Tellez-Nagel I. Source: Neurology. 1978 January; 28(1): 23-31. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=145549

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Juvenile Huntington's chorea. Author(s): Hansotia P, Cleeland CS, Chun RW. Source: Neurology. 1968 March; 18(3): 217-24. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4231060

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Juvenile variant of Huntington's chorea. An expression of disturbed neurotransmission. Author(s): Krishnappa D. Source: The Medical Journal of Australia. 1984 January 7; 140(1): 32-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6087096

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Kainic acid animal model predicts therapeutic agents in Huntington's chorea. Author(s): Borison RL, Diamond BI. Source: Trans Am Neurol Assoc. 1979; 104: 67-9. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=162258

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Kainic acid lesions of the striatum dissociate amphetamine and apomorphine stereotypy: similarities to Huntingdon's chorea. Author(s): Mason ST, Sanberg PR, Fibiger HC. Source: Science. 1978 July 28; 201(4353): 352-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=26976

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Kainic acid lesions of the striatum: behavioural sequalae similar to Huntington's chorea. Author(s): Mason ST, Fibiger HC. Source: Brain Research. 1978 October 27; 155(2): 313-29. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=28817

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Kluver-Bucy syndrome in Huntington's chorea. Author(s): Janati A. Source: The Journal of Nervous and Mental Disease. 1985 October; 173(10): 632-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3161995

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Lack of effect of gamma-hydroxybutyrate in Huntington's chorea. Author(s): McGeer PL, Brown WJ, Zeldowicz L. Source: Can Psychiatr Assoc J. 1977 March; 22(2): 87-9. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=140760

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Late adult onset chorea with typical pathology of Hallervorden-Spatz syndrome. Author(s): Grimes DA, Lang AE, Bergeron C. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2000 September; 69(3): 392-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10945817

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Late appearance of acanthocytes during the course of chorea-acanthocytosis. Author(s): Sorrentino G, De Renzo A, Miniello S, Nori O, Bonavita V. Source: Journal of the Neurological Sciences. 1999 March 1; 163(2): 175-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10371080

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Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome. Author(s): Reuter I, Hu MT, Andrews TC, Brooks DJ, Clough C, Chaudhuri KR. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2000 February; 68(2): 23841. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10644798

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Late-onset variant of Huntington's chorea. Author(s): Faught E, Falgout JC, Leli DA. Source: Southern Medical Journal. 1983 October; 76(10): 1266-70. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6226105

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Laurence-Moon-Biedl-Bardet syndrome with chorea. Author(s): Malik GM, Mubarik M, Khan MD, Lone BA, Kadla SA, Bhat FA. Source: J Assoc Physicians India. 1995 April; 43(4): 295-6. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8713275

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L-dopa effect in Huntington's chorea. Author(s): Fahn S. Source: The New England Journal of Medicine. 1972 August 31; 287(9): 467-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4261530

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Legal problems of Huntington's chorea tests. Author(s): Bains W. Source: Nature. 1986 July 3-9; 322(6074): 20. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2941690

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Letter: Genetic counselling in Huntington's chorea. Author(s): Caro A, Jones M, Stephens J, Evans K, Walmsley W, Randall D, Johnston A, Heald J. Source: British Medical Journal. 1976 August 14; 2(6032): 420. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=132984

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Levodopa and Huntington's chorea. Author(s): Loeb C, Roccatagliata G, La Medica G, Abbruzzese G, Albano C. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1976 October; 39(10): 95861. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=137301

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Levodopa in Huntington's chorea. Author(s): Tan BK, Leijnse-Ybema HJ, Brand HJ v D. Source: Lancet. 1972 April 22; 1(7756): 903. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4111857

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Levodopa in the presymptomatic diagnosis of Huntington's chorea. Author(s): Klawans HL Jr, Goodman RM, Paulson GW, Barbeau A. Source: Lancet. 1972 July 1; 2(7766): 49. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4113651

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Levodopa induced chorea in Meige syndrome. Author(s): Hardie RJ, Lees AJ, Stern GM. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1983 March; 46(3): 286. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6842240

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Lithium treatment of Huntington's chorea. A placebo-controlled clinical trial. Author(s): Vestergaard P, Baastrup PC, Petersson H. Source: Acta Psychiatrica Scandinavica. 1977 September; 56(3): 183-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=143188

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Long latency EMG responses in early diagnosis of Huntington's chorea. Author(s): Leblhuber F, Windhager E, Reisecker F, Rittmannsberger H. Source: European Archives of Psychiatry and Clinical Neuroscience. 1991; 241(2): 113-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1834181

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Long-term penicillin in Sydenham's chorea. Author(s): Walker K, Sprenger K, de Moor M. Source: South African Medical Journal. Suid-Afrikaanse Tydskrif Vir Geneeskunde. 1989 November 18; 76(10): 582. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2588094

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Long-term treatment of juvenile Huntington's chorea with dipropylacetic acid. Author(s): Bachman DS, Butler IJ, McKhann GM. Source: Neurology. 1977 February; 27(2): 193-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=138101

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Loss of striatal histamine H2 receptors in Huntington's chorea but not in Parkinson's disease: comparison with animal models. Author(s): Martinez-Mir MI, Pollard H, Moreau J, Traiffort E, Ruat M, Schwartz JC, Palacios JM. Source: Synapse (New York, N.Y.). 1993 November; 15(3): 209-20. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7904088

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Lupus anticoagulant and chorea. Author(s): de la Fuente-Fernandez R. Source: Neurology. 1997 August; 49(2): 639-40. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9270624

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Lupus-associated chorea in childhood. Author(s): Groothuis JR, Groothuis DR, Mukhopadhyay D, Grossman BJ, Altemeier WA. Source: Am J Dis Child. 1977 October; 131(10): 1131-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=910767

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Management of rheumatic chorea: an observational study. Author(s): Araujo AP, Padua PA, Maia Filho HS. Source: Arquivos De Neuro-Psiquiatria. 2002 June; 60(2-A): 231-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12068350

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Masseter inhibitory reflex in movement disorders. Huntington's chorea, Parkinson's disease, dystonia, and unilateral masticatory spasm. Author(s): Cruccu G, Pauletti G, Agostino R, Berardelli A, Manfredi M. Source: Electroencephalography and Clinical Neurophysiology. 1991 February; 81(1): 24-30. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1705216

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Mimicry and autoantibody-mediated neuronal cell signaling in Sydenham chorea. Author(s): Kirvan CA, Swedo SE, Heuser JS, Cunningham MW. Source: Nature Medicine. 2003 July; 9(7): 914-20. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12819778

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Minimal tissue damage after stimulation of the motor thalamus in a case of choreaacanthocytosis. Author(s): Burbaud P, Vital A, Rougier A, Bouillot S, Guehl D, Cuny E, Ferrer X, Lagueny A, Bioulac B. Source: Neurology. 2002 December 24; 59(12): 1982-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12499498

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Monoamine metabolites and neuropeptides in patients with Parkinson's disease, Huntington's chorea, Shy-Drager syndrome, and torsion dystonia. Author(s): Lindvall B, Olsson JE. Source: Adv Neurol. 1990; 53: 117-22. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1700581

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Morvan's fibrillary chorea. A case with possible manganese poisoning. Author(s): Haug BA, Schoenle PW, Karch BJ, Bardosi A, Holzgraefe M. Source: Clinical Neurology and Neurosurgery. 1989; 91(1): 53-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2538282

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Morvan's fibrillary chorea. Electrodiagnostic and in vitro microelectrode findings. Author(s): Maselli RA, Agius M, Lee EK, Bakshi N, Mandler RN, Ellis W. Source: Annals of the New York Academy of Sciences. 1998 May 13; 841: 497-500. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9668281

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Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma. Author(s): Lee EK, Maselli RA, Ellis WG, Agius MA. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1998 December; 65(6): 85762. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9854961

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Morvan's fibrillary chorea: remission after plasmapheresis. Author(s): Madrid A, Gil-Peralta A, Gil-Neciga E, Gonzalez JR, Jarrin S. Source: Journal of Neurology. 1996 April; 243(4): 350-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8965109

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Moyamoya disease presenting with chorea. Author(s): Watanabe K, Negoro T, Maehara M, Takahashi I, Nomura K, Miura K. Source: Pediatric Neurology. 1990 January-February; 6(1): 40-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2310435

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Moyamoya disease presenting with singing induced chorea. Author(s): Han SH, Kim YG, Cha SH, Chung SY. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2000 December; 69(6): 8334. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11185642

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Moyamoya syndrome in a patient with Down syndrome presenting with chorea. Author(s): Takanashi J, Sugita K, Honda A, Niimi H. Source: Pediatric Neurology. 1993 September-October; 9(5): 396-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8292217

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MRI in hemiballism due to Sydenham's chorea. Author(s): Konagaya M, Konagaya Y. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1992 March; 55(3): 238-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1564496

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Muscle CT scan findings in McLeod syndrome and chorea-acanthocytosis. Author(s): Ishikawa S, Tachibana N, Tabata KI, Fujimori N, Hayashi RI, Takahashi J, Ikeda SI, Hanyu N. Source: Muscle & Nerve. 2000 July; 23(7): 1113-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10883007

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Mutation in the CHAC gene in a family of autosomal dominant choreaacanthocytosis. Author(s): Saiki S, Sakai K, Kitagawa Y, Saiki M, Kataoka S, Hirose G. Source: Neurology. 2003 December 9; 61(11): 1614-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14663054

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Mutational spectrum of the CHAC gene in patients with chorea-acanthocytosis. Author(s): Dobson-Stone C, Danek A, Rampoldi L, Hardie RJ, Chalmers RM, Wood NW, Bohlega S, Dotti MT, Federico A, Shizuka M, Tanaka M, Watanabe M, Ikeda Y, Brin M, Goldfarb LG, Karp BI, Mohiddin S, Fananapazir L, Storch A, Fryer AE, Maddison P, Sibon I, Trevisol-Bittencourt PC, Singer C, Caballero IR, Aasly JO, Schmierer K, Dengler R, Hiersemenzel LP, Zeviani M, Meiner V, Lossos A, Johnson S, Mercado FC, Sorrentino G, Dupre N, Rouleau GA, Volkmann J, Arpa J, Lees A, Geraud G, Chouinard S, Nemeth A, Monaco AP. Source: European Journal of Human Genetics : Ejhg. 2002 November; 10(11): 773-81. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12404112

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Mutations in TITF-1 are associated with benign hereditary chorea. Author(s): Breedveld GJ, van Dongen JW, Danesino C, Guala A, Percy AK, Dure LS, Harper P, Lazarou LP, van der Linde H, Joosse M, Gruters A, MacDonald ME, de Vries BB, Arts WF, Oostra BA, Krude H, Heutink P. Source: Human Molecular Genetics. 2002 April 15; 11(8): 971-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11971878

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Myoglobinuric renal failure in Huntington's chorea. Author(s): Jankovic J. Source: Neurology. 1986 January; 36(1): 138-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2934644

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Neuro-acanthocytosis--a rare cause of chorea. Author(s): Nielsen SM, Temlett JA. Source: South African Medical Journal. Suid-Afrikaanse Tydskrif Vir Geneeskunde. 1997 July; 87(7): 897-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9259728

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Neurodegenerative disorders: George Huntington's description of hereditary chorea. Author(s): Neylan TC. Source: The Journal of Neuropsychiatry and Clinical Neurosciences. 2003 Winter; 15(1): 108. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12556581

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Neuronal activity in the globus pallidus in chorea caused by striatal lacunar infarction. Author(s): Hashimoto T, Morita H, Tada T, Maruyama T, Yamada Y, Ikeda S. Source: Annals of Neurology. 2001 October; 50(4): 528-31. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11601504

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Neuronal loss from the subthalamic nuclei in a patient with progressive chorea. Author(s): Sinard JH, Hedreen JC. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1995 May; 10(3): 305-11. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7651448

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Neuropsychiatric aspects of Sydenham's chorea: a comprehensive review. Author(s): Moore DP. Source: The Journal of Clinical Psychiatry. 1996 September; 57(9): 407-14. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9746449

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Neuropsychological and neuroradiological study of a case of early-onset Huntington's chorea. Author(s): Lenti C, Bianchini E. Source: Developmental Medicine and Child Neurology. 1993 November; 35(11): 1007-10. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8224553

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Neuropsychological functioning following fetal striatal transplantation in Huntington's chorea: three case presentations. Author(s): Philpott LM, Kopyov OV, Lee AJ, Jacques S, Duma CM, Caine S, Yang M, Eagle KS. Source: Cell Transplantation. 1997 May-June; 6(3): 203-12. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9171153

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Non-ketotic hyperglycaemic chorea: a SPECT study. Author(s): Chang MH, Li JY, Lee SR, Men CY. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1996 April; 60(4): 428-30. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8774410

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Normal CAG repeats in the Huntington gene in a family with benign familial chorea. Author(s): Meszaros K, Brucke T, Fuchs K, Gerhard E, Sieghart W, vanDer Meer CH, Aschauer HN. Source: Psychiatric Genetics. 1996 Summer; 6(2): 91-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8840396

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Observations on the dopaminergic nature of hyperthyroid chorea. Author(s): Klawans HL Jr, Shenker DM. Source: Journal of Neural Transmission (Vienna, Austria : 1996). 1972; 33(1): 73-81. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4264577

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Observations on the use of methysergide in Huntington's chorea. Author(s): Klawans HL Jr, Rubovits R, Ringel SP, Weiner WJ. Source: Neurology. 1972 September; 22(9): 929-33. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4343347

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Obsessive-compulsive and related symptoms in children and adolescents with rheumatic fever with and without chorea: a prospective 6-month study. Author(s): Asbahr FR, Negrao AB, Gentil V, Zanetta DM, da Paz JA, Marques-Dias MJ, Kiss MH. Source: The American Journal of Psychiatry. 1998 August; 155(8): 1122-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9699708

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Obsessive-compulsive disorder and rheumatic chorea: is there a connection? Author(s): Abbas S, Khanna S, Taly AB. Source: Psychopathology. 1996; 29(3): 193-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8817740

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On chorea. George Huntington, M.D. Author(s): Huntington G. Source: The Journal of Neuropsychiatry and Clinical Neurosciences. 2003 Winter; 15(1): 109-12. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12556582

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On chorea: possible neuronal mechanisms. Author(s): Kanazawa I. Source: Clinical Neurology and Neurosurgery. 1992; 94 Suppl: S100-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1320477

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On defining Sydenham's chorea: where do we draw the line? Author(s): Murphy TK, Goodman WK, Ayoub EM, Voeller KK. Source: Biological Psychiatry. 2000 May 15; 47(10): 851-7. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10807957

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On the estimation of the age at onset distribution in Huntington's chorea using the EM algorithm. Author(s): Brambilla C, Frontali M, Malaspina P, Rossi C. Source: Annals of Human Genetics. 1990 July; 54 ( Pt 3): 225-33. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2145799

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Oral contraceptive induced chorea: another condition associated with anti-basal ganglia antibodies. Author(s): Miranda M, Cardoso F, Giovannoni G, Church A. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2004 February; 75(2): 3278. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14742621

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Origin of chorea-ballism and multisystem degeneration: pathophysiological implications. Author(s): Obeso JA, Luquin MR, Herrero MT, Guridi J. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1993; 8(1): 123-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8419800

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Pallidotomy for severe generalized chorea of juvenile-onset dentatorubralpallidoluysian atrophy. Author(s): Watarai M, Hashimoto T, Yamamoto K, Matsumoto Y, Tada T, Ikeda S. Source: Neurology. 2003 November 25; 61(10): 1452-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14638982

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Paraneoplastic chorea associated with CRMP-5 neuronal antibody and lung carcinoma. Author(s): Vernino S, Tuite P, Adler CH, Meschia JF, Boeve BF, Boasberg P, Parisi JE, Lennon VA. Source: Annals of Neurology. 2002 May; 51(5): 625-30. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12112110

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Paraneoplastic chorea: case study with autopsy confirmation. Author(s): Tremont-Lukats IW, Fuller GN, Ribalta T, Giglio P, Groves MD. Source: Neuro-Oncology. 2002 July; 4(3): 192-5. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12084350

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Paraneoplastic limbic encephalitis (PLE) and chorea associated with CRMP-5 neuronal antibody. Author(s): Kinirons P, Fulton A, Keoghan M, Brennan P, Farrell MA, Moroney JT. Source: Neurology. 2003 December 9; 61(11): 1623-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14663059

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Paroxysmal choreoathetosis in a patient with idiopathic basal ganglia calcification, chorea, and dystonia. Author(s): Klein C, Vieregge P, Kompf D. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1997 March; 12(2): 254-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9087991

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Persistent chorea triggered by hyperglycemic crisis in diabetics. Author(s): Ahlskog JE, Nishino H, Evidente VG, Tulloch JW, Forbes GS, Caviness JN, Gwinn-Hardy KA. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2001 September; 16(5): 890-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11746619

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Progressive chorea and amyotrophy without acanthocytes: a new case of Fotopoulos syndrome? Author(s): Pageot N, Vial C, Remy C, Chazot G, Broussolle E. Source: Journal of Neurology. 2000 May; 247(5): 392-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10896275

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Proton magnetic resonance spectroscopy of cerebrospinal fluid in neurodegenerative disease: indication of glial energy impairment in Huntington chorea, but not Parkinson disease. Author(s): Garseth M, Sonnewald U, White LR, Rod M, Zwart JA, Nygaard O, Aasly J. Source: Journal of Neuroscience Research. 2000 June 15; 60(6): 779-82. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10861790

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Pure cerebello-olivary degeneration of Marie, Foix, and Alajouanine presenting with progressive cerebellar ataxia, cognitive decline, and chorea. Author(s): Fox SH, Nieves A, Bergeron C, Lang AE. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2003 December; 18(12): 1550-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14673899

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Putaminal petechial haemorrhage as the cause of chorea: a neuroimaging study. Author(s): Chang MH, Chiang HT, Lai PH, Sy CG, Lee SS, Lo YY. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 1997 September; 63(3): 300-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9328243

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Quantification of chorea in Huntington's disease by power spectral analysis. Author(s): Weitzman DO, Rosfnfeld C, Korenyl C, Whittier JR. Source: Dis Nerv Syst. 1976 May; 37(5): 264-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=131027

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Quantitative study of gliosis in schizophrenia and Huntington's chorea. Author(s): Stevens CD, Altshuler LL, Bogerts B, Falkai P. Source: Biological Psychiatry. 1988 October; 24(6): 697-700. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=2971402

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Recombinant interferon-alpha-induced chorea and frontal subcortical dementia. Author(s): Moulignier A, Allo S, Zittoun R, Gout O. Source: Neurology. 2002 January 22; 58(2): 328-30. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11805273

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Recombinant interferon-alpha-induced chorea and frontal subcortical dementia. Author(s): Gilbert GJ. Source: Neurology. 2002 December 10; 59(11): 1821; Author Reply 1821. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12473790

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Refractory coeliac disease, small-bowel lymphoma and chorea. Author(s): Kitiyakara T, Jackson M, Gorard DA. Source: Journal of the Royal Society of Medicine. 2002 March; 95(3): 133-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11872763

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Repeat anesthetic management of a patient with Huntington's chorea. Author(s): Mitra S, Sharma K, Arora S, Deva C, Gombar KK. Source: Canadian Journal of Anaesthesia = Journal Canadien D'anesthesie. 2001 October; 48(9): 933-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11606357

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Reversible chorea and focal dystonia in vitamin B12 deficiency. Author(s): Pacchetti C, Cristina S, Nappi G. Source: The New England Journal of Medicine. 2002 July 25; 347(4): 295. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12140313

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Reversible chorea in primary antiphospholipid syndrome. Author(s): Sunden-Cullberg J, Tedroff J, Aquilonius SM. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1998 January; 13(1): 147-9. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9452340

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Reversible dementia and chorea in a young woman with the lupus anticoagulant. Author(s): Van Horn G, Arnett FC, Dimachkie MM. Source: Neurology. 1996 June; 46(6): 1599-603. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8649556

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Reversible striatal hypermetabolism in a case of Sydenham's chorea. Author(s): Goldman S, Amrom D, Szliwowski HB, Detemmerman D, Goldman S, Bidaut LM, Stanus E, Luxen A. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1993 July; 8(3): 355-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8341301

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Rheumatic chorea. Author(s): Sudhindra BK. Source: Indian Pediatrics. 1996 January; 33(1): 47-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8772953

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Risperidone in chorea and psychosis of Huntington's disease. Author(s): Erdemoglu AK, Boratav C. Source: European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies. 2002 March; 9(2): 182-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11882064

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Successful treatment of steroid-resistant chorea associated with lupus by use of valproic acid and clonidine-HCL patch. Author(s): Song CH, Oftadeh LC, Oh C, Louie J, Yu KT. Source: Clinical Pediatrics. 1997 November; 36(11): 659-62. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9391742

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Sydenham's chorea in the age of MRI: a case report and review. Author(s): Robertson WC Jr, Smith CD. Source: Pediatric Neurology. 2002 July; 27(1): 65-7. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12160978

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Sydenham's chorea may be a risk factor for drug induced parkinsonism. Author(s): Teixeira AL, Cardoso F, Maia DP, Cunningham MC. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2003 September; 74(9): 1350-1. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12933958

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Sydenham's chorea. Author(s): Marques-Dias MJ, Mercadante MT, Tucker D, Lombroso P. Source: The Psychiatric Clinics of North America. 1997 December; 20(4): 809-20. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9443351

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Sydenham's chorea: a resurgence in the 1990s? Author(s): Ryan M, Antony JH, Grattan-Smith PJ. Source: Journal of Paediatrics and Child Health. 2000 February; 36(1): 95-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10723704

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Sydenham's chorea: clinical findings and comparison of the efficacies of sodium valproate and carbamazepine regimens. Author(s): Genel F, Arslanoglu S, Uran N, Saylan B. Source: Brain & Development. 2002 March; 24(2): 73-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11891095

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Sydenham's chorea: MRI and proton spectroscopy. Author(s): Castillo M, Kwock L, Arbelaez A. Source: Neuroradiology. 1999 December; 41(12): 943-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10639673

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Sydenham's chorea: not gone and not forgotten. Author(s): Bonthius DJ, Karacay B. Source: Semin Pediatr Neurol. 2003 March; 10(1): 11-9. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12785743

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Sydenham's chorea: risk factors and the role of prophylactic benzathine penicillin G in preventing recurrence. Author(s): Gebremariam A. Source: Annals of Tropical Paediatrics. 1999 June; 19(2): 161-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10690256

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Sydenham's chorea--clinical and evolutive characteristics. Author(s): Terreri MT, Roja SC, Len CA, Faustino PC, Roberto AM, Hilario MO. Source: Sao Paulo Medical Journal = Revista Paulista De Medicina. 2002 January 3; 120(1): 16-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11836548

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Tetrabenazine in the treatment of severe pediatric chorea. Author(s): Chatterjee A, Frucht SJ. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2003 June; 18(6): 703-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12784277

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Tetrabenazine treatment for Huntington's disease-associated chorea. Author(s): Ondo WG, Tintner R, Thomas M, Jankovic J. Source: Clinical Neuropharmacology. 2002 November-December; 25(6): 300-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12469001

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The chorea of McLeod syndrome. Author(s): Danek A, Tison F, Rubio J, Oechsner M, Kalckreuth W, Monaco AP. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2001 September; 16(5): 882-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11746618

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The high frequency of juvenile Huntington's chorea in South Africa. Author(s): Hayden MR, MacGregor JM, Saffer DS, Beighton PH. Source: Journal of Medical Genetics. 1982 April; 19(2): 94-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6210776

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The Huntington's chorea register of Tanzania. Author(s): Scrimgeour EM. Source: East Afr Med J. 1982 April; 59(4): 280-2. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6216095

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The mutation rate to Huntington's chorea. Author(s): Shaw M, Caro A. Source: Journal of Medical Genetics. 1982 June; 19(3): 161-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6213773

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The response of cells from patients with Huntington's chorea to mutagen-induced chromosome damage. Author(s): Evans HJ, Vijayalaxmi, Newton MS. Source: Annals of Human Genetics. 1982 May; 46(Pt 2): 177-85. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6214203

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Treatment of Huntington's chorea with sulpiride. Author(s): Knowling MR, Wrench W. Source: South African Medical Journal. Suid-Afrikaanse Tydskrif Vir Geneeskunde. 1991 February 2; 79(3): 169. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1825239

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Treatment of Sydenham chorea with corticosteroids. Author(s): Cardoso F, Maia D, Cunningham MC, Valenca G. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2003 November; 18(11): 1374-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14639684

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Two cases of hyperglycemic chorea in diabetic patients. Author(s): Higa M, Kaneko Y, Inokuchi T. Source: Diabetic Medicine : a Journal of the British Diabetic Association. 2004 February; 21(2): 196-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=14984460

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Ubiquitin-reactive neurites in cerebral cortex of subjects with Huntington's chorea: a pathological correlate of dementia? Author(s): Cammarata S, Caponnetto C, Tabaton M. Source: Neuroscience Letters. 1993 June 25; 156(1-2): 96-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=8414198

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Ultrastructural changes of erythrocyte membrane skeletons in chorea-acanthocytosis and McLeod syndrome revealed by the quick-freezing and deep-etching method. Author(s): Terada N, Fujii Y, Ueda H, Kato Y, Baba T, Hayashi R, Ohno S. Source: Acta Haematologica. 1999 March; 101(1): 25-31. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10085435

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Uptake of 14C-dopamine in platelets of Huntington's chorea patients and symptomfree offspring. Author(s): Bonilla E, Vargas-Urribari N, Navas F. Source: Lancet. 1978 July 15; 2(8081): 161-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=78368

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Uptake of 5-hydroxytryptamine by blood platelets in Huntington's chorea and Alzheimer type of presenile dementia. Author(s): Tukiainen E, Wikstrom J, Kilpelainen H. Source: Med Biol. 1981 April; 59(2): 116-20. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6458746

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Uptake of dopamine and 5-hydroxytryptamine by platelets from patients with Huntington's chorea. Author(s): McLean DR, Nihei T. Source: Lancet. 1977 January 29; 1(8005): 249-50. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=64769

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Urinary excretion of some monoamines and metabolites in Huntington's chorea. Author(s): McNamee B, Kelvin AS, Turnbull MJ. Source: Scott Med J. 1971 May; 16(5): 247-9. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4253483

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Use of L-dopa in the detection of presymptomatic Huntington's chorea. Author(s): Klawans HL Jr, Paulson GW, Ringel SP, Barbeau A. Source: The New England Journal of Medicine. 1972 June 22; 286(25): 1332-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4260358

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Usefulness of echocardiography in detection of subclinical carditis in acute rheumatic polyarthritis and rheumatic chorea. Author(s): Agarwal PK, Misra M, Sarkari NB, Gupta AK, Agarwal P. Source: J Assoc Physicians India. 1998 November; 46(11): 937-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11229218

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Valproic acid in Sydenham's chorea. Author(s): McLachlan RS. Source: British Medical Journal (Clinical Research Ed.). 1981 July 25; 283(6286): 274-5. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6788287

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Valproic acid in the treatment of Sydenham chorea. Author(s): Alvarez LA, Novak G. Source: Pediatric Neurology. 1985 September-October; 1(5): 317-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3939747

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Vascular chorea: case report with pathology. Author(s): Bhatia KP, Lera G, Luthert PJ, Marsden CD. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 1994 July; 9(4): 447-50. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7969213

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Vesico-urethral function in Huntington's chorea. Author(s): Wheeler JS, Sax DS, Krane RJ, Siroky MB. Source: British Journal of Urology. 1985 February; 57(1): 63-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=3155979

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Viral encephalitic pathogenesis of Huntington's chorea? Author(s): Averback P. Source: Can Med Assoc J. 1979 October 20; 121(8): 1060, 1062. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=161725

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Visual (VEP) and somatosensory (SSEP) evoked potentials in Huntington's chorea. Author(s): Oepen G, Doerr M, Thoden U. Source: Electroencephalography and Clinical Neurophysiology. 1981 June; 51(6): 666-70. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6165568

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Visuomotor control of leg tracking in patients with Parkinson's disease or chorea. Author(s): Yanagisawa N, Fujimoto S, Tanaka R. Source: Adv Neurol. 1983; 39: 883-8. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6660126

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Vitamin E in treatment of Huntington's chorea. Author(s): Caro A, Caro S. Source: British Medical Journal. 1978 April 1; 1(6116): 859. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=147721

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Vitamin E in treatment of Huntington's chorea. Author(s): Caro AJ, Caro S. Source: British Medical Journal. 1978 January 21; 1(6106): 153. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=145890

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Vocal tics in Sydenham's chorea. Author(s): Mercadante MT, Campos MC, Marques-Dias MJ, Miguel EC, Leckman J. Source: Journal of the American Academy of Child and Adolescent Psychiatry. 1997 March; 36(3): 305-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9055509

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Walk a dark vale-Huntington's chorea. Author(s): Lane CS. Source: Nurs Care. 1976 May; 9(5): 30-1. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=131307

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CHAPTER 2. NUTRITION AND CHOREA Overview In this chapter, we will show you how to find studies dedicated specifically to nutrition and chorea.

Finding Nutrition Studies on Chorea The National Institutes of Health’s Office of Dietary Supplements (ODS) offers a searchable bibliographic database called the IBIDS (International Bibliographic Information on Dietary Supplements; National Institutes of Health, Building 31, Room 1B29, 31 Center Drive, MSC 2086, Bethesda, Maryland 20892-2086, Tel: 301-435-2920, Fax: 301-480-1845, E-mail: [email protected]). The IBIDS contains over 460,000 scientific citations and summaries about dietary supplements and nutrition as well as references to published international, scientific literature on dietary supplements such as vitamins, minerals, and botanicals.4 The IBIDS includes references and citations to both human and animal research studies. As a service of the ODS, access to the IBIDS database is available free of charge at the following Web address: http://ods.od.nih.gov/databases/ibids.html. After entering the search area, you have three choices: (1) IBIDS Consumer Database, (2) Full IBIDS Database, or (3) Peer Reviewed Citations Only. Now that you have selected a database, click on the “Advanced” tab. An advanced search allows you to retrieve up to 100 fully explained references in a comprehensive format. Type “chorea” (or synonyms) into the search box, and click “Go.” To narrow the search, you can also select the “Title” field.

4

Adapted from http://ods.od.nih.gov. IBIDS is produced by the Office of Dietary Supplements (ODS) at the National Institutes of Health to assist the public, healthcare providers, educators, and researchers in locating credible, scientific information on dietary supplements. IBIDS was developed and will be maintained through an interagency partnership with the Food and Nutrition Information Center of the National Agricultural Library, U.S. Department of Agriculture.

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The following information is typical of that found when using the “Full IBIDS Database” to search for “chorea” (or a synonym): •

Induction of chorea and dystonia in parkinsonian primates. Author(s): Department of Cell and Structural Biology, University of Manchester, England. Source: Boyce, S Clarke, C E Luquin, R Peggs, D Robertson, R G Mitchell, I J Sambrook, M A Crossman, A R Mov-Disord. 1990; 5(1): 3-7 0885-3185

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Successful treatment of age-related chorea with sodium valproate. Author(s): Neurobehavior Center, Beth Israel Medical Center, New York, NY 10003. Source: Hoffman, A S Feinberg, T E J-Am-Geriatr-Soc. 1990 January; 38(1): 56-8 00028614

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Therapeutics in retrospect: iron carbonate in chorea and tic douloureux. Author(s): Department of Medicine, University of Natal and Wentworth Hospital, Durban, South Africa. Source: Cosnett, J E J-R-Soc-Med. 1990 June; 83(6): 390-1 0141-0768

Federal Resources on Nutrition In addition to the IBIDS, the United States Department of Health and Human Services (HHS) and the United States Department of Agriculture (USDA) provide many sources of information on general nutrition and health. Recommended resources include: •

healthfinder®, HHS’s gateway to health information, including diet and nutrition: http://www.healthfinder.gov/scripts/SearchContext.asp?topic=238&page=0

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The United States Department of Agriculture’s Web site dedicated to nutrition information: www.nutrition.gov

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The Food and Drug Administration’s Web site for federal food safety information: www.foodsafety.gov

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The National Action Plan on Overweight and Obesity sponsored by the United States Surgeon General: http://www.surgeongeneral.gov/topics/obesity/

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The Center for Food Safety and Applied Nutrition has an Internet site sponsored by the Food and Drug Administration and the Department of Health and Human Services: http://vm.cfsan.fda.gov/

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Center for Nutrition Policy and Promotion sponsored by the United States Department of Agriculture: http://www.usda.gov/cnpp/

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Food and Nutrition Information Center, National Agricultural Library sponsored by the United States Department of Agriculture: http://www.nal.usda.gov/fnic/

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Food and Nutrition Service sponsored by the United States Department of Agriculture: http://www.fns.usda.gov/fns/

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Additional Web Resources A number of additional Web sites offer encyclopedic information covering food and nutrition. The following is a representative sample: •

AOL: http://search.aol.com/cat.adp?id=174&layer=&from=subcats

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Family Village: http://www.familyvillage.wisc.edu/med_nutrition.html

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Google: http://directory.google.com/Top/Health/Nutrition/

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Healthnotes: http://www.healthnotes.com/

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Open Directory Project: http://dmoz.org/Health/Nutrition/

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Yahoo.com: http://dir.yahoo.com/Health/Nutrition/

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WebMDHealth: http://my.webmd.com/nutrition

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WholeHealthMD.com: http://www.wholehealthmd.com/reflib/0,1529,00.html

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CHAPTER 3. ALTERNATIVE MEDICINE AND CHOREA Overview In this chapter, we will begin by introducing you to official information sources on complementary and alternative medicine (CAM) relating to chorea. At the conclusion of this chapter, we will provide additional sources.

National Center for Complementary and Alternative Medicine The National Center for Complementary and Alternative Medicine (NCCAM) of the National Institutes of Health (http://nccam.nih.gov/) has created a link to the National Library of Medicine’s databases to facilitate research for articles that specifically relate to chorea and complementary medicine. To search the database, go to the following Web site: http://www.nlm.nih.gov/nccam/camonpubmed.html. Select “CAM on PubMed.” Enter “chorea” (or synonyms) into the search box. Click “Go.” The following references provide information on particular aspects of complementary and alternative medicine that are related to chorea: •

A comparison of the information processing deficits of patients with Huntington's chorea and Korsakoff's syndrome. Author(s): Butters N, Tarlow S, Cermak LS, Sax D. Source: Cortex. 1976 June; 12(2): 134-44. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=133786

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A medieval dance craze. Was twisting a disease? Author(s): Koerner BI. Source: U.S. News & World Report. 1999 December 20; 127(24): 61. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10724816

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An EEG investigation of decamethrin-induced choreoathetosis in the rat. Author(s): Ray DE.

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Source: Experimental Brain Research. Experimentelle Hirnforschung. Experimentation Cerebrale. 1980 January; 38(2): 221-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7358105 •

Antibodies to human caudate nucleus neurons in Huntington's chorea. Author(s): Husby G, Li L, Davis LE, Wedege E, Kokmen E, Williams RC Jr. Source: The Journal of Clinical Investigation. 1977 May; 59(5): 922-32. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=140183

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Applications of transcranial magnetic stimulation in movement disorders. Author(s): Cantello R. Source: Journal of Clinical Neurophysiology : Official Publication of the American Electroencephalographic Society. 2002 August; 19(4): 272-93. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12436085

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Athetoid and choreiform hyperkinesias produced by caudate lesions in the cat. Author(s): Liles SL, Davis GD. Source: Science. 1969 April 11; 164(876): 195-7. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4886675

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Basal ganglia injury as a complication of the ketogenic diet. Author(s): Erickson JC, Jabbari B, Difazio MP. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2003 April; 18(4): 448-51. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12671955

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Cannabis in movement disorders. Author(s): Muller-Vahl KR, Kolbe H, Schneider U, Emrich HM. Source: Forschende Komplementarmedizin. 1999 October; 6 Suppl 3: 23-7. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10627163

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Cerebellar ataxia. Author(s): Perlman SL. Source: Current Treatment Options in Neurology. 2000 May; 2(3): 215-224. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11096749

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Chorea, eosinophilia, and lupus anticoagulant associated with acute lymphoblastic leukemia. Author(s): Schiff DE, Ortega JA. Source: Pediatric Neurology. 1992 November-December; 8(6): 466-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1476578

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Diagnostic tests for choreoacanthocytosis. Author(s): Feinberg TE, Cianci CD, Morrow JS, Pehta JC, Redman CM, Huima T, Koroshetz WJ. Source: Neurology. 1991 July; 41(7): 1000-6. Erratum In: Neurology 1991 October; 41(10): 1701-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1829792

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Familial paroxysmal dystonic choreoathetosis: clinical findings in a large Japanese family and genetic linkage to 2q. Author(s): Matsuo H, Kamakura K, Saito M, Okano M, Nagase T, Tadano Y, Kaida K, Hirata A, Miyamoto N, Masaki T, Nakamura R, Motoyoshi K, Tanaka H, Tsuji S. Source: Archives of Neurology. 1999 June; 56(6): 721-6. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10369313

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Huntington's chorea: striking the right chord. Author(s): Hoskyns S. Source: Nurs Mirror. 1982 June 2; 154(22): 14-7. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=6211657

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HVA and 5HIAA CSF measurements and 5HTP trials in some patients with involuntary movements. Author(s): Guilleminault C, Tharp BR, Cousin D. Source: Journal of the Neurological Sciences. 1973 April; 18(4): 435-41. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4540626

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Hyperbaric oxygen for carbon monoxide poisoning-induced delayed neuropsychiatric sequelae. Author(s): Lee HF, Mak SC, Chi CS, Hung DZ. Source: Zhonghua Yi Xue Za Zhi (Taipei). 2001 May; 64(5): 310-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11499342

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Learning to live at risk for Huntington's disease. Author(s): Hunt V, Walker FO. Source: The Journal of Neuroscience Nursing : Journal of the American Association of Neuroscience Nurses. 1991 June; 23(3): 179-82. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1831483

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Neurological manifestations of kava intoxication. Author(s): Spillane PK, Fisher DA, Currie BJ. Source: The Medical Journal of Australia. 1997 August 4; 167(3): 172-3. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=9269278

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Neurophysiological study of facial chorea in patients with Huntington's disease. Author(s): Munoz E, Cervera A, Valls-Sole J. Source: Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology. 2003 July; 114(7): 1246-52. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12842721

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New approaches in the management of hyperkinetic movement disorders. Author(s): Fahn S. Source: Advances in Experimental Medicine and Biology. 1977; 90: 157-73. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=303860

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Obsessive compulsive disorder: is there an association with childhood streptococcal infections and altered immune function? Author(s): Murphy TK, Petitto JM, Voeller KK, Goodman WK. Source: Semin Clin Neuropsychiatry. 2001 October; 6(4): 266-76. Review. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=11607922

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Organic lead encephalopathy: behavioral change and movement disorder following gasoline inhalation. Author(s): Goldings AS, Stewart RM. Source: The Journal of Clinical Psychiatry. 1982 February; 43(2): 70-2. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=7056707

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Paroxysmal choreoathetosis precipitated by movement, sound and photic stimulation in a case of arterio-venous malformation in the parietal lobe. Author(s): Shintani S, Shiozawa Z, Tsunoda S, Shiigai T. Source: Clinical Neurology and Neurosurgery. 1991; 93(3): 237-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1660380

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Paroxysmal dystonic choreoathetosis. A family study and review of the literature. Author(s): Richards RN, Barnett HJ. Source: Neurology. 1968 May; 18(5): 461-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=5691173

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Relicts of dancing mania: the dancing procession of Echternach. Author(s): Krack P. Source: Neurology. 1999 December 10; 53(9): 2169-72. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=10599799

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Scalp acupuncture in china. Author(s): Wen W.

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Source: The American Journal of Chinese Medicine. 1977 Spring; 5(1): 101-4. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=848454 •

Scalp needle therapy--acupuncture treatment for central nervous system disorders. Author(s): Liu TH, Sadove MS. Source: The American Journal of Chinese Medicine. 1974 July; 2(3): 261-9. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4415686

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Self-help groups: association to combat Huntington's chorea. Author(s): Jones IH. Source: Nurs Times. 1979 November 8; 75(45): 1949. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=160034

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St. Vitus' dance and rheumatic disease. Author(s): Schechter DC. Source: N Y State J Med. 1975 June; 75(7): 1091-102. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1095979

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Successful hypnotherapy for anxiety neurosis in Huntington's chorea. Author(s): Vann D. Source: The Medical Journal of Australia. 1971 July 17; 2(3): 166. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=4255396

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The effect of hydroxyethyl substituted rutosides on the beta,beta'iminodipropionitrile induced retinopathy in the rat. Author(s): Paterson RA, Heath H. Source: Br J Exp Pathol. 1968 June; 49(3): 283-7. No Abstract Available. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=5665441

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The neurobehavioural effects of kava. Author(s): Cairney S, Maruff P, Clough AR. Source: The Australian and New Zealand Journal of Psychiatry. 2002 October; 36(5): 65762. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=12225450

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The primary dystonias of childhood. Author(s): Moskowitz CB. Source: The Journal of Neuroscience Nursing : Journal of the American Association of Neuroscience Nurses. 1991 June; 23(3): 175-8. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=A bstract&list_uids=1831482

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Additional Web Resources A number of additional Web sites offer encyclopedic information covering CAM and related topics. The following is a representative sample: •

Alternative Medicine Foundation, Inc.: http://www.herbmed.org/

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AOL: http://search.aol.com/cat.adp?id=169&layer=&from=subcats

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Chinese Medicine: http://www.newcenturynutrition.com/

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drkoop.com: http://www.drkoop.com/InteractiveMedicine/IndexC.html

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Family Village: http://www.familyvillage.wisc.edu/med_altn.htm

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Google: http://directory.google.com/Top/Health/Alternative/

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Healthnotes: http://www.healthnotes.com/

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MedWebPlus: http://medwebplus.com/subject/Alternative_and_Complementary_Medicine

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Open Directory Project: http://dmoz.org/Health/Alternative/

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HealthGate: http://www.tnp.com/

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WebMDHealth: http://my.webmd.com/drugs_and_herbs

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WholeHealthMD.com: http://www.wholehealthmd.com/reflib/0,1529,00.html

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Yahoo.com: http://dir.yahoo.com/Health/Alternative_Medicine/

The following is a specific Web list relating to chorea; please note that any particular subject below may indicate either a therapeutic use, or a contraindication (potential danger), and does not reflect an official recommendation: •

Alternative Therapy Curative Eurhythmy Alternative names: curative eurythmy therapeutic eurhythmy Source: The Canoe version of A Dictionary of Alternative-Medicine Methods, by Priorities for Health editor Jack Raso, M.S., R.D. Hyperlink: http://www.canoe.ca/AltmedDictionary/c.html

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Herbs and Supplements Skullcap Source: The Canadian Internet Directory for Holistic Help, WellNet, Health and Wellness Network; www.wellnet.ca

General References A good place to find general background information on CAM is the National Library of Medicine. It has prepared within the MEDLINEplus system an information topic page

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dedicated to complementary and alternative medicine. To access this page, go to the MEDLINEplus site at http://www.nlm.nih.gov/medlineplus/alternativemedicine.html. This Web site provides a general overview of various topics and can lead to a number of general sources.

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CHAPTER 4. PATENTS ON CHOREA Overview Patents can be physical innovations (e.g. chemicals, pharmaceuticals, medical equipment) or processes (e.g. treatments or diagnostic procedures). The United States Patent and Trademark Office defines a patent as a grant of a property right to the inventor, issued by the Patent and Trademark Office.5 Patents, therefore, are intellectual property. For the United States, the term of a new patent is 20 years from the date when the patent application was filed. If the inventor wishes to receive economic benefits, it is likely that the invention will become commercially available within 20 years of the initial filing. It is important to understand, therefore, that an inventor’s patent does not indicate that a product or service is or will be commercially available. The patent implies only that the inventor has “the right to exclude others from making, using, offering for sale, or selling” the invention in the United States. While this relates to U.S. patents, similar rules govern foreign patents. In this chapter, we show you how to locate information on patents and their inventors. If you find a patent that is particularly interesting to you, contact the inventor or the assignee for further information. IMPORTANT NOTE: When following the search strategy described below, you may discover non-medical patents that use the generic term “chorea” (or a synonym) in their titles. To accurately reflect the results that you might find while conducting research on chorea, we have not necessarily excluded non-medical patents in this bibliography.

Patents on Chorea By performing a patent search focusing on chorea, you can obtain information such as the title of the invention, the names of the inventor(s), the assignee(s) or the company that owns or controls the patent, a short abstract that summarizes the patent, and a few excerpts from the description of the patent. The abstract of a patent tends to be more technical in nature, while the description is often written for the public. Full patent descriptions contain much more information than is presented here (e.g. claims, references, figures, diagrams, etc.). We

5Adapted

from the United States Patent and Trademark Office: http://www.uspto.gov/web/offices/pac/doc/general/whatis.htm.

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will tell you how to obtain this information later in the chapter. The following is an example of the type of information that you can expect to obtain from a patent search on chorea: •

Fatty acid treatment Inventor(s): Vaddadi; Krishna S (Clayton, AU) Assignee(s): Scotia Holdings Plc (GB) Patent Number: 5,837,731 Date filed: September 26, 1996 Abstract: A method of treating Huntington's Chorea by the administration of effective amounts of GLA or DGLA and a method of delaying the onset of Huntington's Chorea or preventing it altogether by administering appropriate amounts of GLA or DGLA to clinically normal individuals who are genetic carriers of the disease, and a method of preparation of medications for such purpose using such acids. Excerpt(s): The invention relates to treatment of Huntington's Chorea (HC). The genetic basis for HC has been identified and tests are now available to identify those carriers who will at some time in the future become ill. Much is also known about the neuropathology. In the region of the brain area known as the striatum, specific neurons which employ acetyl choline or gamma-amino-butyric acid (GABA) as their transmitters degenerate. This is followed by widespread loss of neurons in other parts of the brain region known as the basal ganglia where the striatum is found, and also throughout the cerebral cortex. There are no specific treatments for the disease. Drugs which are used in schizophrenia and which interfere with dopamine receptor action may help to sedate psychiatric patients with HC and also reduce the abnormal movements. Most individuals with HC end up in specialised hospital or nursing units where they stay an average of around seven years before death occurs. Web site: http://www.delphion.com/details?pn=US05837731__

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Method and composition for the treatment of apathy-amotivation syndrome Inventor(s): Kaminski; Ram (New York, NY) Assignee(s): The Mount Sinai School of Medicine of the City of New York (New York, NY) Patent Number: 5,453,428 Date filed: September 7, 1993 Abstract: In accordance with the present invention, neuropsychiatric symptoms of apathy-amotivation syndrome and particularly those symptoms in: Alzheimer's disease, multiple sclerosis, Huntington's chorea, frontal lobe lesions, and AIDS dementia can be ameliorated by treating a patient with a histamine H.sub.2 -antagonist that passes the blood-brain barrier. Suitable H.sub.2 -antagonists include famotidine and ranitidine. The H.sub.2 -antagonists may be co-administered with other compounds which are known to be useful in the treatment of the above neuropsychiatric conditions, and in one aspect of the invention can be formulated with such other compounds into a therapeutic composition. Excerpt(s): This application relates to a method and composition for use in the treatment of apathy-amotivation syndrome (also known as abulia or anergia syndrome) in

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neuropsychiatric disorders. The syndrome of apathy is defined as absence of or decrease in motivation (Marin, Differential Diagnosis and Classification of Apathy, American Journal of Psychiatry 147: 22-30, Jan. 1990). Slowness of thinking and diminished ability to shift from one set of thinking to the other. This can occur in a variety of apparently unrelated neuropsychiatric disorders. Some of the most common conditions that result in apathy-amotivation syndrome are: schizophrenia, Parkinson's disease, Alzheimer's disease, multiple sclerosis, Huntington's chorea and frontal lobe lesions. (Marin, Apathy: A Neuropsychiatric Syndrome, Journal of Neuropsychiatry and Clinical Neurosciences, 3: 243-254, 1991). In each case apathy-amotivation is just one of a plurality of symptoms associated with the disease. In the past, treatments of other aspects of these conditions such as motor and sensory deficits, dementia, and depression have little or no benefit with respect to apathy-amotivation syndrome. There is large body of evidence that describe apathy-amotivation syndrome and its manifestation in neuropsychiatric disorders. (Starkstein, et al., Reliability, Validity, and Clinical Correlates of Apathy in Parkinson's Disease, Journal of Neuropsychiatry, Volume 4, Number 2, Spring 1992; Bozzola, et al., Personality Changes in Alzheimer's Disease, Arch Neurol, Volume 49, March 1992; Burns, et al., Clinical Assessment of Irritability, Aggression, and Apathy in Huntington and Alzheimer Disease, The Journal of Nervous and Mental Disease Vol. 178, No. 1; Mendez, et al., Neurobehavioral changes associated with caudate lesions, Neurology 39, Mar. 1989; Robinson, et al., Mood Disorders in Stroke Patients, Brain (1984); House, et al., Mood Disorders in the Year after First Stroke, British Journal of Psychiatry (1991); Benson, et al., Psychiatric Aspects of Neurological Disease, Personality Changes With Frontal and Temporal Lobe Lesions, Grune & Stratton, Inc., 1975; Cummings, Clinical Neuropsychiatry, Grune & Stratton, Inc., 1985; McHugh, et al., Psychiatric Aspects of Neurological Disease, Psychiatric Syndromes of Huntington's Chorea: A Clinical and Phenomenologic Study, Grune & Stratton, 1975; Caine, et al., Psychiatric Syndromes in Huntington's Disease, Am J Psychiatry 140:6, June 1983; Ho, et al., The Acquired Immunodeficiency Syndrome (AIDS) Dementia Complex, Annals of Internal Medicine, Volume III, Number 5, Sep. 1, 1989; Cohen, et al., Amantadine Treatment of Fatigue Associated With Multiple Sclerosis, Arch Neurol Vol. 46, Jun. 1989). The common scientific view is to ascribe apathy-amotivation to deficits in the function of the frontal lobes. (Hecaen, et al., Psychiatric Aspects of Neurological Disease, Disorders of Mental Functioning Related to Frontal Lobe Pathology, Grune & Stratton, 1975; Trimble, Psychopathology of Frontal Lobe Syndromes, Seminars in Neurology, Volume 10, No. 3, Sep. 1990; Strub, Frontal Lobe Syndrome in a Patient With Bilateral Globus Pallidus Lesions, Arch Neurol, Vol. 46, Sep. 1989). In recent years, studies have implicated other brain structures that may be involved in the production of the apathy-amotivation syndrome (e.g., striatum and hippocampal formation) (Wang, Neurobehavioral Changes Following Caudate Infarct: A Case Report with Literature Review, Chin Med J (Taipei), 1991; Hedreen, et al., Neuronol loss in layers V and VI of cerebral cortex in Huntington's disease, Neuroscience Letters, 133 (1991); Alvarez, et al., The role of histamine in the anterior hypothalamus and its functional interaction with the hippocampus on exploratory behavior in adult male rats, Behavioural Brain Research, 48: 127-33, 1992) but their specific contribution is yet to be elucidated. Web site: http://www.delphion.com/details?pn=US05453428__

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Patent Applications on Chorea As of December 2000, U.S. patent applications are open to public viewing.6 Applications are patent requests which have yet to be granted. (The process to achieve a patent can take several years.) The following patent applications have been filed since December 2000 relating to chorea: •

Method of treatment of huntington's chorea with a protein extractable from mammalian organs Inventor(s): Panerai, Alberto; (Milano, IT) Correspondence: Nixon & Vanderhye, PC; 1100 N Glebe Road; 8th Floor; Arlington; VA; 22201-4714; US Patent Application Number: 20030153511 Date filed: April 17, 2003 Abstract: A method of treatment of patients affected by Huntington's chorea comprising the administration of an effective amount of a 14 kDa protein extractable from mammalian organs, particularly mammalian liver. Excerpt(s): The present invention concerns a method of treatment of patients affected by Huntington's chorea comprising the administration of an effective amount of a 14 kDa protein extractable from mammalian organs, particularly mammalian liver. Huntington's chorea (incidence of about 6.4/100,000) is an autosomal dominant degenerative disorder characterized by paralysis and intellectual deterioration beginning in young age and rapidly causing death. As in the case of Alzheimer's disease, this pathology also shows accumulation of protein agglomerates (huntingtine) which presumably lead to neurodegeneration. Several biochemical and genetic factors seem to be involved in the pathogenesis of Huntington's chorea, which remains however to be still elucidated. The formation of huntingtine in the brain of affected patients seems to be anyhow one of the main causes of the neurodegenerative effects typical of this disease. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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Use of fumaric acid derivatives for treating mitochondrial diseases Inventor(s): Joshi, Rajendra Kumar; (Zurich, CH), Strebel, Hans-Peter; (Luzern, CH) Correspondence: Sieberth & Patty; 2924 Brakley Drive Suite A 1; Baton Rouge; LA; 70816 Patent Application Number: 20030013761 Date filed: May 28, 2002 Abstract: The present invention relates to the use of individual fumaric acid derivatives or mixtures thereof for preparing a pharmaceutical composition for treating mitochondrial diseases, especially for treating Parkinson's syndrome, Alzheimer's disease, Chorea Huntington disease, retinopathia pigmentosa and mitochondrial encephalomyopathy. Preferably, the fumaric acid derivative(s) is/are those selected from the group consisting of fumaric acid dialkyl esters or fumaric acid monoalkyl esters in the form of the free acid or a salt thereof.

6

This has been a common practice outside the United States prior to December 2000.

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Excerpt(s): The mitochondria have an independent genetic system of DNA (mtDNA) and RNA and are therefore able to synthesise certain proteins on their own. Both genes of the cell nucleus and the mitochondrial genome code for the components of oxidative phosophorylation and the citrate cycle. A genetic defect of the mtDNA may therefore affect oxidative phosphorylation and the citrate cycle, respectively, and cause malfunctions. Such defects or malfunctions have been associated with the so-called mitochondrial diseases. Genetic defects of mtDNA may be the result of local mutation by which one base is replaced by another. Such point mutations are associated with neurogenetic myasthenia, ataxia and retinopathia pigmentosa, for example. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

Keeping Current In order to stay informed about patents and patent applications dealing with chorea, you can access the U.S. Patent Office archive via the Internet at the following Web address: http://www.uspto.gov/patft/index.html. You will see two broad options: (1) Issued Patent, and (2) Published Applications. To see a list of issued patents, perform the following steps: Under “Issued Patents,” click “Quick Search.” Then, type “chorea” (or synonyms) into the “Term 1” box. After clicking on the search button, scroll down to see the various patents which have been granted to date on chorea. You can also use this procedure to view pending patent applications concerning chorea. Simply go back to http://www.uspto.gov/patft/index.html. Select “Quick Search” under “Published Applications.” Then proceed with the steps listed above.

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CHAPTER 5. BOOKS ON CHOREA Overview This chapter provides bibliographic book references relating to chorea. In addition to online booksellers such as www.amazon.com and www.bn.com, excellent sources for book titles on chorea include the Combined Health Information Database and the National Library of Medicine. Your local medical library also may have these titles available for loan.

Chapters on Chorea In order to find chapters that specifically relate to chorea, an excellent source of abstracts is the Combined Health Information Database. You will need to limit your search to book chapters and chorea using the “Detailed Search” option. Go to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find book chapters, use the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer, and the format option “Book Chapter.” Type “chorea” (or synonyms) into the “For these words:” box. The following is a typical result when searching for book chapters on chorea: •

Neurological Disorders Source: in Scully, C. and Cawson, R.A. Medical Problems in Dentistry. 4th ed. Woburn, MA: Butterworth-Heinemann. 1998. p. 336-373. Contact: Available from Butterworth-Heinemann. 225 Wildwood Avenue, Woburn, MA 01801-2041. (800) 366-2665 or (781) 904-2500. Fax (800) 446-6520 or (781) 933-6333. E-mail: [email protected]. Website: www.bh.com. PRICE: $110.00. ISBN: 0723610568. Summary: Dental staff should be able to recognize abnormalities involving the cranial nerves, especially the trigeminal, facial, glossopharyngeal, vagal and hypoglossal nerves. This chapter on neurologic disorders is from a text that covers the general medical and surgical conditions relevant to the oral health care sciences. Topics include congenital neurological disorders, including cerebral palsy (CP), neural tube defects (spina bifida), syringomyelia, Huntington's chorea, and Friedreich's ataxia; acquired neurological disorders, including the examination and lesions of the cranial nerves, facial sensory loss (facial pain is covered in a separate chapter), facial paralysis, Bell's

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palsy, trigeminal motor neuropathy, abnormal facial movements (dystonias, dyskinesias, facial tics, Tourette syndrome), multiple cranial nerve palsies, blindness and visual impairment, deafness and hearing impairment, Meniere's disease, autonomic dysfunction, epilepsy, syncope (fainting), raised intracranial pressure, hypoxic encephalopathy, infections of the nervous system (including HIV and syphilis), cerebrovascular accidents (stroke), Parkinson's disease, multiple sclerosis, Guillain-Barre syndrome (infective or idiopathic polyneuritis), motor neurone disease, mercury intoxication, tumors of the central nervous system (CNS), myasthenia gravis, patients with respiratory paralysis, and peripheral neuropathies. For each condition, the authors discuss general aspects, diagnosis and management issues, dental aspects, and patient care strategies. The chapter includes a summary of the points covered. 1 appendix. 4 figures. 15 tables. 52 references. •

Alzheimer's Disease and Other Organic Causes of Mental Disorders Source: in Kass, F.I.; et al., eds. Columbia University College of Physicians and Surgeons: Complete Home Guide to Mental Health. New York, NY: Henry Holt and Company, Inc. 1992. p. 206-219. Contact: Available from Henry Holt and Company, Inc. 115 West 18th Street, New York, NY 10011. PRICE: $35.00. ISBN: 0805007245. Summary: Numerous disorders affecting mood, memory, concentration, intellect, and personality arise from physical abnormalities in the brain and often require medical intervention. Psychiatric and psychological treatments may ease some symptoms and may help patients and their families adjust to the diseases and deficits caused by them. However, these treatments often overlook the underlying physical conditions. Consequently, appropriate treatments for some dementias require investigating and addressing the underlying illness or physical problem. As part of a home reference guide, this chapter describes physical conditions that produce mental symptoms. Topics include: Alzheimer's disease (symptoms, progression, diagnosis, medical treatment, care, family caregiver stress, possible causes, and stages); ways of dealing with dementia on a daily basis; other primary dementias (multi-infarct dementia, Huntington's chorea dementia, and Pick's disease dementia); other physical brain disorders (AIDS dementia and other infectious diseases); ways for families to obtain help and information on dementia; medical treatments and medical illnesses that commonly produce organic (physical) mental symptoms (brain tumors and cancer, nutritional deficiencies and toxins, liver disease, endocrine disorders, drug and alcohol use and withdrawal, and trauma); delirium; and financial and legal issues for families caring for patients with Alzheimer's disease or other dementias.

•

Rheumatic Fever Source: in Maddison, P.J.; et al., Eds. Oxford Textbook of Rheumatology. Volume 2. New York, NY: Oxford University Press, Inc. 1993. p. 613-620. Contact: Available from Oxford University Press, Inc., New York, NY. Summary: This chapter for health professionals presents an overview of rheumatic fever. The classical ways in which rheumatic fever may manifest itself are outlined. The epidemiology and pathogenesis are discussed. The role of group A streptococcus and genetics in the disease process of rheumatic fever is examined. Theories on the pathological mechanisms of group A streptococcus in rheumatic fever are presented. The clinical features of acute rheumatic fever are described, including arthritis, carditis, rheumatic heart disease, chorea, subcutaneous nodules, and erythema marginatum. The

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minor manifestations of rheumatic fever are identified, including fever, abdominal pain, epistaxis, and rheumatic pneumonia. The use of laboratory tests in the diagnosis of rheumatic fever is discussed. The clinical course and treatment of rheumatic fever is explained, and the use of antibiotic prophylaxis following resolution of the acute episode is considered. 56 references, 1 figure, and 3 tables. •

Chapter 67: Movement Disorders Source: in Berkow, R., ed. The Merck Manual of Medical Information: Home Edition (online version). Rahway, NJ: Merck and Company, Inc. 2000. 13 p. Contact: Available online from Merck and Company, Inc. (800) 819-9456. Website: www.merck.com/pubs/mmanual_home/contents.htm. Also available from your local book store. PRICE: $29.95 plus shipping. Summary: This chapter provides information on the symptoms, diagnosis, and treatment of various movement disorders, including tremors, cramps, myoclonus, hiccups, Tourette's syndrome, chorea, athetosis, Huntington's disease, dystonia, Parkinson's disease, progressive supranuclear palsy, Shy-Drager syndrome, and coordination disorders. Damage to or an abnormality of an area of the nervous system that regulates movement may cause a person to experience a movement disorder. A tremor is an involuntary, rhythmic, shaking movement that occurs when muscles repeatedly contract and relax. Types of tremors include action, resting, intention, essential, senile, and familial tremors. Although treatment is usually not needed for movement disorders, drugs may be helpful for some people. A cramp is a sudden, brief, usually painful contraction of a shortened muscle or group of muscles. Cramps are common in healthy people and do not need to be treated. Myoclonus causes a synchronous quick jerk of affected muscles. Antiseizure drugs may be used to treat severe cases of myoclonic jerks. Hiccups are repeated spasms of the diaphragm followed by quick, noisy closings of the glottis. They are caused when a stimulus triggers the nerves that contract the diaphragm. Most cures for hiccups require holding the breath to increase the amount of carbon dioxide in the blood. Stimulating the vagus nerve may also help. Tourette's syndrome is a hereditary disorder characterized by motor and vocal tics. Antipsychotic drugs may help suppress them. Chorea and athetosis are symptoms that can result from several different diseases, including Huntington's disease and Sydenham's disease. Drugs that block the action of dopamine may help control abnormal movements. Huntington's disease is an inherited disease characterized by abnormal movements and gradual loss of brain cells. There is no cure for Huntington's disease, but drugs may help relieve symptoms and control behavior. In people who have dystonia, muscles may freeze in the middle of an action. Dystonia seems to be caused by overactivity in several areas of the brain. Types of dystonia are idiopathic torsion dystonia, blepharospasm, torticollis, and spasmodic dysphonia. Treatment is limited. Drugs with anticholinergic properties may be helpful. Parkinson's disease is a progressive, degenerative disorder of the nervous system. Although the cause of nerve cell degeneration and dopamine loss is usually not known, Parkinson's disease is sometimes a very late complication of viral encephalitis or a complication of using certain drugs. Muscle stiffness develops in Parkinson's disease, and initiating a movement is difficult. Various drugs are used to treat this disease, including levodopa, bromocriptine, pergolide, selegiline, anticholinergics, antihistamines, antidepressants, propranolol, and amantadine. Progressive supranuclear palsy causes muscle rigidity, inability to roll the eyes upward, and weakness of the throat muscles. The cause is unknown, and there is no completely effective treatment. Shy-Drager syndrome causes malfunction and destruction of the autonomic nervous system. Treatment is the same as

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for Parkinson's disease but includes the drug fludrocortisone. Coordination disorders occur when the cerebellum is damaged. •

Neurologic Disorders Affecting the Voice in Performance Source: in Sataloff, R.T., ed. Professional Voice: The Science and Art of Clinical Care. 2nd ed. San Diego, CA: Singular Publishing Group, Inc. 1997. p. 479-498. Contact: Available from Singular Publishing Group, Inc. 401 West 'A' Street, Suite 325, San Diego, CA 92101-7904. (800) 521-8545 or (619) 238-6777. Fax (800) 774-8398 or (619) 238-6789. E-mail: [email protected]. Website: www.singpub.com. PRICE: $325.00 plus shipping and handling. ISBN: 1565937287. Summary: This chapter, from a book on the clinical care of the professional voice, discusses neurologic disorders affecting the voice in performance. The complex functions necessary for normal voice function require coordinated interactions among multiple body systems. Neurologic dysfunction that impairs control of these interactions commonly causes voice dysfunction. The authors note that it is not unusual for voice disorders to be the presenting complaint in patients with neurologic disease. Topics include neurolaryngology, neuroanatomy and neurophysiology of phonation, neurological dysfunction and voice, vocal cord paralysis, dysarthria (imperfect articulation in speech), vocal tremor, Parkinson disease, postpolio syndrome, stuttering, myasthenia gravis, and other neurological conditions affecting voice performance, including amyotrophic lateral sclerosis, multiple sclerosis, Huntington's chorea, Gilles de la Tourette syndrome, cerebrovascular accident (stroke), quadriplegia, facial paralysis in singers and actors, headache, and dizziness. The authors conclude that familiarity with the latest concepts in neurolaryngology, clinical voice disorders, and a close working relationship between laryngologists and neurologists may optimize treatment. 5 figures. 31 references.

•

Chapter 12-E: Infectious Disorders: Rheumatic Fever Source: in Klippel, J.H., et al., eds. Primer on the Rheumatic Diseases. 12th ed. Atlanta, GA: Arthritis Foundation. 2001. p. 279-283. Contact: Available from Arthritis Foundation. P.O. Box 1616, Alpharetta, GA 300091616. (800) 207-8633. Fax (credit card orders only) (770) 442-9742. Website: www.arthritis.org. PRICE: $69.95 plus shipping and handling. ISBN: 0912423293. Summary: This section of a chapter on infectious disorders provides health professionals with information on the epidemiology, pathogenesis, clinical features, diagnosis, and treatment of rheumatic fever. The disease is a delayed, nonsuppurative sequela of a pharyngeal infection by group A streptococci. Although the tissues of patients who have rheumatic fever show little evidence of group A streptococci infection, there is epidemiologic evidence indirectly implicating the bacteria in the initiation of the disease. Clinical features of rheumatic fever include arthritis that affects several joints in quick succession for a short time, heart murmurs, cardiomegaly, congestive heart failure, and pericarditis. Rheumatic heart disease, the most severe sequelae of acute rheumatic fever, occurs 10 to 20 years after the original attack and is the major cause of acquired valvular disease. Other clinical features include chorea, subcutaneous nodules, and erythema marginatum. Although the diagnosis of rheumatic fever cannot be established readily by laboratory tests, helpful tests include serial chest radiographs, electrocardiograms, streptococcal antibody tests, and acute phase reactant tests. Most cases can be treated with antiinflammatory agents, usually aspirin. Oral prednisone may be used to treat carditis. Antibiotic therapy with penicillin should be initiated and maintained for at

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least 10 days, whether or not signs of pharyngitis are present. Antibiotic prophylaxis with penicillin should start immediately after resolution of the acute episode and continue until the patient is a young adult. 1 figure, 1 table, and 16 references.

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CHAPTER 6. PERIODICALS AND NEWS ON CHOREA Overview In this chapter, we suggest a number of news sources and present various periodicals that cover chorea.

News Services and Press Releases One of the simplest ways of tracking press releases on chorea is to search the news wires. In the following sample of sources, we will briefly describe how to access each service. These services only post recent news intended for public viewing. PR Newswire To access the PR Newswire archive, simply go to http://www.prnewswire.com/. Select your country. Type “chorea” (or synonyms) into the search box. You will automatically receive information on relevant news releases posted within the last 30 days. The search results are shown by order of relevance. Reuters Health The Reuters’ Medical News and Health eLine databases can be very useful in exploring news archives relating to chorea. While some of the listed articles are free to view, others are available for purchase for a nominal fee. To access this archive, go to http://www.reutershealth.com/en/index.html and search by “chorea” (or synonyms). The following was recently listed in this archive for chorea: •

Tetrabenazine can be helpful in chorea associated with Huntington's disease Source: Reuters Industry Breifing Date: May 08, 2001

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The NIH Within MEDLINEplus, the NIH has made an agreement with the New York Times Syndicate, the AP News Service, and Reuters to deliver news that can be browsed by the public. Search news releases at http://www.nlm.nih.gov/medlineplus/alphanews_a.html. MEDLINEplus allows you to browse across an alphabetical index. Or you can search by date at the following Web page: http://www.nlm.nih.gov/medlineplus/newsbydate.html. Often, news items are indexed by MEDLINEplus within its search engine. Business Wire Business Wire is similar to PR Newswire. To access this archive, simply go to http://www.businesswire.com/. You can scan the news by industry category or company name. Market Wire Market Wire is more focused on technology than the other wires. To browse the latest press releases by topic, such as alternative medicine, biotechnology, fitness, healthcare, legal, nutrition, and pharmaceuticals, access Market Wire’s Medical/Health channel at http://www.marketwire.com/mw/release_index?channel=MedicalHealth. Or simply go to Market Wire’s home page at http://www.marketwire.com/mw/home, type “chorea” (or synonyms) into the search box, and click on “Search News.” As this service is technology oriented, you may wish to use it when searching for press releases covering diagnostic procedures or tests. Search Engines Medical news is also available in the news sections of commercial Internet search engines. See the health news page at Yahoo (http://dir.yahoo.com/Health/News_and_Media/), or you can use this Web site’s general news search page at http://news.yahoo.com/. Type in “chorea” (or synonyms). If you know the name of a company that is relevant to chorea, you can go to any stock trading Web site (such as http://www.etrade.com/) and search for the company name there. News items across various news sources are reported on indicated hyperlinks. Google offers a similar service at http://news.google.com/. BBC Covering news from a more European perspective, the British Broadcasting Corporation (BBC) allows the public free access to their news archive located at http://www.bbc.co.uk/. Search by “chorea” (or synonyms).

Academic Periodicals covering Chorea Numerous periodicals are currently indexed within the National Library of Medicine’s PubMed database that are known to publish articles relating to chorea. In addition to these

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sources, you can search for articles covering chorea that have been published by any of the periodicals listed in previous chapters. To find the latest studies published, go to http://www.ncbi.nlm.nih.gov/pubmed, type the name of the periodical into the search box, and click “Go.” If you want complete details about the historical contents of a journal, you can also visit the following Web site: http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi. Here, type in the name of the journal or its abbreviation, and you will receive an index of published articles. At http://locatorplus.gov/, you can retrieve more indexing information on medical periodicals (e.g. the name of the publisher). Select the button “Search LOCATORplus.” Then type in the name of the journal and select the advanced search option “Journal Title Search.”

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CHAPTER 7. RESEARCHING MEDICATIONS Overview While a number of hard copy or CD-ROM resources are available for researching medications, a more flexible method is to use Internet-based databases. Broadly speaking, there are two sources of information on approved medications: public sources and private sources. We will emphasize free-to-use public sources.

U.S. Pharmacopeia Because of historical investments by various organizations and the emergence of the Internet, it has become rather simple to learn about the medications recommended for chorea. One such source is the United States Pharmacopeia. In 1820, eleven physicians met in Washington, D.C. to establish the first compendium of standard drugs for the United States. They called this compendium the U.S. Pharmacopeia (USP). Today, the USP is a nonprofit organization consisting of 800 volunteer scientists, eleven elected officials, and 400 representatives of state associations and colleges of medicine and pharmacy. The USP is located in Rockville, Maryland, and its home page is located at http://www.usp.org/. The USP currently provides standards for over 3,700 medications. The resulting USP DI Advice for the Patient can be accessed through the National Library of Medicine of the National Institutes of Health. The database is partially derived from lists of federally approved medications in the Food and Drug Administration’s (FDA) Drug Approvals database, located at http://www.fda.gov/cder/da/da.htm. While the FDA database is rather large and difficult to navigate, the Phamacopeia is both user-friendly and free to use. It covers more than 9,000 prescription and over-the-counter medications. To access this database, simply type the following hyperlink into your Web browser: http://www.nlm.nih.gov/medlineplus/druginformation.html. To view examples of a given medication (brand names, category, description, preparation, proper use, precautions, side effects, etc.), simply follow the hyperlinks indicated within the United States Pharmacopeia (USP). Below, we have compiled a list of medications associated with chorea. If you would like more information on a particular medication, the provided hyperlinks will direct you to ample documentation (e.g. typical dosage, side effects, drug-interaction risks, etc.). The

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following drugs have been mentioned in the Pharmacopeia and other sources as being potentially applicable to chorea: Haloperidol •

Systemic - U.S. Brands: Haldol; Haldol Decanoate http://www.nlm.nih.gov/medlineplus/druginfo/uspdi/202278.html

Phenothiazines •

Systemic - U.S. Brands: Chlorpromazine Hydrochloride Intensol; Compazine; Compazine Spansule; Mellaril; Mellaril Concentrate; Mellaril-S; Permitil; Permitil Concentrate; Prolixin; Prolixin Concentrate; Prolixin Decanoate; Prolixin Enanthate; Serentil; Serentil Concentrate; Stelazine; Stelazine Concentrate; Thorazine; Thorazine Spansule; Trilafon; Trilafon Concentrate; Vesprin http://www.nlm.nih.gov/medlineplus/druginfo/uspdi/202457.html

Commercial Databases In addition to the medications listed in the USP above, a number of commercial sites are available by subscription to physicians and their institutions. Or, you may be able to access these sources from your local medical library.

Mosby’s Drug Consult Mosby’s Drug Consult database (also available on CD-ROM and book format) covers 45,000 drug products including generics and international brands. It provides prescribing information, drug interactions, and patient information. Subscription information is available at the following hyperlink: http://www.mosbysdrugconsult.com/.

PDRhealth The PDRhealth database is a free-to-use, drug information search engine that has been written for the public in layman’s terms. It contains FDA-approved drug information adapted from the Physicians’ Desk Reference (PDR) database. PDRhealth can be searched by brand name, generic name, or indication. It features multiple drug interactions reports. Search PDRhealth at http://www.pdrhealth.com/drug_info/index.html. Other Web Sites Drugs.com (www.drugs.com) reproduces the information in the Pharmacopeia as well as commercial information. You may also want to consider the Web site of the Medical Letter, Inc. (http://www.medletter.com/) which allows users to download articles on various drugs and therapeutics for a nominal fee. If you have any questions about a medical treatment, the FDA may have an office near you. Look for their number in the blue pages of the phone book. You can also contact the FDA through its toll-free number, 1-888-INFO-FDA (1-888-463-6332), or on the World Wide Web at www.fda.gov.

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APPENDICES

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APPENDIX A. PHYSICIAN RESOURCES Overview In this chapter, we focus on databases and Internet-based guidelines and information resources created or written for a professional audience.

NIH Guidelines Commonly referred to as “clinical” or “professional” guidelines, the National Institutes of Health publish physician guidelines for the most common diseases. Publications are available at the following by relevant Institute7: •

Office of the Director (OD); guidelines consolidated across agencies available at http://www.nih.gov/health/consumer/conkey.htm

•

National Institute of General Medical Sciences (NIGMS); fact sheets available at http://www.nigms.nih.gov/news/facts/

•

National Library of Medicine (NLM); extensive encyclopedia (A.D.A.M., Inc.) with guidelines: http://www.nlm.nih.gov/medlineplus/healthtopics.html

•

National Cancer Institute (NCI); guidelines available at http://www.cancer.gov/cancerinfo/list.aspx?viewid=5f35036e-5497-4d86-8c2c714a9f7c8d25

•

National Eye Institute (NEI); guidelines available at http://www.nei.nih.gov/order/index.htm

•

National Heart, Lung, and Blood Institute (NHLBI); guidelines available at http://www.nhlbi.nih.gov/guidelines/index.htm

•

National Human Genome Research Institute (NHGRI); research available at http://www.genome.gov/page.cfm?pageID=10000375

•

National Institute on Aging (NIA); guidelines available at http://www.nia.nih.gov/health/

7

These publications are typically written by one or more of the various NIH Institutes.

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•

National Institute on Alcohol Abuse and Alcoholism (NIAAA); guidelines available at http://www.niaaa.nih.gov/publications/publications.htm

•

National Institute of Allergy and Infectious Diseases (NIAID); guidelines available at http://www.niaid.nih.gov/publications/

•

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS); fact sheets and guidelines available at http://www.niams.nih.gov/hi/index.htm

•

National Institute of Child Health and Human Development (NICHD); guidelines available at http://www.nichd.nih.gov/publications/pubskey.cfm

•

National Institute on Deafness and Other Communication Disorders (NIDCD); fact sheets and guidelines at http://www.nidcd.nih.gov/health/

•

National Institute of Dental and Craniofacial Research (NIDCR); guidelines available at http://www.nidr.nih.gov/health/

•

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK); guidelines available at http://www.niddk.nih.gov/health/health.htm

•

National Institute on Drug Abuse (NIDA); guidelines available at http://www.nida.nih.gov/DrugAbuse.html

•

National Institute of Environmental Health Sciences (NIEHS); environmental health information available at http://www.niehs.nih.gov/external/facts.htm

•

National Institute of Mental Health (NIMH); guidelines available at http://www.nimh.nih.gov/practitioners/index.cfm

•

National Institute of Neurological Disorders and Stroke (NINDS); neurological disorder information pages available at http://www.ninds.nih.gov/health_and_medical/disorder_index.htm

•

National Institute of Nursing Research (NINR); publications on selected illnesses at http://www.nih.gov/ninr/news-info/publications.html

•

National Institute of Biomedical Imaging and Bioengineering; general information at http://grants.nih.gov/grants/becon/becon_info.htm

•

Center for Information Technology (CIT); referrals to other agencies based on keyword searches available at http://kb.nih.gov/www_query_main.asp

•

National Center for Complementary and Alternative Medicine (NCCAM); health information available at http://nccam.nih.gov/health/

•

National Center for Research Resources (NCRR); various information directories available at http://www.ncrr.nih.gov/publications.asp

•

Office of Rare Diseases; various fact sheets available at http://rarediseases.info.nih.gov/html/resources/rep_pubs.html

•

Centers for Disease Control and Prevention; various fact sheets on infectious diseases available at http://www.cdc.gov/publications.htm

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NIH Databases In addition to the various Institutes of Health that publish professional guidelines, the NIH has designed a number of databases for professionals.8 Physician-oriented resources provide a wide variety of information related to the biomedical and health sciences, both past and present. The format of these resources varies. Searchable databases, bibliographic citations, full-text articles (when available), archival collections, and images are all available. The following are referenced by the National Library of Medicine:9 •

Bioethics: Access to published literature on the ethical, legal, and public policy issues surrounding healthcare and biomedical research. This information is provided in conjunction with the Kennedy Institute of Ethics located at Georgetown University, Washington, D.C.: http://www.nlm.nih.gov/databases/databases_bioethics.html

•

HIV/AIDS Resources: Describes various links and databases dedicated to HIV/AIDS research: http://www.nlm.nih.gov/pubs/factsheets/aidsinfs.html

•

NLM Online Exhibitions: Describes “Exhibitions in the History of Medicine”: http://www.nlm.nih.gov/exhibition/exhibition.html. Additional resources for historical scholarship in medicine: http://www.nlm.nih.gov/hmd/hmd.html

•

Biotechnology Information: Access to public databases. The National Center for Biotechnology Information conducts research in computational biology, develops software tools for analyzing genome data, and disseminates biomedical information for the better understanding of molecular processes affecting human health and disease: http://www.ncbi.nlm.nih.gov/

•

Population Information: The National Library of Medicine provides access to worldwide coverage of population, family planning, and related health issues, including family planning technology and programs, fertility, and population law and policy: http://www.nlm.nih.gov/databases/databases_population.html

•

Cancer Information: Access to cancer-oriented databases: http://www.nlm.nih.gov/databases/databases_cancer.html

•

Profiles in Science: Offering the archival collections of prominent twentieth-century biomedical scientists to the public through modern digital technology: http://www.profiles.nlm.nih.gov/

•

Chemical Information: Provides links to various chemical databases and references: http://sis.nlm.nih.gov/Chem/ChemMain.html

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Clinical Alerts: Reports the release of findings from the NIH-funded clinical trials where such release could significantly affect morbidity and mortality: http://www.nlm.nih.gov/databases/alerts/clinical_alerts.html

•

Space Life Sciences: Provides links and information to space-based research (including NASA): http://www.nlm.nih.gov/databases/databases_space.html

•

MEDLINE: Bibliographic database covering the fields of medicine, nursing, dentistry, veterinary medicine, the healthcare system, and the pre-clinical sciences: http://www.nlm.nih.gov/databases/databases_medline.html

8

Remember, for the general public, the National Library of Medicine recommends the databases referenced in MEDLINEplus (http://medlineplus.gov/ or http://www.nlm.nih.gov/medlineplus/databases.html). 9 See http://www.nlm.nih.gov/databases/databases.html.

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•

Toxicology and Environmental Health Information (TOXNET): Databases covering toxicology and environmental health: http://sis.nlm.nih.gov/Tox/ToxMain.html

•

Visible Human Interface: Anatomically detailed, three-dimensional representations of normal male and female human bodies: http://www.nlm.nih.gov/research/visible/visible_human.html

The NLM Gateway10 The NLM (National Library of Medicine) Gateway is a Web-based system that lets users search simultaneously in multiple retrieval systems at the U.S. National Library of Medicine (NLM). It allows users of NLM services to initiate searches from one Web interface, providing one-stop searching for many of NLM’s information resources or databases.11 To use the NLM Gateway, simply go to the search site at http://gateway.nlm.nih.gov/gw/Cmd. Type “chorea” (or synonyms) into the search box and click “Search.” The results will be presented in a tabular form, indicating the number of references in each database category. Results Summary Category Journal Articles Books / Periodicals / Audio Visual Consumer Health Meeting Abstracts Other Collections Total

Items Found 4584 105 866 2 97 5654

HSTAT12 HSTAT is a free, Web-based resource that provides access to full-text documents used in healthcare decision-making.13 These documents include clinical practice guidelines, quickreference guides for clinicians, consumer health brochures, evidence reports and technology assessments from the Agency for Healthcare Research and Quality (AHRQ), as well as AHRQ’s Put Prevention Into Practice.14 Simply search by “chorea” (or synonyms) at the following Web site: http://text.nlm.nih.gov.

10

Adapted from NLM: http://gateway.nlm.nih.gov/gw/Cmd?Overview.x.

11

The NLM Gateway is currently being developed by the Lister Hill National Center for Biomedical Communications (LHNCBC) at the National Library of Medicine (NLM) of the National Institutes of Health (NIH). 12 Adapted from HSTAT: http://www.nlm.nih.gov/pubs/factsheets/hstat.html. 13 14

The HSTAT URL is http://hstat.nlm.nih.gov/.

Other important documents in HSTAT include: the National Institutes of Health (NIH) Consensus Conference Reports and Technology Assessment Reports; the HIV/AIDS Treatment Information Service (ATIS) resource documents; the Substance Abuse and Mental Health Services Administration's Center for Substance Abuse Treatment (SAMHSA/CSAT) Treatment Improvement Protocols (TIP) and Center for Substance Abuse Prevention (SAMHSA/CSAP) Prevention Enhancement Protocols System (PEPS); the Public Health Service (PHS) Preventive Services Task Force's Guide to Clinical Preventive Services; the independent, nonfederal Task Force on Community Services’ Guide to Community Preventive Services; and the Health Technology Advisory Committee (HTAC) of the Minnesota Health Care Commission (MHCC) health technology evaluations.

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Coffee Break: Tutorials for Biologists15 Coffee Break is a general healthcare site that takes a scientific view of the news and covers recent breakthroughs in biology that may one day assist physicians in developing treatments. Here you will find a collection of short reports on recent biological discoveries. Each report incorporates interactive tutorials that demonstrate how bioinformatics tools are used as a part of the research process. Currently, all Coffee Breaks are written by NCBI staff.16 Each report is about 400 words and is usually based on a discovery reported in one or more articles from recently published, peer-reviewed literature.17 This site has new articles every few weeks, so it can be considered an online magazine of sorts. It is intended for general background information. You can access the Coffee Break Web site at the following hyperlink: http://www.ncbi.nlm.nih.gov/Coffeebreak/.

Other Commercial Databases In addition to resources maintained by official agencies, other databases exist that are commercial ventures addressing medical professionals. Here are some examples that may interest you: •

CliniWeb International: Index and table of contents to selected clinical information on the Internet; see http://www.ohsu.edu/cliniweb/.

•

Medical World Search: Searches full text from thousands of selected medical sites on the Internet; see http://www.mwsearch.com/.

15 Adapted 16

from http://www.ncbi.nlm.nih.gov/Coffeebreak/Archive/FAQ.html.

The figure that accompanies each article is frequently supplied by an expert external to NCBI, in which case the source of the figure is cited. The result is an interactive tutorial that tells a biological story. 17 After a brief introduction that sets the work described into a broader context, the report focuses on how a molecular understanding can provide explanations of observed biology and lead to therapies for diseases. Each vignette is accompanied by a figure and hypertext links that lead to a series of pages that interactively show how NCBI tools and resources are used in the research process.

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APPENDIX B. PATIENT RESOURCES Overview Official agencies, as well as federally funded institutions supported by national grants, frequently publish a variety of guidelines written with the patient in mind. These are typically called “Fact Sheets” or “Guidelines.” They can take the form of a brochure, information kit, pamphlet, or flyer. Often they are only a few pages in length. Since new guidelines on chorea can appear at any moment and be published by a number of sources, the best approach to finding guidelines is to systematically scan the Internet-based services that post them.

Patient Guideline Sources The remainder of this chapter directs you to sources which either publish or can help you find additional guidelines on topics related to chorea. Due to space limitations, these sources are listed in a concise manner. Do not hesitate to consult the following sources by either using the Internet hyperlink provided, or, in cases where the contact information is provided, contacting the publisher or author directly. The National Institutes of Health The NIH gateway to patients is located at http://health.nih.gov/. From this site, you can search across various sources and institutes, a number of which are summarized below. Topic Pages: MEDLINEplus The National Library of Medicine has created a vast and patient-oriented healthcare information portal called MEDLINEplus. Within this Internet-based system are “health topic pages” which list links to available materials relevant to chorea. To access this system, log on to http://www.nlm.nih.gov/medlineplus/healthtopics.html. From there you can either search using the alphabetical index or browse by broad topic areas. Recently, MEDLINEplus listed the following when searched for “chorea”:

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Degenerative Nerve Diseases http://www.nlm.nih.gov/medlineplus/degenerativenervediseases.html Movement Disorders http://www.nlm.nih.gov/medlineplus/movementdisorders.html Neuromuscular Disorders http://www.nlm.nih.gov/medlineplus/neuromusculardisorders.html Tremor http://www.nlm.nih.gov/medlineplus/tremor.html

Within the health topic page dedicated to chorea, the following was listed: •

Diagnosis/Symptoms Creatine Kinase Test Source: Muscular Dystrophy Association http://www.mdausa.org/publications/Quest/q71ss-cktest.html Electromyography Source: National Institutes of Health, Clinical Center http://www.cc.nih.gov/ccc/patient_education/procdiag/electromyography.pdf Electrophysiology Source: We Move http://www.mdvu.org/library/pediatric/diagnostics/dia_exa_electro.html Muscle Biopsies Source: Muscular Dystrophy Association http://www.mdausa.org/publications/Quest/q74ss.html Stiffness, Cramps and Twitching Source: Muscular Dystrophy Association http://www.mdausa.org/publications/Quest/q73ss.html Summary of Clinical Features and Diagnosis of Angelman Syndrome Source: Angelman Syndrome Foundation http://www.angelman.org/twopgClinical.htm

•

Treatment Botulinum Toxin Injections: A Treatment for Muscle Spasms Source: American Academy of Family Physicians http://familydoctor.org/017.xml

•

Coping Self Help Skills Source: Angelman Syndrome Foundation http://www.angelman.org/asselfhelp.htm

Patient Resources

•

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Latest News Mental Problems May Follow Strep Infection in Kids Source: 06/29/2004, Reuters Health http://www.nlm.nih.gov//www.nlm.nih.gov/medlineplus/news/fullstory_18646 .html

•

Organizations Angelman Syndrome Foundation http://www.angelman.org/ Friedreich's Ataxia Research Alliance http://www.frda.org/ National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/ We Move http://www.wemove.org/

•

Research Study Finds Psychiatric Disorders are Common in People with Cerebellar Degeneration Source: National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/news_and_events/news_article_cerebellar.htm

You may also choose to use the search utility provided by MEDLINEplus at the following Web address: http://www.nlm.nih.gov/medlineplus/. Simply type a keyword into the search box and click “Search.” This utility is similar to the NIH search utility, with the exception that it only includes materials that are linked within the MEDLINEplus system (mostly patient-oriented information). It also has the disadvantage of generating unstructured results. We recommend, therefore, that you use this method only if you have a very targeted search. The Combined Health Information Database (CHID) CHID Online is a reference tool that maintains a database directory of thousands of journal articles and patient education guidelines on chorea. CHID offers summaries that describe the guidelines available, including contact information and pricing. CHID’s general Web site is http://chid.nih.gov/. To search this database, go to http://chid.nih.gov/detail/detail.html. In particular, you can use the advanced search options to look up pamphlets, reports, brochures, and information kits. The following was recently posted in this archive: •

Huntington's Disease Source: Danbury, CT: National Organization for Rare Disorders, Inc. 2002. 10 p. Contact: Available from National Organization for Rare Disorders, Inc. (NORD). P. O. Box 1968, Danbury, CT 06813-1968. (203) 744-0100; (800) 999-6673; TDD (203) 797-9590. E-mail: [email protected]. Website: www.rarediseases.org. PRICE: Free for 1st copy, $7.50 per each additional copy.

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Summary: This fact sheet from the National Organization for Rare Disorders presents comprehensive information on Huntington's Disease, an inherited progressively degenerative disorder of the nervous system. The various synonyms for the disease are listed. The general discussion covers symptoms, causes, the affected population, related disorders (e.g., Hallervorden-Spatz Disease, Olivopontocerebellar Atrophy, Syndenham's Chorea, Wilson's Disease, and Tourette Syndrome), standard therapies, and investigational therapies with contact information for the investigators. Treatments are symptomatic and supportive. Neuroplastic medications such as haloperidon can partially suppress the involuntary movements. Additional contacts and resources, including those for genetic information and genetic counseling, are listed. 26 references. The NIH Search Utility The NIH search utility allows you to search for documents on over 100 selected Web sites that comprise the NIH-WEB-SPACE. Each of these servers is “crawled” and indexed on an ongoing basis. Your search will produce a list of various documents, all of which will relate in some way to chorea. The drawbacks of this approach are that the information is not organized by theme and that the references are often a mix of information for professionals and patients. Nevertheless, a large number of the listed Web sites provide useful background information. We can only recommend this route, therefore, for relatively rare or specific disorders, or when using highly targeted searches. To use the NIH search utility, visit the following Web page: http://search.nih.gov/index.html. Additional Web Sources A number of Web sites are available to the public that often link to government sites. These can also point you in the direction of essential information. The following is a representative sample: •

AOL: http://search.aol.com/cat.adp?id=168&layer=&from=subcats

•

Family Village: http://www.familyvillage.wisc.edu/specific.htm

•

Google: http://directory.google.com/Top/Health/Conditions_and_Diseases/

•

Med Help International: http://www.medhelp.org/HealthTopics/A.html

•

Open Directory Project: http://dmoz.org/Health/Conditions_and_Diseases/

•

Yahoo.com: http://dir.yahoo.com/Health/Diseases_and_Conditions/

•

WebMDHealth: http://my.webmd.com/health_topics

Finding Associations There are several Internet directories that provide lists of medical associations with information on or resources relating to chorea. By consulting all of associations listed in this chapter, you will have nearly exhausted all sources for patient associations concerned with chorea.

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The National Health Information Center (NHIC) The National Health Information Center (NHIC) offers a free referral service to help people find organizations that provide information about chorea. For more information, see the NHIC’s Web site at http://www.health.gov/NHIC/ or contact an information specialist by calling 1-800-336-4797. Directory of Health Organizations The Directory of Health Organizations, provided by the National Library of Medicine Specialized Information Services, is a comprehensive source of information on associations. The Directory of Health Organizations database can be accessed via the Internet at http://www.sis.nlm.nih.gov/Dir/DirMain.html. It is composed of two parts: DIRLINE and Health Hotlines. The DIRLINE database comprises some 10,000 records of organizations, research centers, and government institutes and associations that primarily focus on health and biomedicine. To access DIRLINE directly, go to the following Web site: http://dirline.nlm.nih.gov/. Simply type in “chorea” (or a synonym), and you will receive information on all relevant organizations listed in the database. Health Hotlines directs you to toll-free numbers to over 300 organizations. You can access this database directly at http://www.sis.nlm.nih.gov/hotlines/. On this page, you are given the option to search by keyword or by browsing the subject list. When you have received your search results, click on the name of the organization for its description and contact information. The Combined Health Information Database Another comprehensive source of information on healthcare associations is the Combined Health Information Database. Using the “Detailed Search” option, you will need to limit your search to “Organizations” and “chorea”. Type the following hyperlink into your Web browser: http://chid.nih.gov/detail/detail.html. To find associations, use the drop boxes at the bottom of the search page where “You may refine your search by.” For publication date, select “All Years.” Then, select your preferred language and the format option “Organization Resource Sheet.” Type “chorea” (or synonyms) into the “For these words:” box. You should check back periodically with this database since it is updated every three months. The National Organization for Rare Disorders, Inc. The National Organization for Rare Disorders, Inc. has prepared a Web site that provides, at no charge, lists of associations organized by health topic. You can access this database at the following Web site: http://www.rarediseases.org/search/orgsearch.html. Type “chorea” (or a synonym) into the search box, and click “Submit Query.”

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APPENDIX C. FINDING MEDICAL LIBRARIES Overview In this Appendix, we show you how to quickly find a medical library in your area.

Preparation Your local public library and medical libraries have interlibrary loan programs with the National Library of Medicine (NLM), one of the largest medical collections in the world. According to the NLM, most of the literature in the general and historical collections of the National Library of Medicine is available on interlibrary loan to any library. If you would like to access NLM medical literature, then visit a library in your area that can request the publications for you.18

Finding a Local Medical Library The quickest method to locate medical libraries is to use the Internet-based directory published by the National Network of Libraries of Medicine (NN/LM). This network includes 4626 members and affiliates that provide many services to librarians, health professionals, and the public. To find a library in your area, simply visit http://nnlm.gov/members/adv.html or call 1-800-338-7657.

Medical Libraries in the U.S. and Canada In addition to the NN/LM, the National Library of Medicine (NLM) lists a number of libraries with reference facilities that are open to the public. The following is the NLM’s list and includes hyperlinks to each library’s Web site. These Web pages can provide information on hours of operation and other restrictions. The list below is a small sample of

18

Adapted from the NLM: http://www.nlm.nih.gov/psd/cas/interlibrary.html.

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libraries recommended by the National Library of Medicine (sorted alphabetically by name of the U.S. state or Canadian province where the library is located)19: •

Alabama: Health InfoNet of Jefferson County (Jefferson County Library Cooperative, Lister Hill Library of the Health Sciences), http://www.uab.edu/infonet/

•

Alabama: Richard M. Scrushy Library (American Sports Medicine Institute)

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Arizona: Samaritan Regional Medical Center: The Learning Center (Samaritan Health System, Phoenix, Arizona), http://www.samaritan.edu/library/bannerlibs.htm

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California: Kris Kelly Health Information Center (St. Joseph Health System, Humboldt), http://www.humboldt1.com/~kkhic/index.html

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California: Community Health Library of Los Gatos, http://www.healthlib.org/orgresources.html

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California: Consumer Health Program and Services (CHIPS) (County of Los Angeles Public Library, Los Angeles County Harbor-UCLA Medical Center Library) - Carson, CA, http://www.colapublib.org/services/chips.html

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California: Gateway Health Library (Sutter Gould Medical Foundation)

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California: Health Library (Stanford University Medical Center), http://wwwmed.stanford.edu/healthlibrary/

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California: Patient Education Resource Center - Health Information and Resources (University of California, San Francisco), http://sfghdean.ucsf.edu/barnett/PERC/default.asp

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California: Redwood Health Library (Petaluma Health Care District), http://www.phcd.org/rdwdlib.html

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California: Los Gatos PlaneTree Health Library, http://planetreesanjose.org/

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California: Sutter Resource Library (Sutter Hospitals Foundation, Sacramento), http://suttermedicalcenter.org/library/

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California: Health Sciences Libraries (University of California, Davis), http://www.lib.ucdavis.edu/healthsci/

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California: ValleyCare Health Library & Ryan Comer Cancer Resource Center (ValleyCare Health System, Pleasanton), http://gaelnet.stmarysca.edu/other.libs/gbal/east/vchl.html

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California: Washington Community Health Resource Library (Fremont), http://www.healthlibrary.org/

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Colorado: William V. Gervasini Memorial Library (Exempla Healthcare), http://www.saintjosephdenver.org/yourhealth/libraries/

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Connecticut: Hartford Hospital Health Science Libraries (Hartford Hospital), http://www.harthosp.org/library/

•

Connecticut: Healthnet: Connecticut Consumer Health Information Center (University of Connecticut Health Center, Lyman Maynard Stowe Library), http://library.uchc.edu/departm/hnet/

19

Abstracted from http://www.nlm.nih.gov/medlineplus/libraries.html.

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•

Connecticut: Waterbury Hospital Health Center Library (Waterbury Hospital, Waterbury), http://www.waterburyhospital.com/library/consumer.shtml

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Delaware: Consumer Health Library (Christiana Care Health System, Eugene du Pont Preventive Medicine & Rehabilitation Institute, Wilmington), http://www.christianacare.org/health_guide/health_guide_pmri_health_info.cfm

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Delaware: Lewis B. Flinn Library (Delaware Academy of Medicine, Wilmington), http://www.delamed.org/chls.html

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Georgia: Family Resource Library (Medical College of Georgia, Augusta), http://cmc.mcg.edu/kids_families/fam_resources/fam_res_lib/frl.htm

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Georgia: Health Resource Center (Medical Center of Central Georgia, Macon), http://www.mccg.org/hrc/hrchome.asp

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Hawaii: Hawaii Medical Library: Consumer Health Information Service (Hawaii Medical Library, Honolulu), http://hml.org/CHIS/

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Idaho: DeArmond Consumer Health Library (Kootenai Medical Center, Coeur d’Alene), http://www.nicon.org/DeArmond/index.htm

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Illinois: Health Learning Center of Northwestern Memorial Hospital (Chicago), http://www.nmh.org/health_info/hlc.html

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Illinois: Medical Library (OSF Saint Francis Medical Center, Peoria), http://www.osfsaintfrancis.org/general/library/

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Kentucky: Medical Library - Services for Patients, Families, Students & the Public (Central Baptist Hospital, Lexington), http://www.centralbap.com/education/community/library.cfm

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Kentucky: University of Kentucky - Health Information Library (Chandler Medical Center, Lexington), http://www.mc.uky.edu/PatientEd/

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Louisiana: Alton Ochsner Medical Foundation Library (Alton Ochsner Medical Foundation, New Orleans), http://www.ochsner.org/library/

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Louisiana: Louisiana State University Health Sciences Center Medical LibraryShreveport, http://lib-sh.lsuhsc.edu/

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Maine: Franklin Memorial Hospital Medical Library (Franklin Memorial Hospital, Farmington), http://www.fchn.org/fmh/lib.htm

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Maine: Gerrish-True Health Sciences Library (Central Maine Medical Center, Lewiston), http://www.cmmc.org/library/library.html

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Maine: Hadley Parrot Health Science Library (Eastern Maine Healthcare, Bangor), http://www.emh.org/hll/hpl/guide.htm

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Maine: Maine Medical Center Library (Maine Medical Center, Portland), http://www.mmc.org/library/

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Maine: Parkview Hospital (Brunswick), http://www.parkviewhospital.org/

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Maine: Southern Maine Medical Center Health Sciences Library (Southern Maine Medical Center, Biddeford), http://www.smmc.org/services/service.php3?choice=10

•

Maine: Stephens Memorial Hospital’s Health Information Library (Western Maine Health, Norway), http://www.wmhcc.org/Library/

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•

Manitoba, Canada: Consumer & Patient Health Information Service (University of Manitoba Libraries), http://www.umanitoba.ca/libraries/units/health/reference/chis.html

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Manitoba, Canada: J.W. Crane Memorial Library (Deer Lodge Centre, Winnipeg), http://www.deerlodge.mb.ca/crane_library/about.asp

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Maryland: Health Information Center at the Wheaton Regional Library (Montgomery County, Dept. of Public Libraries, Wheaton Regional Library), http://www.mont.lib.md.us/healthinfo/hic.asp

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Massachusetts: Baystate Medical Center Library (Baystate Health System), http://www.baystatehealth.com/1024/

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Massachusetts: Boston University Medical Center Alumni Medical Library (Boston University Medical Center), http://med-libwww.bu.edu/library/lib.html

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Massachusetts: Lowell General Hospital Health Sciences Library (Lowell General Hospital, Lowell), http://www.lowellgeneral.org/library/HomePageLinks/WWW.htm

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Massachusetts: Paul E. Woodard Health Sciences Library (New England Baptist Hospital, Boston), http://www.nebh.org/health_lib.asp

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Massachusetts: St. Luke’s Hospital Health Sciences Library (St. Luke’s Hospital, Southcoast Health System, New Bedford), http://www.southcoast.org/library/

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Massachusetts: Treadwell Library Consumer Health Reference Center (Massachusetts General Hospital), http://www.mgh.harvard.edu/library/chrcindex.html

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Massachusetts: UMass HealthNet (University of Massachusetts Medical School, Worchester), http://healthnet.umassmed.edu/

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Michigan: Botsford General Hospital Library - Consumer Health (Botsford General Hospital, Library & Internet Services), http://www.botsfordlibrary.org/consumer.htm

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Michigan: Helen DeRoy Medical Library (Providence Hospital and Medical Centers), http://www.providence-hospital.org/library/

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Michigan: Marquette General Hospital - Consumer Health Library (Marquette General Hospital, Health Information Center), http://www.mgh.org/center.html

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Michigan: Patient Education Resouce Center - University of Michigan Cancer Center (University of Michigan Comprehensive Cancer Center, Ann Arbor), http://www.cancer.med.umich.edu/learn/leares.htm

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Michigan: Sladen Library & Center for Health Information Resources - Consumer Health Information (Detroit), http://www.henryford.com/body.cfm?id=39330

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Montana: Center for Health Information (St. Patrick Hospital and Health Sciences Center, Missoula)

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National: Consumer Health Library Directory (Medical Library Association, Consumer and Patient Health Information Section), http://caphis.mlanet.org/directory/index.html

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National: National Network of Libraries of Medicine (National Library of Medicine) provides library services for health professionals in the United States who do not have access to a medical library, http://nnlm.gov/

•

National: NN/LM List of Libraries Serving the Public (National Network of Libraries of Medicine), http://nnlm.gov/members/

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Nevada: Health Science Library, West Charleston Library (Las Vegas-Clark County Library District, Las Vegas), http://www.lvccld.org/special_collections/medical/index.htm

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New Hampshire: Dartmouth Biomedical Libraries (Dartmouth College Library, Hanover), http://www.dartmouth.edu/~biomed/resources.htmld/conshealth.htmld/

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New Jersey: Consumer Health Library (Rahway Hospital, Rahway), http://www.rahwayhospital.com/library.htm

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New Jersey: Dr. Walter Phillips Health Sciences Library (Englewood Hospital and Medical Center, Englewood), http://www.englewoodhospital.com/links/index.htm

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New Jersey: Meland Foundation (Englewood Hospital and Medical Center, Englewood), http://www.geocities.com/ResearchTriangle/9360/

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New York: Choices in Health Information (New York Public Library) - NLM Consumer Pilot Project participant, http://www.nypl.org/branch/health/links.html

•

New York: Health Information Center (Upstate Medical University, State University of New York, Syracuse), http://www.upstate.edu/library/hic/

•

New York: Health Sciences Library (Long Island Jewish Medical Center, New Hyde Park), http://www.lij.edu/library/library.html

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New York: ViaHealth Medical Library (Rochester General Hospital), http://www.nyam.org/library/

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Ohio: Consumer Health Library (Akron General Medical Center, Medical & Consumer Health Library), http://www.akrongeneral.org/hwlibrary.htm

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Oklahoma: The Health Information Center at Saint Francis Hospital (Saint Francis Health System, Tulsa), http://www.sfh-tulsa.com/services/healthinfo.asp

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Oregon: Planetree Health Resource Center (Mid-Columbia Medical Center, The Dalles), http://www.mcmc.net/phrc/

•

Pennsylvania: Community Health Information Library (Milton S. Hershey Medical Center, Hershey), http://www.hmc.psu.edu/commhealth/

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Pennsylvania: Community Health Resource Library (Geisinger Medical Center, Danville), http://www.geisinger.edu/education/commlib.shtml

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Pennsylvania: HealthInfo Library (Moses Taylor Hospital, Scranton), http://www.mth.org/healthwellness.html

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Pennsylvania: Hopwood Library (University of Pittsburgh, Health Sciences Library System, Pittsburgh), http://www.hsls.pitt.edu/guides/chi/hopwood/index_html

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Pennsylvania: Koop Community Health Information Center (College of Physicians of Philadelphia), http://www.collphyphil.org/kooppg1.shtml

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Pennsylvania: Learning Resources Center - Medical Library (Susquehanna Health System, Williamsport), http://www.shscares.org/services/lrc/index.asp

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Pennsylvania: Medical Library (UPMC Health System, Pittsburgh), http://www.upmc.edu/passavant/library.htm

•

Quebec, Canada: Medical Library (Montreal General Hospital), http://www.mghlib.mcgill.ca/

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South Dakota: Rapid City Regional Hospital Medical Library (Rapid City Regional Hospital), http://www.rcrh.org/Services/Library/Default.asp

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Texas: Houston HealthWays (Houston Academy of Medicine-Texas Medical Center Library), http://hhw.library.tmc.edu/

•

Washington: Community Health Library (Kittitas Valley Community Hospital), http://www.kvch.com/

•

Washington: Southwest Washington Medical Center Library (Southwest Washington Medical Center, Vancouver), http://www.swmedicalcenter.com/body.cfm?id=72

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ONLINE GLOSSARIES The Internet provides access to a number of free-to-use medical dictionaries. The National Library of Medicine has compiled the following list of online dictionaries: •

ADAM Medical Encyclopedia (A.D.A.M., Inc.), comprehensive medical reference: http://www.nlm.nih.gov/medlineplus/encyclopedia.html

•

MedicineNet.com Medical Dictionary (MedicineNet, Inc.): http://www.medterms.com/Script/Main/hp.asp

•

Merriam-Webster Medical Dictionary (Inteli-Health, Inc.): http://www.intelihealth.com/IH/

•

Multilingual Glossary of Technical and Popular Medical Terms in Eight European Languages (European Commission) - Danish, Dutch, English, French, German, Italian, Portuguese, and Spanish: http://allserv.rug.ac.be/~rvdstich/eugloss/welcome.html

•

On-line Medical Dictionary (CancerWEB): http://cancerweb.ncl.ac.uk/omd/

•

Rare Diseases Terms (Office of Rare Diseases): http://ord.aspensys.com/asp/diseases/diseases.asp

•

Technology Glossary (National Library of Medicine) - Health Care Technology: http://www.nlm.nih.gov/nichsr/ta101/ta10108.htm

Beyond these, MEDLINEplus contains a very patient-friendly encyclopedia covering every aspect of medicine (licensed from A.D.A.M., Inc.). The ADAM Medical Encyclopedia can be accessed at http://www.nlm.nih.gov/medlineplus/encyclopedia.html. ADAM is also available on commercial Web sites such as drkoop.com (http://www.drkoop.com/) and Web MD (http://my.webmd.com/adam/asset/adam_disease_articles/a_to_z/a).

Online Dictionary Directories The following are additional online directories compiled by the National Library of Medicine, including a number of specialized medical dictionaries: •

Medical Dictionaries: Medical & Biological (World Health Organization): http://www.who.int/hlt/virtuallibrary/English/diction.htm#Medical

•

MEL-Michigan Electronic Library List of Online Health and Medical Dictionaries (Michigan Electronic Library): http://mel.lib.mi.us/health/health-dictionaries.html

•

Patient Education: Glossaries (DMOZ Open Directory Project): http://dmoz.org/Health/Education/Patient_Education/Glossaries/

•

Web of Online Dictionaries (Bucknell University): http://www.yourdictionary.com/diction5.html#medicine

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CHOREA DICTIONARY The definitions below are derived from official public sources, including the National Institutes of Health [NIH] and the European Union [EU]. 3-dimensional: 3-D. A graphic display of depth, width, and height. Three-dimensional radiation therapy uses computers to create a 3-dimensional picture of the tumor. This allows doctors to give the highest possible dose of radiation to the tumor, while sparing the normal tissue as much as possible. [NIH] Abdomen: That portion of the body that lies between the thorax and the pelvis. [NIH] Abdominal: Having to do with the abdomen, which is the part of the body between the chest and the hips that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs. [NIH] Abdominal Pain: Sensation of discomfort, distress, or agony in the abdominal region. [NIH] Abulia: Impairment or loss of will power. [NIH] Acanthocytes: Erythrocytes with protoplasmic projections giving the cell a thorny appearance. [NIH] Acetylcholine: A neurotransmitter. Acetylcholine in vertebrates is the major transmitter at neuromuscular junctions, autonomic ganglia, parasympathetic effector junctions, a subset of sympathetic effector junctions, and at many sites in the central nervous system. It is generally not used as an administered drug because it is broken down very rapidly by cholinesterases, but it is useful in some ophthalmological applications. [NIH] Acetylglucosamine: The N-acetyl derivative of glucosamine. [NIH] Acquired Immunodeficiency Syndrome: An acquired defect of cellular immunity associated with infection by the human immunodeficiency virus (HIV), a CD4-positive Tlymphocyte count under 200 cells/microliter or less than 14% of total lymphocytes, and increased susceptibility to opportunistic infections and malignant neoplasms. Clinical manifestations also include emaciation (wasting) and dementia. These elements reflect criteria for AIDS as defined by the CDC in 1993. [NIH] Actin: Essential component of the cell skeleton. [NIH] Acute lymphoblastic leukemia: ALL. A quickly progressing disease in which too many immature white blood cells called lymphoblasts are found in the blood and bone marrow. Also called acute lymphocytic leukemia. [NIH] Acute lymphocytic leukemia: ALL. A quickly progressing disease in which too many immature white blood cells called lymphoblasts are found in the blood and bone marrow. Also called acute lymphoblastic leukemia. [NIH] Acute renal: A condition in which the kidneys suddenly stop working. In most cases, kidneys can recover from almost complete loss of function. [NIH] Adaptability: Ability to develop some form of tolerance to conditions extremely different from those under which a living organism evolved. [NIH] Adaptation: 1. The adjustment of an organism to its environment, or the process by which it enhances such fitness. 2. The normal ability of the eye to adjust itself to variations in the intensity of light; the adjustment to such variations. 3. The decline in the frequency of firing of a neuron, particularly of a receptor, under conditions of constant stimulation. 4. In dentistry, (a) the proper fitting of a denture, (b) the degree of proximity and interlocking of

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restorative material to a tooth preparation, (c) the exact adjustment of bands to teeth. 5. In microbiology, the adjustment of bacterial physiology to a new environment. [EU] Adenine: A purine base and a fundamental unit of adenine nucleotides. [NIH] Adenosine: A nucleoside that is composed of adenine and d-ribose. Adenosine or adenosine derivatives play many important biological roles in addition to being components of DNA and RNA. Adenosine itself is a neurotransmitter. [NIH] Adrenergic: Activated by, characteristic of, or secreting epinephrine or substances with similar activity; the term is applied to those nerve fibres that liberate norepinephrine at a synapse when a nerve impulse passes, i.e., the sympathetic fibres. [EU] Adverse Effect: An unwanted side effect of treatment. [NIH] Aerobic: In biochemistry, reactions that need oxygen to happen or happen when oxygen is present. [NIH] Afferent: Concerned with the transmission of neural impulse toward the central part of the nervous system. [NIH] Affinity: 1. Inherent likeness or relationship. 2. A special attraction for a specific element, organ, or structure. 3. Chemical affinity; the force that binds atoms in molecules; the tendency of substances to combine by chemical reaction. 4. The strength of noncovalent chemical binding between two substances as measured by the dissociation constant of the complex. 5. In immunology, a thermodynamic expression of the strength of interaction between a single antigen-binding site and a single antigenic determinant (and thus of the stereochemical compatibility between them), most accurately applied to interactions among simple, uniform antigenic determinants such as haptens. Expressed as the association constant (K litres mole -1), which, owing to the heterogeneity of affinities in a population of antibody molecules of a given specificity, actually represents an average value (mean intrinsic association constant). 6. The reciprocal of the dissociation constant. [EU] Agonist: In anatomy, a prime mover. In pharmacology, a drug that has affinity for and stimulates physiologic activity at cell receptors normally stimulated by naturally occurring substances. [EU] Alanine: A non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases immunity, and provides energy for muscle tissue, brain, and the central nervous system. [NIH] Albumin: 1. Any protein that is soluble in water and moderately concentrated salt solutions and is coagulable by heat. 2. Serum albumin; the major plasma protein (approximately 60 per cent of the total), which is responsible for much of the plasma colloidal osmotic pressure and serves as a transport protein carrying large organic anions, such as fatty acids, bilirubin, and many drugs, and also carrying certain hormones, such as cortisol and thyroxine, when their specific binding globulins are saturated. Albumin is synthesized in the liver. Low serum levels occur in protein malnutrition, active inflammation and serious hepatic and renal disease. [EU] Alexia: The inability to recognize or comprehend written or printed words. [NIH] Algorithms: A procedure consisting of a sequence of algebraic formulas and/or logical steps to calculate or determine a given task. [NIH] Alkaline: Having the reactions of an alkali. [EU] Alkaloid: A member of a large group of chemicals that are made by plants and have nitrogen in them. Some alkaloids have been shown to work against cancer. [NIH] Alleles: Mutually exclusive forms of the same gene, occupying the same locus on

Dictionary 123

homologous chromosomes, and governing the same biochemical and developmental process. [NIH] Allergen: An antigenic substance capable of producing immediate-type hypersensitivity (allergy). [EU] Alternative medicine: Practices not generally recognized by the medical community as standard or conventional medical approaches and used instead of standard treatments. Alternative medicine includes the taking of dietary supplements, megadose vitamins, and herbal preparations; the drinking of special teas; and practices such as massage therapy, magnet therapy, spiritual healing, and meditation. [NIH] Amantadine: An antiviral that is used in the prophylactic or symptomatic treatment of Influenza A. It is also used as an antiparkinsonian agent, to treat extrapyramidal reactions, and for postherpetic neuralgia. The mechanisms of its effects in movement disorders are not well understood but probably reflect an increase in synthesis and release of dopamine, with perhaps some inhibition of dopamine uptake. [NIH] Ameliorated: A changeable condition which prevents the consequence of a failure or accident from becoming as bad as it otherwise would. [NIH] Amenorrhea: Absence of menstruation. [NIH] Amine: An organic compound containing nitrogen; any member of a group of chemical compounds formed from ammonia by replacement of one or more of the hydrogen atoms by organic (hydrocarbon) radicals. The amines are distinguished as primary, secondary, and tertiary, according to whether one, two, or three hydrogen atoms are replaced. The amines include allylamine, amylamine, ethylamine, methylamine, phenylamine, propylamine, and many other compounds. [EU] Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining protein conformation. [NIH] Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins. [NIH] Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins. [NIH] Amphetamine: A powerful central nervous system stimulant and sympathomimetic. Amphetamine has multiple mechanisms of action including blocking uptake of adrenergics and dopamine, stimulation of release of monamines, and inhibiting monoamine oxidase. Amphetamine is also a drug of abuse and a psychotomimetic. The l- and the d,l-forms are included here. The l-form has less central nervous system activity but stronger cardiovascular effects. The d-form is dextroamphetamine. [NIH] Amyotrophy: A type of diabetic neuropathy that causes muscle weakness and wasting. [NIH] Anaesthesia: Loss of feeling or sensation. Although the term is used for loss of tactile sensibility, or of any of the other senses, it is applied especially to loss of the sensation of pain, as it is induced to permit performance of surgery or other painful procedures. [EU] Anal: Having to do with the anus, which is the posterior opening of the large bowel. [NIH] Analog: In chemistry, a substance that is similar, but not identical, to another. [NIH] Anatomical: Pertaining to anatomy, or to the structure of the organism. [EU] Anergy: Absence of immune response to particular substances. [NIH]

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Aneurysm: A sac formed by the dilatation of the wall of an artery, a vein, or the heart. [NIH] Angina: Chest pain that originates in the heart. [NIH] Angina Pectoris: The symptom of paroxysmal pain consequent to myocardial ischemia usually of distinctive character, location and radiation, and provoked by a transient stressful situation during which the oxygen requirements of the myocardium exceed the capacity of the coronary circulation to supply it. [NIH] Animal model: An animal with a disease either the same as or like a disease in humans. Animal models are used to study the development and progression of diseases and to test new treatments before they are given to humans. Animals with transplanted human cancers or other tissues are called xenograft models. [NIH] Annealing: The spontaneous alignment of two single DNA strands to form a double helix. [NIH]

Anophthalmia: Absence of an eye or eyes in the newborn due to failure of development of the optic cup or to disappearance of the eyes after partial development. [NIH] Antecedent: Existing or occurring before in time or order often with consequential effects. [EU]

Anterograde: Moving or extending forward; called also antegrade. [EU] Antibacterial: A substance that destroys bacteria or suppresses their growth or reproduction. [EU] Antibiotic: A drug used to treat infections caused by bacteria and other microorganisms. [NIH]

Antibiotic Prophylaxis: Use of antibiotics before, during, or after a diagnostic, therapeutic, or surgical procedure to prevent infectious complications. [NIH] Antibodies: Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the antigen that induced their synthesis in cells of the lymphoid series (especially plasma cells), or with an antigen closely related to it. [NIH] Antibodies, Anticardiolipin: Antiphospholipid antibodies found in association with systemic lupus erythematosus (lupus erythematosus, systemic), antiphospholipid syndrome, and in a variety of other diseases as well as in healthy individuals. The antibodies are detected by solid-phase immunoassay employing the purified phospholipid antigen cardiolipin. [NIH] Antibodies, Antiphospholipid: Autoantibodies directed against phospholipids. These antibodies are characteristically found in patients with systemic lupus erythematosus, antiphospholipid syndrome, related autoimmune diseases, some non-autoimmune diseases, and also in healthy individuals. [NIH] Antibody: A type of protein made by certain white blood cells in response to a foreign substance (antigen). Each antibody can bind to only a specific antigen. The purpose of this binding is to help destroy the antigen. Antibodies can work in several ways, depending on the nature of the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen. [NIH] Anticholinergic: An agent that blocks the parasympathetic nerves. Called also parasympatholytic. [EU] Anticoagulant: A drug that helps prevent blood clots from forming. Also called a blood thinner. [NIH] Anticonvulsant: An agent that prevents or relieves convulsions. [EU] Antifungal: Destructive to fungi, or suppressing their reproduction or growth; effective

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against fungal infections. [EU] Antigen: Any substance which is capable, under appropriate conditions, of inducing a specific immune response and of reacting with the products of that response, that is, with specific antibody or specifically sensitized T-lymphocytes, or both. Antigens may be soluble substances, such as toxins and foreign proteins, or particulate, such as bacteria and tissue cells; however, only the portion of the protein or polysaccharide molecule known as the antigenic determinant (q.v.) combines with antibody or a specific receptor on a lymphocyte. Abbreviated Ag. [EU] Antihypertensive: An agent that reduces high blood pressure. [EU] Anti-inflammatory: Having to do with reducing inflammation. [NIH] Anti-Inflammatory Agents: Substances that reduce or suppress inflammation. [NIH] Antimetabolite: A chemical that is very similar to one required in a normal biochemical reaction in cells. Antimetabolites can stop or slow down the reaction. [NIH] Antimicrobial: Killing microorganisms, or suppressing their multiplication or growth. [EU] Antineoplastic: Inhibiting or preventing the development of neoplasms, checking the maturation and proliferation of malignant cells. [EU] Antiphospholipid Syndrome: The presence of antibodies directed against phospholipids (antibodies, antiphospholipid). The condition is associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses. In pregnancy it can cause abortion. Of the phospholipids, the cardiolipins show markedly elevated levels of anticardiolipin antibodies (antibodies, anticardiolipin). Present also are high levels of lupus anticoagulant (lupus coagulation inhibitor). [NIH] Antipsychotic: Effective in the treatment of psychosis. Antipsychotic drugs (called also neuroleptic drugs and major tranquilizers) are a chemically diverse (including phenothiazines, thioxanthenes, butyrophenones, dibenzoxazepines, dibenzodiazepines, and diphenylbutylpiperidines) but pharmacologically similar class of drugs used to treat schizophrenic, paranoid, schizoaffective, and other psychotic disorders; acute delirium and dementia, and manic episodes (during induction of lithium therapy); to control the movement disorders associated with Huntington's chorea, Gilles de la Tourette's syndrome, and ballismus; and to treat intractable hiccups and severe nausea and vomiting. Antipsychotic agents bind to dopamine, histamine, muscarinic cholinergic, a-adrenergic, and serotonin receptors. Blockade of dopaminergic transmission in various areas is thought to be responsible for their major effects : antipsychotic action by blockade in the mesolimbic and mesocortical areas; extrapyramidal side effects (dystonia, akathisia, parkinsonism, and tardive dyskinesia) by blockade in the basal ganglia; and antiemetic effects by blockade in the chemoreceptor trigger zone of the medulla. Sedation and autonomic side effects (orthostatic hypotension, blurred vision, dry mouth, nasal congestion and constipation) are caused by blockade of histamine, cholinergic, and adrenergic receptors. [EU] Antiviral: Destroying viruses or suppressing their replication. [EU] Anxiety: Persistent feeling of dread, apprehension, and impending disaster. [NIH] Aorta: The main trunk of the systemic arteries. [NIH] Apathy: Lack of feeling or emotion; indifference. [EU] Apomorphine: A derivative of morphine that is a dopamine D2 agonist. It is a powerful emetic and has been used for that effect in acute poisoning. It has also been used in the diagnosis and treatment of parkinsonism, but its adverse effects limit its use. [NIH] Apoptosis: One of the two mechanisms by which cell death occurs (the other being the

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pathological process of necrosis). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA (DNA fragmentation) at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth. [NIH] Aqueous: Having to do with water. [NIH] Arginine: An essential amino acid that is physiologically active in the L-form. [NIH] Arterial: Pertaining to an artery or to the arteries. [EU] Artery: Vessel-carrying blood from the heart to various parts of the body. [NIH] Articulation: The relationship of two bodies by means of a moveable joint. [NIH] Aspirin: A drug that reduces pain, fever, inflammation, and blood clotting. Aspirin belongs to the family of drugs called nonsteroidal anti-inflammatory agents. It is also being studied in cancer prevention. [NIH] Assay: Determination of the amount of a particular constituent of a mixture, or of the biological or pharmacological potency of a drug. [EU] Astringents: Agents, usually topical, that cause the contraction of tissues for the control of bleeding or secretions. [NIH] Astrocytes: The largest and most numerous neuroglial cells in the brain and spinal cord. Astrocytes (from "star" cells) are irregularly shaped with many long processes, including those with "end feet" which form the glial (limiting) membrane and directly and indirectly contribute to the blood brain barrier. They regulate the extracellular ionic and chemical environment, and "reactive astrocytes" (along with microglia) respond to injury. Astrocytes have high- affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitter, but their role in signaling (as in many other functions) is not well understood. [NIH] Ataxia: Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharnyx, larnyx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. [NIH] Athetosis: A derangement marked by ceaseless occurrence of slow, sinuous, writhing movements, especially severe in the hands, and performed involuntarily; it may occur after hemiplegia, and is then known as posthemiplegic chorea. Called also mobile spasm. [EU] Atrium: A chamber; used in anatomical nomenclature to designate a chamber affording entrance to another structure or organ. Usually used alone to designate an atrium of the heart. [EU] Atrophy: Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. [NIH] Atypical: Irregular; not conformable to the type; in microbiology, applied specifically to strains of unusual type. [EU] Auditory: Pertaining to the sense of hearing. [EU] Auscultation: Act of listening for sounds within the body. [NIH]

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Autoantibodies: Antibodies that react with self-antigens (autoantigens) of the organism that produced them. [NIH] Autoantigens: Endogenous tissue constituents that have the ability to interact with autoantibodies and cause an immune response. [NIH] Autoimmune disease: A condition in which the body recognizes its own tissues as foreign and directs an immune response against them. [NIH] Autoimmunity: Process whereby the immune system reacts against the body's own tissues. Autoimmunity may produce or be caused by autoimmune diseases. [NIH] Autonomic Nervous System: The enteric, parasympathetic, and sympathetic nervous systems taken together. Generally speaking, the autonomic nervous system regulates the internal environment during both peaceful activity and physical or emotional stress. Autonomic activity is controlled and integrated by the central nervous system, especially the hypothalamus and the solitary nucleus, which receive information relayed from visceral afferents; these and related central and sensory structures are sometimes (but not here) considered to be part of the autonomic nervous system itself. [NIH] Autopsy: Postmortem examination of the body. [NIH] Autoradiography: A process in which radioactive material within an object produces an image when it is in close proximity to a radiation sensitive emulsion. [NIH] Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body. [NIH] Bacteria: Unicellular prokaryotic microorganisms which generally possess rigid cell walls, multiply by cell division, and exhibit three principal forms: round or coccal, rodlike or bacillary, and spiral or spirochetal. [NIH] Bacteriostatic: 1. Inhibiting the growth or multiplication of bacteria. 2. An agent that inhibits the growth or multiplication of bacteria. [EU] Bacterium: Microscopic organism which may have a spherical, rod-like, or spiral unicellular or non-cellular body. Bacteria usually reproduce through asexual processes. [NIH] Basal Ganglia: Large subcortical nuclear masses derived from the telencephalon and located in the basal regions of the cerebral hemispheres. [NIH] Basal Ganglia Diseases: Diseases of the basal ganglia including the putamen; globus pallidus; claustrum; amygdala; and caudate nucleus. Dyskinesias (most notably involuntary movements and alterations of the rate of movement) represent the primary clinical manifestations of these disorders. Common etiologies include cerebrovascular disease; neurodegenerative diseases; and craniocerebral trauma. [NIH] Base: In chemistry, the nonacid part of a salt; a substance that combines with acids to form salts; a substance that dissociates to give hydroxide ions in aqueous solutions; a substance whose molecule or ion can combine with a proton (hydrogen ion); a substance capable of donating a pair of electrons (to an acid) for the formation of a coordinate covalent bond. [EU] Benign: Not cancerous; does not invade nearby tissue or spread to other parts of the body. [NIH]

Bifida: A defect in development of the vertebral column in which there is a central deficiency of the vertebral lamina. [NIH] Bilateral: Affecting both the right and left side of body. [NIH] Bile: An emulsifying agent produced in the liver and secreted into the duodenum. Its composition includes bile acids and salts, cholesterol, and electrolytes. It aids digestion of fats in the duodenum. [NIH]

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Biochemical: Relating to biochemistry; characterized by, produced by, or involving chemical reactions in living organisms. [EU] Biological response modifier: BRM. A substance that stimulates the body's response to infection and disease. [NIH] Biological therapy: Treatment to stimulate or restore the ability of the immune system to fight infection and disease. Also used to lessen side effects that may be caused by some cancer treatments. Also known as immunotherapy, biotherapy, or biological response modifier (BRM) therapy. [NIH] Biomarkers: Substances sometimes found in an increased amount in the blood, other body fluids, or tissues and that may suggest the presence of some types of cancer. Biomarkers include CA 125 (ovarian cancer), CA 15-3 (breast cancer), CEA (ovarian, lung, breast, pancreas, and GI tract cancers), and PSA (prostate cancer). Also called tumor markers. [NIH] Biosynthesis: The building up of a chemical compound in the physiologic processes of a living organism. [EU] Biotechnology: Body of knowledge related to the use of organisms, cells or cell-derived constituents for the purpose of developing products which are technically, scientifically and clinically useful. Alteration of biologic function at the molecular level (i.e., genetic engineering) is a central focus; laboratory methods used include transfection and cloning technologies, sequence and structure analysis algorithms, computer databases, and gene and protein structure function analysis and prediction. [NIH] Biotin: Hexahydro-2-oxo-1H-thieno(3,4-d)imidazole-4-pentanoic acid. Growth factor present in minute amounts in every living cell. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk.The biotin content of cancerous tissue is higher than that of normal tissue. [NIH] Biotransformation: The chemical alteration of an exogenous substance by or in a biological system. The alteration may inactivate the compound or it may result in the production of an active metabolite of an inactive parent compound. The alteration may be either nonsynthetic (oxidation-reduction, hydrolysis) or synthetic (glucuronide formation, sulfate conjugation, acetylation, methylation). This also includes metabolic detoxication and clearance. [NIH] Bipolar Disorder: A major affective disorder marked by severe mood swings (manic or major depressive episodes) and a tendency to remission and recurrence. [NIH] Bladder: The organ that stores urine. [NIH] Blepharospasm: Excessive winking; tonic or clonic spasm of the orbicularis oculi muscle. [NIH]

Blood Coagulation: The process of the interaction of blood coagulation factors that results in an insoluble fibrin clot. [NIH] Blood Platelets: Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation. [NIH] Blood pressure: The pressure of blood against the walls of a blood vessel or heart chamber. Unless there is reference to another location, such as the pulmonary artery or one of the heart chambers, it refers to the pressure in the systemic arteries, as measured, for example, in the forearm. [NIH] Blood vessel: A tube in the body through which blood circulates. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins. [NIH] Blood-Brain Barrier: Specialized non-fenestrated tightly-joined endothelial cells (tight junctions) that form a transport barrier for certain substances between the cerebral

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capillaries and the brain tissue. [NIH] Blot: To transfer DNA, RNA, or proteins to an immobilizing matrix such as nitrocellulose. [NIH]

Body Fluids: Liquid components of living organisms. [NIH] Bone Marrow: The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. [NIH] Bone scan: A technique to create images of bones on a computer screen or on film. A small amount of radioactive material is injected into a blood vessel and travels through the bloodstream; it collects in the bones and is detected by a scanner. [NIH] Bowel: The long tube-shaped organ in the abdomen that completes the process of digestion. There is both a small and a large bowel. Also called the intestine. [NIH] Bradykinin: A nonapeptide messenger that is enzymatically produced from kallidin in the blood where it is a potent but short-lived agent of arteriolar dilation and increased capillary permeability. Bradykinin is also released from mast cells during asthma attacks, from gut walls as a gastrointestinal vasodilator, from damaged tissues as a pain signal, and may be a neurotransmitter. [NIH] Brain Diseases: Pathologic conditions affecting the brain, which is composed of the intracranial components of the central nervous system. This includes (but is not limited to) the cerebral cortex; intracranial white matter; basal ganglia; thalamus; hypothalamus; brain stem; and cerebellum. [NIH] Brain Infarction: The formation of an area of necrosis in the brain, including the cerebral hemispheres (cerebral infarction), thalami, basal ganglia, brain stem (brain stem infarctions), or cerebellum secondary to an insufficiency of arterial or venous blood flow. [NIH] Brain Neoplasms: Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain. [NIH] Brain Stem: The part of the brain that connects the cerebral hemispheres with the spinal cord. It consists of the mesencephalon, pons, and medulla oblongata. [NIH] Brain Stem Infarctions: Infarctions that occur in the brain stem which is comprised of the midbrain, pons, and medulla. There are several named syndromes characterized by their distinctive clinical manifestations and specific sites of ischemic injury. [NIH] Bromocriptine: A semisynthetic ergot alkaloid that is a dopamine D2 agonist. It suppresses prolactin secretion and is used to treat amenorrhea, galactorrhea, and female infertility, and has been proposed for Parkinson disease. [NIH] Bromodeoxyuridine: A nucleoside that substitutes for thymidine in DNA and thus acts as an antimetabolite. It causes breaks in chromosomes and has been proposed as an antiviral and antineoplastic agent. It has been given orphan drug status for use in the treatment of primary brain tumors. [NIH] Bronchial: Pertaining to one or more bronchi. [EU] Bronchopulmonary: Pertaining to the lungs and their air passages; both bronchial and

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pulmonary. [EU] Bronchopulmonary Dysplasia: A chronic lung disease appearing in certain newborn infants treated for respiratory distress syndrome with mechanical ventilation and elevated concentration of inspired oxygen. [NIH] Buccal: Pertaining to or directed toward the cheek. In dental anatomy, used to refer to the buccal surface of a tooth. [EU] Bulbar: Pertaining to a bulb; pertaining to or involving the medulla oblongata, as bulbar paralysis. [EU] Butyric Acid: A four carbon acid, CH3CH2CH2COOH, with an unpleasant odor that occurs in butter and animal fat as the glycerol ester. [NIH] Calcification: Deposits of calcium in the tissues of the breast. Calcification in the breast can be seen on a mammogram, but cannot be detected by touch. There are two types of breast calcification, macrocalcification and microcalcification. Macrocalcifications are large deposits and are usually not related to cancer. Microcalcifications are specks of calcium that may be found in an area of rapidly dividing cells. Many microcalcifications clustered together may be a sign of cancer. [NIH] Calcium: A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes. [NIH] Carbamazepine: An anticonvulsant used to control grand mal and psychomotor or focal seizures. Its mode of action is not fully understood, but some of its actions resemble those of phenytoin; although there is little chemical resemblance between the two compounds, their three-dimensional structure is similar. [NIH] Carbohydrates: The largest class of organic compounds, including starches, glycogens, cellulose, gums, and simple sugars. Carbohydrates are composed of carbon, hydrogen, and oxygen in a ratio of Cn(H2O)n. [NIH] Carbon Dioxide: A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. [NIH] Carbon Monoxide Poisoning: Toxic asphyxiation due to the displacement of oxygen from oxyhemoglobin by carbon monoxide. [NIH] Carbonate Dehydratase: A zinc-containing enzyme of erythrocytes with molecular weight of 30 kD. It is among the most active of known enzymes and catalyzes the reversible hydration of carbon dioxide, which is significant in the transport of CO2 from the tissues to the lungs. The enzyme is inhibited by acetazolamide. EC 4.2.1.1. [NIH] Carbonic Anhydrase Inhibitors: A class of compounds that reduces the secretion of H+ ions by the proximal kidney tubule through inhibition of carbonic anhydrase (carbonate dehydratase). [NIH] Carcinogenic: Producing carcinoma. [EU] Carcinoid: A type of tumor usually found in the gastrointestinal system (most often in the appendix), and sometimes in the lungs or other sites. Carcinoid tumors are usually benign. [NIH]

Carcinoma: Cancer that begins in the skin or in tissues that line or cover internal organs. [NIH]

Cardiac: Having to do with the heart. [NIH]

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Cardiolipins: Acidic phospholipids composed of two molecules of phosphatidic acid covalently linked to a molecule of glycerol. They occur primarily in mitochondrial inner membranes and in bacterial plasma membranes. They are the main antigenic components of the Wassermann-type antigen that is used in nontreponemal syphilis serodiagnosis. [NIH] Cardiomegaly: Hypertrophy or enlargement of the heart. [NIH] Cardiomyopathy: A general diagnostic term designating primary myocardial disease, often of obscure or unknown etiology. [EU] Cardiopulmonary: Having to do with the heart and lungs. [NIH] Cardiopulmonary Bypass: Diversion of the flow of blood from the entrance of the right atrium directly to the aorta (or femoral artery) via an oxygenator thus bypassing both the heart and lungs. [NIH] Cardioselective: Having greater activity on heart tissue than on other tissue. [EU] Cardiovascular: Having to do with the heart and blood vessels. [NIH] Case report: A detailed report of the diagnosis, treatment, and follow-up of an individual patient. Case reports also contain some demographic information about the patient (for example, age, gender, ethnic origin). [NIH] Cataracts: In medicine, an opacity of the crystalline lens of the eye obstructing partially or totally its transmission of light. [NIH] Catecholamine: A group of chemical substances manufactured by the adrenal medulla and secreted during physiological stress. [NIH] Caudal: Denoting a position more toward the cauda, or tail, than some specified point of reference; same as inferior, in human anatomy. [EU] Caudate Nucleus: Elongated gray mass of the neostriatum located adjacent to the lateral ventricle of the brain. [NIH] Causal: Pertaining to a cause; directed against a cause. [EU] Cell: The individual unit that makes up all of the tissues of the body. All living things are made up of one or more cells. [NIH] Cell Death: The termination of the cell's ability to carry out vital functions such as metabolism, growth, reproduction, responsiveness, and adaptability. [NIH] Cell Differentiation: Progressive restriction of the developmental potential and increasing specialization of function which takes place during the development of the embryo and leads to the formation of specialized cells, tissues, and organs. [NIH] Cell Division: The fission of a cell. [NIH] Cell Extracts: Preparations of cell constituents or subcellular materials, isolates, or substances. [NIH] Cell membrane: Cell membrane = plasma membrane. The structure enveloping a cell, enclosing the cytoplasm, and forming a selective permeability barrier; it consists of lipids, proteins, and some carbohydrates, the lipids thought to form a bilayer in which integral proteins are embedded to varying degrees. [EU] Cell proliferation: An increase in the number of cells as a result of cell growth and cell division. [NIH] Cell Respiration: The metabolic process of all living cells (animal and plant) in which oxygen is used to provide a source of energy for the cell. [NIH] Cell Size: The physical dimensions of a cell. It refers mainly to changes in dimensions correlated with physiological or pathological changes in cells. [NIH]

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Cell Survival: The span of viability of a cell characterized by the capacity to perform certain functions such as metabolism, growth, reproduction, some form of responsiveness, and adaptability. [NIH] Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges. [NIH] Central Nervous System Infections: Pathogenic infections of the brain, spinal cord, and meninges. DNA virus infections; RNA virus infections; bacterial infections; mycoplasma infections; Spirochaetales infections; fungal infections; protozoan infections; helminthiasis; and prion diseases may involve the central nervous system as a primary or secondary process. [NIH] Cerebellar: Pertaining to the cerebellum. [EU] Cerebellar Diseases: Diseases that affect the structure or function of the cerebellum. Cardinal manifestations of cerebellar dysfunction include dysmetria, gait ataxia, and muscle hypotonia. [NIH] Cerebellum: Part of the metencephalon that lies in the posterior cranial fossa behind the brain stem. It is concerned with the coordination of movement. [NIH] Cerebral: Of or pertaining of the cerebrum or the brain. [EU] Cerebral Cortex: The thin layer of gray matter on the surface of the cerebral hemisphere that develops from the telencephalon and folds into gyri. It reaches its highest development in man and is responsible for intellectual faculties and higher mental functions. [NIH] Cerebral hemispheres: The two halves of the cerebrum, the part of the brain that controls muscle functions of the body and also controls speech, emotions, reading, writing, and learning. The right hemisphere controls muscle movement on the left side of the body, and the left hemisphere controls muscle movement on the right side of the body. [NIH] Cerebral Infarction: The formation of an area of necrosis in the cerebrum caused by an insufficiency of arterial or venous blood flow. Infarcts of the cerebrum are generally classified by hemisphere (i.e., left vs. right), lobe (e.g., frontal lobe infarction), arterial distribution (e.g., infarction, anterior cerebral artery), and etiology (e.g., embolic infarction). [NIH]

Cerebral Palsy: Refers to a motor disability caused by a brain dysfunction. [NIH] Cerebrospinal: Pertaining to the brain and spinal cord. [EU] Cerebrospinal fluid: CSF. The fluid flowing around the brain and spinal cord. Cerebrospinal fluid is produced in the ventricles in the brain. [NIH] Cerebrovascular: Pertaining to the blood vessels of the cerebrum, or brain. [EU] Cerebrum: The largest part of the brain. It is divided into two hemispheres, or halves, called the cerebral hemispheres. The cerebrum controls muscle functions of the body and also controls speech, emotions, reading, writing, and learning. [NIH] Cervical: Relating to the neck, or to the neck of any organ or structure. Cervical lymph nodes are located in the neck; cervical cancer refers to cancer of the uterine cervix, which is the lower, narrow end (the "neck") of the uterus. [NIH] Character: In current usage, approximately equivalent to personality. The sum of the relatively fixed personality traits and habitual modes of response of an individual. [NIH] Chin: The anatomical frontal portion of the mandible, also known as the mentum, that contains the line of fusion of the two separate halves of the mandible (symphysis menti). This line of fusion divides inferiorly to enclose a triangular area called the mental protuberance. On each side, inferior to the second premolar tooth, is the mental foramen for

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the passage of blood vessels and a nerve. [NIH] Cholesterol: The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils. [NIH] Choline: A basic constituent of lecithin that is found in many plants and animal organs. It is important as a precursor of acetylcholine, as a methyl donor in various metabolic processes, and in lipid metabolism. [NIH] Cholinergic: Resembling acetylcholine in pharmacological action; stimulated by or releasing acetylcholine or a related compound. [EU] Cholinergic Agents: Any drug used for its actions on cholinergic systems. Included here are agonists and antagonists, drugs that affect the life cycle of acetylcholine, and drugs that affect the survival of cholinergic neurons. The term cholinergic agents is sometimes still used in the narrower sense of muscarinic agonists, although most modern texts discourage that usage. [NIH] Choreatic Disorders: Acquired and hereditary conditions which feature chorea as a primary manifestation of the disease process. [NIH] Chromatin: The material of chromosomes. It is a complex of DNA, histones, and nonhistone proteins (chromosomal proteins, non-histone) found within the nucleus of a cell. [NIH] Chromosome: Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes. [NIH] Chromosome Abnormalities: Defects in the structure or number of chromosomes resulting in structural aberrations or manifesting as disease. [NIH] Chromosome Fragility: Susceptibility of chromosomes to breakage and translocation or other aberrations. Chromosome fragile sites are regions that show up in karyotypes as a gap (uncondensed stretch) on the chromatid arm. They are associated with chromosome break sites and other aberrations. A fragile site on the X chromosome is associated with fragile X syndrome. Fragile sites are designated by the letters "FRA" followed by the designation for the specific chromosome and a letter which refers to the different fragile sites on a chromosome (e.g. FRAXA). [NIH] Chronic: A disease or condition that persists or progresses over a long period of time. [NIH] Clamp: A u-shaped steel rod used with a pin or wire for skeletal traction in the treatment of certain fractures. [NIH] Clear cell carcinoma: A rare type of tumor of the female genital tract in which the inside of the cells looks clear when viewed under a microscope. [NIH] Cleft Lip: Congenital defect in the upper lip where the maxillary prominence fails to merge with the merged medial nasal prominences. It is thought to be caused by faulty migration of the mesoderm in the head region. [NIH] Cleft Palate: Congenital fissure of the soft and/or hard palate, due to faulty fusion. [NIH] Clinical study: A research study in which patients receive treatment in a clinic or other medical facility. Reports of clinical studies can contain results for single patients (case reports) or many patients (case series or clinical trials). [NIH] Clinical trial: A research study that tests how well new medical treatments or other interventions work in people. Each study is designed to test new methods of screening, prevention, diagnosis, or treatment of a disease. [NIH] Clonic: Pertaining to or of the nature of clonus. [EU] Cloning: The production of a number of genetically identical individuals; in genetic engineering, a process for the efficient replication of a great number of identical DNA

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molecules. [NIH] Clot Retraction: Retraction of a clot resulting from contraction of platelet pseudopods attached to fibrin strands that is dependent on the contractile protein thrombosthenin. Used as a measure of platelet function. [NIH] Coagulation: 1. The process of clot formation. 2. In colloid chemistry, the solidification of a sol into a gelatinous mass; an alteration of a disperse phase or of a dissolved solid which causes the separation of the system into a liquid phase and an insoluble mass called the clot or curd. Coagulation is usually irreversible. 3. In surgery, the disruption of tissue by physical means to form an amorphous residuum, as in electrocoagulation and photocoagulation. [EU] Coca: Any of several South American shrubs of the Erythroxylon genus (and family) that yield cocaine; the leaves are chewed with alum for CNS stimulation. [NIH] Cocaine: An alkaloid ester extracted from the leaves of plants including coca. It is a local anesthetic and vasoconstrictor and is clinically used for that purpose, particularly in the eye, ear, nose, and throat. It also has powerful central nervous system effects similar to the amphetamines and is a drug of abuse. Cocaine, like amphetamines, acts by multiple mechanisms on brain catecholaminergic neurons; the mechanism of its reinforcing effects is thought to involve inhibition of dopamine uptake. [NIH] Cofactor: A substance, microorganism or environmental factor that activates or enhances the action of another entity such as a disease-causing agent. [NIH] Cognition: Intellectual or mental process whereby an organism becomes aware of or obtains knowledge. [NIH] Collagen: A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of skin, connective tissue, and the organic substance of bones and teeth. Different forms of collagen are produced in the body but all consist of three alpha-polypeptide chains arranged in a triple helix. Collagen is differentiated from other fibrous proteins, such as elastin, by the content of proline, hydroxyproline, and hydroxylysine; by the absence of tryptophan; and particularly by the high content of polar groups which are responsible for its swelling properties. [NIH] Coloboma: Congenital anomaly in which some of the structures of the eye are absent due to incomplete fusion of the fetal intraocular fissure during gestation. [NIH] Combinatorial: A cut-and-paste process that churns out thousands of potentially valuable compounds at once. [NIH] Complement: A term originally used to refer to the heat-labile factor in serum that causes immune cytolysis, the lysis of antibody-coated cells, and now referring to the entire functionally related system comprising at least 20 distinct serum proteins that is the effector not only of immune cytolysis but also of other biologic functions. Complement activation occurs by two different sequences, the classic and alternative pathways. The proteins of the classic pathway are termed 'components of complement' and are designated by the symbols C1 through C9. C1 is a calcium-dependent complex of three distinct proteins C1q, C1r and C1s. The proteins of the alternative pathway (collectively referred to as the properdin system) and complement regulatory proteins are known by semisystematic or trivial names. Fragments resulting from proteolytic cleavage of complement proteins are designated with lower-case letter suffixes, e.g., C3a. Inactivated fragments may be designated with the suffix 'i', e.g. C3bi. Activated components or complexes with biological activity are designated by a bar over the symbol e.g. C1 or C4b,2a. The classic pathway is activated by the binding of C1 to classic pathway activators, primarily antigen-antibody complexes containing IgM, IgG1, IgG3; C1q binds to a single IgM molecule or two adjacent IgG molecules. The alternative

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pathway can be activated by IgA immune complexes and also by nonimmunologic materials including bacterial endotoxins, microbial polysaccharides, and cell walls. Activation of the classic pathway triggers an enzymatic cascade involving C1, C4, C2 and C3; activation of the alternative pathway triggers a cascade involving C3 and factors B, D and P. Both result in the cleavage of C5 and the formation of the membrane attack complex. Complement activation also results in the formation of many biologically active complement fragments that act as anaphylatoxins, opsonins, or chemotactic factors. [EU] Complementary and alternative medicine: CAM. Forms of treatment that are used in addition to (complementary) or instead of (alternative) standard treatments. These practices are not considered standard medical approaches. CAM includes dietary supplements, megadose vitamins, herbal preparations, special teas, massage therapy, magnet therapy, spiritual healing, and meditation. [NIH] Complementary medicine: Practices not generally recognized by the medical community as standard or conventional medical approaches and used to enhance or complement the standard treatments. Complementary medicine includes the taking of dietary supplements, megadose vitamins, and herbal preparations; the drinking of special teas; and practices such as massage therapy, magnet therapy, spiritual healing, and meditation. [NIH] Complete remission: The disappearance of all signs of cancer. Also called a complete response. [NIH] Compulsions: In psychology, an irresistible urge, sometimes amounting to obsession to perform a particular act which usually is carried out against the performer's will or better judgment. [NIH] Computational Biology: A field of biology concerned with the development of techniques for the collection and manipulation of biological data, and the use of such data to make biological discoveries or predictions. This field encompasses all computational methods and theories applicable to molecular biology and areas of computer-based techniques for solving biological problems including manipulation of models and datasets. [NIH] Computed tomography: CT scan. A series of detailed pictures of areas inside the body, taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computerized tomography and computerized axial tomography (CAT) scan. [NIH] Computerized axial tomography: A series of detailed pictures of areas inside the body, taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography (CT scan), or computerized tomography. [NIH] Conception: The onset of pregnancy, marked by implantation of the blastocyst; the formation of a viable zygote. [EU] Confounding: Extraneous variables resulting in outcome effects that obscure or exaggerate the "true" effect of an intervention. [NIH] Confusion: A mental state characterized by bewilderment, emotional disturbance, lack of clear thinking, and perceptual disorientation. [NIH] Congestion: Excessive or abnormal accumulation of blood in a part. [EU] Congestive heart failure: Weakness of the heart muscle that leads to a buildup of fluid in body tissues. [NIH] Conjugated: Acting or operating as if joined; simultaneous. [EU] Conjunctiva: The mucous membrane that lines the inner surface of the eyelids and the anterior part of the sclera. [NIH]

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Connective Tissue: Tissue that supports and binds other tissues. It consists of connective tissue cells embedded in a large amount of extracellular matrix. [NIH] Connective Tissue: Tissue that supports and binds other tissues. It consists of connective tissue cells embedded in a large amount of extracellular matrix. [NIH] Connective Tissue Cells: A group of cells that includes fibroblasts, cartilage cells, adipocytes, smooth muscle cells, and bone cells. [NIH] Connective Tissue Diseases: A heterogeneous group of disorders, some hereditary, others acquired, characterized by abnormal structure or function of one or more of the elements of connective tissue, i.e., collagen, elastin, or the mucopolysaccharides. [NIH] Consciousness: Sense of awareness of self and of the environment. [NIH] Constitutional: 1. Affecting the whole constitution of the body; not local. 2. Pertaining to the constitution. [EU] Constriction: The act of constricting. [NIH] Contraceptive: An agent that diminishes the likelihood of or prevents conception. [EU] Contraindications: Any factor or sign that it is unwise to pursue a certain kind of action or treatment, e. g. giving a general anesthetic to a person with pneumonia. [NIH] Contralateral: Having to do with the opposite side of the body. [NIH] Controlled clinical trial: A clinical study that includes a comparison (control) group. The comparison group receives a placebo, another treatment, or no treatment at all. [NIH] Controlled study: An experiment or clinical trial that includes a comparison (control) group. [NIH]

Convulsions: A general term referring to sudden and often violent motor activity of cerebral or brainstem origin. Convulsions may also occur in the absence of an electrical cerebral discharge (e.g., in response to hypotension). [NIH] Coordination: Muscular or motor regulation or the harmonious cooperation of muscles or groups of muscles, in a complex action or series of actions. [NIH] Corpus: The body of the uterus. [NIH] Corpus Striatum: Striped gray and white matter consisting of the neostriatum and paleostriatum (globus pallidus). It is located in front of and lateral to the thalamus in each cerebral hemisphere. The gray substance is made up of the caudate nucleus and the lentiform nucleus (the latter consisting of the globus pallidus and putamen). The white matter is the internal capsule. [NIH] Corpuscle: A small mass or body; a sensory nerve end bulb; a cell, especially that of the blood or the lymph. [NIH] Cortex: The outer layer of an organ or other body structure, as distinguished from the internal substance. [EU] Cortical: Pertaining to or of the nature of a cortex or bark. [EU] Cortices: The outer layer of an organ; used especially of the cerebrum and cerebellum. [NIH] Corticosteroids: Hormones that have antitumor activity in lymphomas and lymphoid leukemias; in addition, corticosteroids (steroids) may be used for hormone replacement and for the management of some of the complications of cancer and its treatment. [NIH] Cortisone: A natural steroid hormone produced in the adrenal gland. It can also be made in the laboratory. Cortisone reduces swelling and can suppress immune responses. [NIH] Cranial: Pertaining to the cranium, or to the anterior (in animals) or superior (in humans) end of the body. [EU]

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Cranial Nerves: Twelve pairs of nerves that carry general afferent, visceral afferent, special afferent, somatic efferent, and autonomic efferent fibers. [NIH] Craniocerebral Trauma: Traumatic injuries involving the cranium and intracranial structures (i.e., brain; cranial nerves; meninges; and other structures). Injuries may be classified by whether or not the skull is penetrated (i.e., penetrating vs. nonpenetrating) or whether there is an associated hemorrhage. [NIH] Cribriform: Pierced with small holes as in a sieve. Refers to the appearance of a tumor when viewed under a microscope. The tumor appears to have open spaces or small holes inside. [NIH]

Crossing-over: The exchange of corresponding segments between chromatids of homologous chromosomes during meiosia, forming a chiasma. [NIH] Crowns: A prosthetic restoration that reproduces the entire surface anatomy of the visible natural crown of a tooth. It may be partial (covering three or more surfaces of a tooth) or complete (covering all surfaces). It is made of gold or other metal, porcelain, or resin. [NIH] Curative: Tending to overcome disease and promote recovery. [EU] Cutaneous: Having to do with the skin. [NIH] Cyclic: Pertaining to or occurring in a cycle or cycles; the term is applied to chemical compounds that contain a ring of atoms in the nucleus. [EU] Cyclopia: Elements of the two eyes fused into one median eye in the center of the forehead of a fetal monster. [NIH] Cytochrome: Any electron transfer hemoprotein having a mode of action in which the transfer of a single electron is effected by a reversible valence change of the central iron atom of the heme prosthetic group between the +2 and +3 oxidation states; classified as cytochromes a in which the heme contains a formyl side chain, cytochromes b, which contain protoheme or a closely similar heme that is not covalently bound to the protein, cytochromes c in which protoheme or other heme is covalently bound to the protein, and cytochromes d in which the iron-tetrapyrrole has fewer conjugated double bonds than the hemes have. Well-known cytochromes have been numbered consecutively within groups and are designated by subscripts (beginning with no subscript), e.g. cytochromes c, c1, C2, . New cytochromes are named according to the wavelength in nanometres of the absorption maximum of the a-band of the iron (II) form in pyridine, e.g., c-555. [EU] Cytokine: Small but highly potent protein that modulates the activity of many cell types, including T and B cells. [NIH] Cytoplasm: The protoplasm of a cell exclusive of that of the nucleus; it consists of a continuous aqueous solution (cytosol) and the organelles and inclusions suspended in it (phaneroplasm), and is the site of most of the chemical activities of the cell. [EU] Cytoskeleton: The network of filaments, tubules, and interconnecting filamentous bridges which give shape, structure, and organization to the cytoplasm. [NIH] Cytotoxic: Cell-killing. [NIH] Decarboxylation: The removal of a carboxyl group, usually in the form of carbon dioxide, from a chemical compound. [NIH] Degenerative: Undergoing degeneration : tending to degenerate; having the character of or involving degeneration; causing or tending to cause degeneration. [EU] Deletion: A genetic rearrangement through loss of segments of DNA (chromosomes), bringing sequences, which are normally separated, into close proximity. [NIH] Delirium: (DSM III-R) an acute, reversible organic mental disorder characterized by reduced

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ability to maintain attention to external stimuli and disorganized thinking as manifested by rambling, irrelevant, or incoherent speech; there are also a reduced level of consciousness, sensory misperceptions, disturbance of the sleep-wakefulness cycle and level of psychomotor activity, disorientation to time, place, or person, and memory impairment. Delirium may be caused by a large number of conditions resulting in derangement of cerebral metabolism, including systemic infection, poisoning, drug intoxication or withdrawal, seizures or head trauma, and metabolic disturbances such as hypoxia, hypoglycaemia, fluid, electrolyte, or acid-base imbalances, or hepatic or renal failure. Called also acute confusional state and acute brain syndrome. [EU] Delusions: A false belief regarding the self or persons or objects outside the self that persists despite the facts, and is not considered tenable by one's associates. [NIH] Dementia: An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is usually progressive, and initially spares the level of consciousness. [NIH] Denaturation: Rupture of the hydrogen bonds by heating a DNA solution and then cooling it rapidly causes the two complementary strands to separate. [NIH] Dendrites: Extensions of the nerve cell body. They are short and branched and receive stimuli from other neurons. [NIH] Dendritic: 1. Branched like a tree. 2. Pertaining to or possessing dendrites. [EU] Dental Caries: Localized destruction of the tooth surface initiated by decalcification of the enamel followed by enzymatic lysis of organic structures and leading to cavity formation. If left unchecked, the cavity may penetrate the enamel and dentin and reach the pulp. The three most prominent theories used to explain the etiology of the disase are that acids produced by bacteria lead to decalcification; that micro-organisms destroy the enamel protein; or that keratolytic micro-organisms produce chelates that lead to decalcification. [NIH]

Dentate Gyrus: Gray matter situated above the gyrus hippocampi. It is composed of three layers. The molecular layer is continuous with the hippocampus in the hippocampal fissure. The granular layer consists of closely arranged spherical or oval neurons, called granule cells, whose axons pass through the polymorphic layer ending on the dendrites of pyramidal cells in the hippocampus. [NIH] Depolarization: The process or act of neutralizing polarity. In neurophysiology, the reversal of the resting potential in excitable cell membranes when stimulated, i.e., the tendency of the cell membrane potential to become positive with respect to the potential outside the cell. [EU] DES: Diethylstilbestrol. A synthetic hormone that was prescribed from the early 1940s until 1971 to help women with complications of pregnancy. DES has been linked to an increased risk of clear cell carcinoma of the vagina in daughters of women who used DES. DES may also increase the risk of breast cancer in women who used DES. [NIH] Desensitization: The prevention or reduction of immediate hypersensitivity reactions by administration of graded doses of allergen; called also hyposensitization and immunotherapy. [EU] Dextroamphetamine: The d-form of amphetamine. It is a central nervous system stimulant and a sympathomimetic. It has also been used in the treatment of narcolepsy and of attention deficit disorders and hyperactivity in children. Dextroamphetamine has multiple mechanisms of action including blocking uptake of adrenergics and dopamine, stimulating release of monamines, and inhibiting monoamine oxidase. It is also a drug of abuse and a

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psychotomimetic. [NIH] Diabetes Mellitus: A heterogeneous group of disorders that share glucose intolerance in common. [NIH] Diagnostic procedure: A method used to identify a disease. [NIH] Diaphragm: The musculofibrous partition that separates the thoracic cavity from the abdominal cavity. Contraction of the diaphragm increases the volume of the thoracic cavity aiding inspiration. [NIH] Digestion: The process of breakdown of food for metabolism and use by the body. [NIH] Dilated cardiomyopathy: Heart muscle disease that leads to enlargement of the heart's chambers, robbing the heart of its pumping ability. [NIH] Dilation: A process by which the pupil is temporarily enlarged with special eye drops (mydriatic); allows the eye care specialist to better view the inside of the eye. [NIH] Direct: 1. Straight; in a straight line. 2. Performed immediately and without the intervention of subsidiary means. [EU] Disease Susceptibility: A constitution or condition of the body which makes the tissues react in special ways to certain extrinsic stimuli and thus tends to make the individual more than usually susceptible to certain diseases. [NIH] Disorientation: The loss of proper bearings, or a state of mental confusion as to time, place, or identity. [EU] Dissociation: 1. The act of separating or state of being separated. 2. The separation of a molecule into two or more fragments (atoms, molecules, ions, or free radicals) produced by the absorption of light or thermal energy or by solvation. 3. In psychology, a defense mechanism in which a group of mental processes are segregated from the rest of a person's mental activity in order to avoid emotional distress, as in the dissociative disorders (q.v.), or in which an idea or object is segregated from its emotional significance; in the first sense it is roughly equivalent to splitting, in the second, to isolation. 4. A defect of mental integration in which one or more groups of mental processes become separated off from normal consciousness and, thus separated, function as a unitary whole. [EU] Dizziness: An imprecise term which may refer to a sense of spatial disorientation, motion of the environment, or lightheadedness. [NIH] Dopa: The racemic or DL form of DOPA, an amino acid found in various legumes. The dextro form has little physiologic activity but the levo form (levodopa) is a very important physiologic mediator and precursor and pharmacological agent. [NIH] Dopamine: An endogenous catecholamine and prominent neurotransmitter in several systems of the brain. In the synthesis of catecholamines from tyrosine, it is the immediate precursor to norepinephrine and epinephrine. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. A family of dopaminergic receptor subtypes mediate its action. Dopamine is used pharmacologically for its direct (beta adrenergic agonist) and indirect (adrenergic releasing) sympathomimetic effects including its actions as an inotropic agent and as a renal vasodilator. [NIH] Dopamine Agonists: Drugs that bind to and activate dopamine receptors. [NIH] Dorsal: 1. Pertaining to the back or to any dorsum. 2. Denoting a position more toward the back surface than some other object of reference; same as posterior in human anatomy; superior in the anatomy of quadrupeds. [EU] Dorsum: A plate of bone which forms the posterior boundary of the sella turcica. [NIH] Double-blind: Pertaining to a clinical trial or other experiment in which neither the subject

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nor the person administering treatment knows which treatment any particular subject is receiving. [EU] Drug Interactions: The action of a drug that may affect the activity, metabolism, or toxicity of another drug. [NIH] Duct: A tube through which body fluids pass. [NIH] Dura mater: The outermost, toughest, and most fibrous of the three membranes (meninges) covering the brain and spinal cord; called also pachymeninx. [EU] Dysarthria: Imperfect articulation of speech due to disturbances of muscular control which result from damage to the central or peripheral nervous system. [EU] Dyskinesia: Impairment of the power of voluntary movement, resulting in fragmentary or incomplete movements. [EU] Dyslexia: Partial alexia in which letters but not words may be read, or in which words may be read but not understood. [NIH] Dysphonia: Difficulty or pain in speaking; impairment of the voice. [NIH] Dystonia: Disordered tonicity of muscle. [EU] Dystrophin: A muscle protein localized in surface membranes which is the product of the Duchenne/Becker muscular dystrophy gene. Individuals with Duchenne muscular dystrophy usually lack dystrophin completely while those with Becker muscular dystrophy have dystrophin of an altered size. It shares features with other cytoskeletal proteins such as spectrin and alpha-actinin but the precise function of dystrophin is not clear. One possible role might be to preserve the integrity and alignment of the plasma membrane to the myofibrils during muscle contraction and relaxation. MW 400 kDa. [NIH] Echocardiography: Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. [NIH] Effector: It is often an enzyme that converts an inactive precursor molecule into an active second messenger. [NIH] Efferent: Nerve fibers which conduct impulses from the central nervous system to muscles and glands. [NIH] Efficacy: The extent to which a specific intervention, procedure, regimen, or service produces a beneficial result under ideal conditions. Ideally, the determination of efficacy is based on the results of a randomized control trial. [NIH] Electrolyte: A substance that dissociates into ions when fused or in solution, and thus becomes capable of conducting electricity; an ionic solute. [EU] Electromyography: Recording of the changes in electric potential of muscle by means of surface or needle electrodes. [NIH] Electrons: Stable elementary particles having the smallest known negative charge, present in all elements; also called negatrons. Positively charged electrons are called positrons. The numbers, energies and arrangement of electrons around atomic nuclei determine the chemical identities of elements. Beams of electrons are called cathode rays or beta rays, the latter being a high-energy biproduct of nuclear decay. [NIH] Elementary Particles: Individual components of atoms, usually subatomic; subnuclear particles are usually detected only when the atomic nucleus decays and then only transiently, as most of them are unstable, often yielding pure energy without substance, i.e., radiation. [NIH] Emaciation: Clinical manifestation of excessive leanness usually caused by disease or a lack of nutrition. [NIH]

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Embolus: Bit of foreign matter which enters the blood stream at one point and is carried until it is lodged or impacted in an artery and obstructs it. It may be a blood clot, an air bubble, fat or other tissue, or clumps of bacteria. [NIH] Emetic: An agent that causes vomiting. [EU] Emulsion: A preparation of one liquid distributed in small globules throughout the body of a second liquid. The dispersed liquid is the discontinuous phase, and the dispersion medium is the continuous phase. When oil is the dispersed liquid and an aqueous solution is the continuous phase, it is known as an oil-in-water emulsion, whereas when water or aqueous solution is the dispersed phase and oil or oleaginous substance is the continuous phase, it is known as a water-in-oil emulsion. Pharmaceutical emulsions for which official standards have been promulgated include cod liver oil emulsion, cod liver oil emulsion with malt, liquid petrolatum emulsion, and phenolphthalein in liquid petrolatum emulsion. [EU] Encephalitis: Inflammation of the brain due to infection, autoimmune processes, toxins, and other conditions. Viral infections (see encephalitis, viral) are a relatively frequent cause of this condition. [NIH] Encephalitis, Viral: Inflammation of brain parenchymal tissue as a result of viral infection. Encephalitis may occur as primary or secondary manifestation of Togaviridae infections; Herpesviridae infections; Adenoviridae infections; Flaviviridae infections; Bunyaviridae infections; Picornaviridae infections; Paramyxoviridae infections; Orthomyxoviridae infections; Retroviridae infections; and Arenaviridae infections. [NIH] Encephalocele: Cerebral tissue herniation through a congenital or acquired defect in the skull. The majority of congenital encephaloceles occur in the occipital or frontal regions. Clinical features include a protuberant mass that may be pulsatile. The quantity and location of protruding neural tissue determines the type and degree of neurologic deficit. Visual defects, psychomotor developmental delay, and persistent motor deficits frequently occur. [NIH]

Encephalopathy: A disorder of the brain that can be caused by disease, injury, drugs, or chemicals. [NIH] Endemic: Present or usually prevalent in a population or geographical area at all times; said of a disease or agent. Called also endemial. [EU] Endogenous: Produced inside an organism or cell. The opposite is external (exogenous) production. [NIH] Endorphins: One of the three major groups of endogenous opioid peptides. They are large peptides derived from the pro-opiomelanocortin precursor. The known members of this group are alpha-, beta-, and gamma-endorphin. The term endorphin is also sometimes used to refer to all opioid peptides, but the narrower sense is used here; opioid peptides is used for the broader group. [NIH] Endothelial cell: The main type of cell found in the inside lining of blood vessels, lymph vessels, and the heart. [NIH] Endothelium: A layer of epithelium that lines the heart, blood vessels (endothelium, vascular), lymph vessels (endothelium, lymphatic), and the serous cavities of the body. [NIH] Endothelium-derived: Small molecule that diffuses to the adjacent muscle layer and relaxes it. [NIH] Endotoxic: Of, relating to, or acting as an endotoxin (= a heat-stable toxin, associated with the outer membranes of certain gram-negative bacteria. Endotoxins are not secreted and are released only when the cells are disrupted). [EU] Enkephalin: A natural opiate painkiller, in the hypothalamus. [NIH]

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Entorhinal Cortex: Cortex where the signals are combined with those from other sensory systems. [NIH] Environmental Health: The science of controlling or modifying those conditions, influences, or forces surrounding man which relate to promoting, establishing, and maintaining health. [NIH]

Enzymatic: Phase where enzyme cuts the precursor protein. [NIH] Enzyme: A protein that speeds up chemical reactions in the body. [NIH] Eosinophilia: Abnormal increase in eosinophils in the blood, tissues or organs. [NIH] Eosinophils: Granular leukocytes with a nucleus that usually has two lobes connected by a slender thread of chromatin, and cytoplasm containing coarse, round granules that are uniform in size and stainable by eosin. [NIH] Epidemic: Occurring suddenly in numbers clearly in excess of normal expectancy; said especially of infectious diseases but applied also to any disease, injury, or other healthrelated event occurring in such outbreaks. [EU] Epinephrine: The active sympathomimetic hormone from the adrenal medulla in most species. It stimulates both the alpha- and beta- adrenergic systems, causes systemic vasoconstriction and gastrointestinal relaxation, stimulates the heart, and dilates bronchi and cerebral vessels. It is used in asthma and cardiac failure and to delay absorption of local anesthetics. [NIH] Epistaxis: Bleeding from the nose. [NIH] Ergot: Cataract due to ergot poisoning caused by eating of rye cereals contaminated by a fungus. [NIH] Ergot Alkaloids: Alkaloids isolated from the ergot fungus Claviceps purpurea (Hypocreaceae). The ergot alkaloids were the first alpha-adrenergic antagonists discovered, but side effects generally prevent their administration in doses that would produce more than a minimal blockade in humans. Their smooth muscle-stimulating activities may be attributed to alpha-agonistic properties, thus characterizing these alkaloids as a series of partial agonists. They have many clinical applications, notably in obstetrics and the treatment of migraine. (From Martindale, The Extra Pharmacopoeia, 28th ed, p662). [NIH] Erythema: Redness of the skin produced by congestion of the capillaries. This condition may result from a variety of causes. [NIH] Erythrocyte Membrane: The semipermeable outer portion of the red corpuscle. It is known as a 'ghost' after hemolysis. [NIH] Erythrocytes: Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing hemoglobin whose function is to transport oxygen. [NIH] Erythromycin: A bacteriostatic antibiotic substance produced by Streptomyces erythreus. Erythromycin A is considered its major active component. In sensitive organisms, it inhibits protein synthesis by binding to 50S ribosomal subunits. This binding process inhibits peptidyl transferase activity and interferes with translocation of amino acids during translation and assembly of proteins. [NIH] Estrogen: One of the two female sex hormones. [NIH] Ethmoid: An unpaired cranial bone which helps form the medial walls of the orbits and contains the themoidal air cells which drain into the nose. [NIH] Eukaryotic Cells: Cells of the higher organisms, containing a true nucleus bounded by a nuclear membrane. [NIH] Evoke: The electric response recorded from the cerebral cortex after stimulation of a

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peripheral sense organ. [NIH] Excitability: Property of a cardiac cell whereby, when the cell is depolarized to a critical level (called threshold), the membrane becomes permeable and a regenerative inward current causes an action potential. [NIH] Excitation: An act of irritation or stimulation or of responding to a stimulus; the addition of energy, as the excitation of a molecule by absorption of photons. [EU] Excitatory: When cortical neurons are excited, their output increases and each new input they receive while they are still excited raises their output markedly. [NIH] Excitatory Amino Acid Agonists: Drugs that bind to and activate excitatory amino acid receptors. [NIH] Exogenous: Developed or originating outside the organism, as exogenous disease. [EU] Exon: The part of the DNA that encodes the information for the actual amino acid sequence of the protein. In many eucaryotic genes, the coding sequences consist of a series of exons alternating with intron sequences. [NIH] Exotoxin: Toxic substance excreted by living bacterial cells. [NIH] Expiratory: The volume of air which leaves the breathing organs in each expiration. [NIH] Exploratory Behavior: The tendency to explore or investigate a novel environment. It is considered a motivation not clearly distinguishable from curiosity. [NIH] Extracellular: Outside a cell or cells. [EU] Extracellular Matrix: A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere. [NIH] Extrapyramidal: Outside of the pyramidal tracts. [EU] Extravasation: A discharge or escape, as of blood, from a vessel into the tissues. [EU] Eye Movements: Voluntary or reflex-controlled movements of the eye. [NIH] Facial: Of or pertaining to the face. [EU] Facial Pain: Pain in the facial region including orofacial pain and craniofacial pain. Associated conditions include local inflammatory and neoplastic disorders and neuralgic syndromes involving the trigeminal, facial, and glossopharyngeal nerves. Conditions which feature recurrent or persistent facial pain as the primary manifestation of disease are referred to as facial pain syndromes. [NIH] Facial Paralysis: Severe or complete loss of facial muscle motor function. This condition may result from central or peripheral lesions. Damage to CNS motor pathways from the cerebral cortex to the facial nuclei in the pons leads to facial weakness that generally spares the forehead muscles. Facial nerve diseases generally results in generalized hemifacial weakness. Neuromuscular junction diseases and muscular diseases may also cause facial paralysis or paresis. [NIH] Family Planning: Programs or services designed to assist the family in controlling reproduction by either improving or diminishing fertility. [NIH] Famotidine: A competitive histamine H2-receptor antagonist. Its main pharmacodynamic effect is the inhibition of gastric secretion. [NIH] Fat: Total lipids including phospholipids. [NIH] Femoral: Pertaining to the femur, or to the thigh. [EU] Femoral Artery: The main artery of the thigh, a continuation of the external iliac artery. [NIH]

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Fetus: The developing offspring from 7 to 8 weeks after conception until birth. [NIH] Fibrin: A protein derived from fibrinogen in the presence of thrombin, which forms part of the blood clot. [NIH] Fibroblasts: Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules. [NIH] Fibrosis: Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. [NIH] Fissure: Any cleft or groove, normal or otherwise; especially a deep fold in the cerebral cortex which involves the entire thickness of the brain wall. [EU] Flow Cytometry: Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake. [NIH] Fludrocortisone: A synthetic mineralocorticoid with anti-inflammatory activity. [NIH] Fluorescence: The property of emitting radiation while being irradiated. The radiation emitted is usually of longer wavelength than that incident or absorbed, e.g., a substance can be irradiated with invisible radiation and emit visible light. X-ray fluorescence is used in diagnosis. [NIH] Fluorescent Dyes: Dyes that emit light when exposed to light. The wave length of the emitted light is usually longer than that of the incident light. Fluorochromes are substances that cause fluorescence in other substances, i.e., dyes used to mark or label other compounds with fluorescent tags. They are used as markers in biochemistry and immunology. [NIH] Fluorine: A nonmetallic, diatomic gas that is a trace element and member of the halogen family. It is used in dentistry as flouride to prevent dental caries. [NIH] Fossa: A cavity, depression, or pit. [NIH] Frontal Lobe: The anterior part of the cerebral hemisphere. [NIH] Gait: Manner or style of walking. [NIH] Gallbladder: The pear-shaped organ that sits below the liver. Bile is concentrated and stored in the gallbladder. [NIH] Gamma Rays: Very powerful and penetrating, high-energy electromagnetic radiation of shorter wavelength than that of x-rays. They are emitted by a decaying nucleus, usually between 0.01 and 10 MeV. They are also called nuclear x-rays. [NIH] Gamma-hydroxybutyrate: Anxiolytic. [NIH] Ganglia: Clusters of multipolar neurons surrounded by a capsule of loosely organized connective tissue located outside the central nervous system. [NIH] Gap Junctions: Connections between cells which allow passage of small molecules and electric current. Gap junctions were first described anatomically as regions of close apposition between cells with a narrow (1-2 nm) gap between cell membranes. The variety in the properties of gap junctions is reflected in the number of connexins, the family of proteins which form the junctions. [NIH] Gas: Air that comes from normal breakdown of food. The gases are passed out of the body

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through the rectum (flatus) or the mouth (burp). [NIH] Gasoline: Volative flammable fuel (liquid hydrocarbons) derived from crude petroleum by processes such as distillation reforming, polymerization, etc. [NIH] Gastric: Having to do with the stomach. [NIH] Gastrin: A hormone released after eating. Gastrin causes the stomach to produce more acid. [NIH]

Gastrointestinal: Refers to the stomach and intestines. [NIH] Gastrointestinal tract: The stomach and intestines. [NIH] Gene: The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein. [NIH]

Gene Expression: The phenotypic manifestation of a gene or genes by the processes of gene action. [NIH] Gene Expression Profiling: The determination of the pattern of genes expressed i.e., transcribed, under specific circumstances or in a specific cell. [NIH] Gene Targeting: The integration of exogenous DNA into the genome of an organism at sites where its expression can be suitably controlled. This integration occurs as a result of homologous recombination. [NIH] Genetic Engineering: Directed modification of the gene complement of a living organism by such techniques as altering the DNA, substituting genetic material by means of a virus, transplanting whole nuclei, transplanting cell hybrids, etc. [NIH] Genetic testing: Analyzing DNA to look for a genetic alteration that may indicate an increased risk for developing a specific disease or disorder. [NIH] Genetics: The biological science that deals with the phenomena and mechanisms of heredity. [NIH] Genotype: The genetic constitution of the individual; the characterization of the genes. [NIH] Germ Cells: The reproductive cells in multicellular organisms. [NIH] Gland: An organ that produces and releases one or more substances for use in the body. Some glands produce fluids that affect tissues or organs. Others produce hormones or participate in blood production. [NIH] Gliosis: The production of a dense fibrous network of neuroglia; includes astrocytosis, which is a proliferation of astrocytes in the area of a degenerative lesion. [NIH] Globus Pallidus: The representation of the phylogenetically oldest part of the corpus striatum called the paleostriatum. It forms the smaller, more medial part of the lentiform nucleus. [NIH] Glomerular: Pertaining to or of the nature of a glomerulus, especially a renal glomerulus. [EU]

Glomeruli: Plural of glomerulus. [NIH] Glomerulonephritis: Glomerular disease characterized by an inflammatory reaction, with leukocyte infiltration and cellular proliferation of the glomeruli, or that appears to be the result of immune glomerular injury. [NIH] Glomerulus: A tiny set of looping blood vessels in the nephron where blood is filtered in the kidney. [NIH] Glossopharyngeal Nerve: The 9th cranial nerve. The glossopharyngeal nerve is a mixed motor and sensory nerve; it conveys somatic and autonomic efferents as well as general,

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special, and visceral afferents. Among the connections are motor fibers to the stylopharyngeus muscle, parasympathetic fibers to the parotid glands, general and taste afferents from the posterior third of the tongue, the nasopharynx, and the palate, and afferents from baroreceptors and chemoreceptors of the carotid sinus. [NIH] Glottis: The vocal apparatus of the larynx, consisting of the true vocal cords (plica vocalis) and the opening between them (rima glottidis). [NIH] Glucocorticoid: A compound that belongs to the family of compounds called corticosteroids (steroids). Glucocorticoids affect metabolism and have anti-inflammatory and immunosuppressive effects. They may be naturally produced (hormones) or synthetic (drugs). [NIH] Glucose: D-Glucose. A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. [NIH] Glucose Intolerance: A pathological state in which the fasting plasma glucose level is less than 140 mg per deciliter and the 30-, 60-, or 90-minute plasma glucose concentration following a glucose tolerance test exceeds 200 mg per deciliter. This condition is seen frequently in diabetes mellitus but also occurs with other diseases. [NIH] Glutamate: Excitatory neurotransmitter of the brain. [NIH] Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid (glutamate) is the most common excitatory neurotransmitter in the central nervous system. [NIH]

Glycerol: A trihydroxy sugar alcohol that is an intermediate in carbohydrate and lipid metabolism. It is used as a solvent, emollient, pharmaceutical agent, and sweetening agent. [NIH]

Glycine: A non-essential amino acid. It is found primarily in gelatin and silk fibroin and used therapeutically as a nutrient. It is also a fast inhibitory neurotransmitter. [NIH] Glycoproteins: Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins. [NIH] Gonadal: Pertaining to a gonad. [EU] Governing Board: The group in which legal authority is vested for the control of healthrelated institutions and organizations. [NIH] Gram-positive: Retaining the stain or resisting decolorization by alcohol in Gram's method of staining, a primary characteristic of bacteria whose cell wall is composed of a thick layer of peptidologlycan with attached teichoic acids. [EU] Granulocytes: Leukocytes with abundant granules in the cytoplasm. They are divided into three groups: neutrophils, eosinophils, and basophils. [NIH] Gravis: Eruption of watery blisters on the skin among those handling animals and animal products. [NIH] Growth factors: Substances made by the body that function to regulate cell division and cell survival. Some growth factors are also produced in the laboratory and used in biological therapy. [NIH] Guanidines: A family of iminourea derivatives. The parent compound has been isolated from mushrooms, corn germ, rice hulls, mussels, earthworms, and turnip juice. Derivatives may have antiviral and antifungal properties. [NIH] Guanylate Cyclase: An enzyme that catalyzes the conversion of GTP to 3',5'-cyclic GMP and pyrophosphate. It also acts on ITP and dGTP. (From Enzyme Nomenclature, 1992) EC 4.6.1.2. [NIH]

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Haematoma: A localized collection of blood, usually clotted, in an organ, space, or tissue, due to a break in the wall of a blood vessel. [EU] Haemorrhage: The escape of blood from the vessels; bleeding. Small haemorrhages are classified according to size as petechiae (very small), purpura (up to 1 cm), and ecchymoses (larger). The massive accumulation of blood within a tissue is called a haematoma. [EU] Haloperidol: Butyrophenone derivative. [NIH] Haptens: Small antigenic determinants capable of eliciting an immune response only when coupled to a carrier. Haptens bind to antibodies but by themselves cannot elicit an antibody response. [NIH] Headache: Pain in the cranial region that may occur as an isolated and benign symptom or as a manifestation of a wide variety of conditions including subarachnoid hemorrhage; craniocerebral trauma; central nervous system infections; intracranial hypertension; and other disorders. In general, recurrent headaches that are not associated with a primary disease process are referred to as headache disorders (e.g., migraine). [NIH] Headache Disorders: Common conditions characterized by persistent or recurrent headaches. Headache syndrome classification systems may be based on etiology (e.g., vascular headache, post-traumatic headaches, etc.), temporal pattern (e.g., cluster headache, paroxysmal hemicrania, etc.), and precipitating factors (e.g., cough headache). [NIH] Heart failure: Loss of pumping ability by the heart, often accompanied by fatigue, breathlessness, and excess fluid accumulation in body tissues. [NIH] Heart Murmurs: Abnormal heart sounds heard during auscultation caused by alterations in the flow of blood into a chamber, through a valve, or by a valve opening or closing abnormally. They are classified by the time of occurrence during the cardiac cycle, the duration, and the intensity of the sound on a scale of I to V. [NIH] Heart Sounds: The sounds heard over the cardiac region produced by the functioning of the heart. There are four distinct sounds: the first occurs at the beginning of systole and is heard as a "lubb" sound; the second is produced by the closing of the aortic and pulmonary valves and is heard as a "dupp" sound; the third is produced by vibrations of the ventricular walls when suddenly distended by the rush of blood from the atria; and the fourth is produced by atrial contraction and ventricular filling but is rarely audible in the normal heart. The physiological concept of heart sounds is differentiated from the pathological heart murmurs. [NIH]

Hematoma: An extravasation of blood localized in an organ, space, or tissue. [NIH] Hemiplegia: Severe or complete loss of motor function on one side of the body. This condition is usually caused by BRAIN DISEASES that are localized to the cerebral hemisphere opposite to the side of weakness. Less frequently, BRAIN STEM lesions; cervical spinal cord diseases; peripheral nervous system diseases; and other conditions may manifest as hemiplegia. The term hemiparesis (see paresis) refers to mild to moderate weakness involving one side of the body. [NIH] Hemoglobin: One of the fractions of glycosylated hemoglobin A1c. Glycosylated hemoglobin is formed when linkages of glucose and related monosaccharides bind to hemoglobin A and its concentration represents the average blood glucose level over the previous several weeks. HbA1c levels are used as a measure of long-term control of plasma glucose (normal, 4 to 6 percent). In controlled diabetes mellitus, the concentration of glycosylated hemoglobin A is within the normal range, but in uncontrolled cases the level may be 3 to 4 times the normal conentration. Generally, complications are substantially lower among patients with Hb levels of 7 percent or less than in patients with HbA1c levels of 9 percent or more. [NIH]

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Hemolysis: The destruction of erythrocytes by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity. [NIH] Hemolytic: A disease that affects the blood and blood vessels. It destroys red blood cells, cells that cause the blood to clot, and the lining of blood vessels. HUS is often caused by the Escherichia coli bacterium in contaminated food. People with HUS may develop acute renal failure. [NIH] Hemorrhage: Bleeding or escape of blood from a vessel. [NIH] Hemostasis: The process which spontaneously arrests the flow of blood from vessels carrying blood under pressure. It is accomplished by contraction of the vessels, adhesion and aggregation of formed blood elements, and the process of blood or plasma coagulation. [NIH]

Hepatic: Refers to the liver. [NIH] Hereditary: Of, relating to, or denoting factors that can be transmitted genetically from one generation to another. [NIH] Heredity: 1. The genetic transmission of a particular quality or trait from parent to offspring. 2. The genetic constitution of an individual. [EU] Hermetic: Impervious to air; airtight. [EU] Heterogeneity: The property of one or more samples or populations which implies that they are not identical in respect of some or all of their parameters, e. g. heterogeneity of variance. [NIH]

Hippocampus: A curved elevation of gray matter extending the entire length of the floor of the temporal horn of the lateral ventricle (Dorland, 28th ed). The hippocampus, subiculum, and dentate gyrus constitute the hippocampal formation. Sometimes authors include the entorhinal cortex in the hippocampal formation. [NIH] Histamine: 1H-Imidazole-4-ethanamine. A depressor amine derived by enzymatic decarboxylation of histidine. It is a powerful stimulant of gastric secretion, a constrictor of bronchial smooth muscle, a vasodilator, and also a centrally acting neurotransmitter. [NIH] Histidine: An essential amino acid important in a number of metabolic processes. It is required for the production of histamine. [NIH] Holoprosencephaly: Anterior midline brain, cranial, and facial malformations resulting from the failure of the embryonic prosencephalon to undergo segmentation and cleavage. Alobar prosencephaly is the most severe form and features anophthalmia; cyclopia; severe mental retardation; cleft lip; cleft palate; seizures; and microcephaly. Semilobar holoprosencepaly is characterized by hypotelorism, microphthalmia, coloboma, nasal malformations, and variable degrees of mental retardation. Lobar holoprosencephaly is associated with mild (or absent) facial malformations and intellectual abilities that range from mild mental retardation to normal. Holoprosencephlay is associated with chromosome abnormalities. [NIH] Homeobox: Distinctive sequence of DNA bases. [NIH] Homologous: Corresponding in structure, position, origin, etc., as (a) the feathers of a bird and the scales of a fish, (b) antigen and its specific antibody, (c) allelic chromosomes. [EU] Hormonal: Pertaining to or of the nature of a hormone. [EU] Hormone: A substance in the body that regulates certain organs. Hormones such as gastrin help in breaking down food. Some hormones come from cells in the stomach and small intestine. [NIH] Human growth hormone: A protein hormone, secreted by the anterior lobe of the pituitary, which promotes growth of the whole body by stimulating protein synthesis. The human

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gene has already been cloned and successfully expressed in bacteria. [NIH] Hybrid: Cross fertilization between two varieties or, more usually, two species of vines, see also crossing. [NIH] Hybridization: The genetic process of crossbreeding to produce a hybrid. Hybrid nucleic acids can be formed by nucleic acid hybridization of DNA and RNA molecules. Protein hybridization allows for hybrid proteins to be formed from polypeptide chains. [NIH] Hydrocephalus: Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, intracranial hypertension; headache; lethargy; urinary incontinence; and ataxia (and in infants macrocephaly). This condition may be caused by obstruction of cerebrospinal fluid pathways due to neurologic abnormalities, intracranial hemorrhages; central nervous system infections; brain neoplasms; craniocerebral trauma; and other conditions. Impaired resorption of cerebrospinal fluid from the arachnoid villi results in a communicating form of hydrocephalus. Hydrocephalus ex-vacuo refers to ventricular dilation that occurs as a result of brain substance loss from cerebral infarction and other conditions. [NIH] Hydrogen: The first chemical element in the periodic table. It has the atomic symbol H, atomic number 1, and atomic weight 1. It exists, under normal conditions, as a colorless, odorless, tasteless, diatomic gas. Hydrogen ions are protons. Besides the common H1 isotope, hydrogen exists as the stable isotope deuterium and the unstable, radioactive isotope tritium. [NIH] Hydrolysis: The process of cleaving a chemical compound by the addition of a molecule of water. [NIH] Hyperglycaemia: Abnormally increased content of sugar in the blood. [EU] Hyperglycemia: Abnormally high blood sugar. [NIH] Hyperkinesia: Abnormally increased motor function or activity; hyperactivity. [EU] Hyperplasia: An increase in the number of cells in a tissue or organ, not due to tumor formation. It differs from hypertrophy, which is an increase in bulk without an increase in the number of cells. [NIH] Hyperreflexia: Exaggeration of reflexes. [EU] Hypersensitivity: Altered reactivity to an antigen, which can result in pathologic reactions upon subsequent exposure to that particular antigen. [NIH] Hypertension: Persistently high arterial blood pressure. Currently accepted threshold levels are 140 mm Hg systolic and 90 mm Hg diastolic pressure. [NIH] Hyperthyroidism: Excessive functional activity of the thyroid gland. [NIH] Hypertrophy: General increase in bulk of a part or organ, not due to tumor formation, nor to an increase in the number of cells. [NIH] Hypnotherapy: Sleeping-cure. [NIH] Hypoglossal Nerve: The 12th cranial nerve. The hypoglossal nerve originates in the hypoglossal nucleus of the medulla and supplies motor innervation to all of the muscles of the tongue except the palatoglossus (which is supplied by the vagus). This nerve also contains proprioceptive afferents from the tongue muscles. [NIH] Hypoglycaemia: An abnormally diminished concentration of glucose in the blood, which may lead to tremulousness, cold sweat, piloerection, hypothermia, and headache, accompanied by irritability, confusion, hallucinations, bizarre behaviour, and ultimately, convulsions and coma. [EU] Hypokinesia: Slow or diminished movement of body musculature. It may be associated

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with basal ganglia diseases; mental disorders; prolonged inactivity due to illness; experimental protocols used to evaluate the physiologic effects of immobility; and other conditions. [NIH] Hypothalamic: Of or involving the hypothalamus. [EU] Hypothalamus: Ventral part of the diencephalon extending from the region of the optic chiasm to the caudal border of the mammillary bodies and forming the inferior and lateral walls of the third ventricle. [NIH] Hypothermia: Lower than normal body temperature, especially in warm-blooded animals; in man usually accidental or unintentional. [NIH] Hypoxia: Reduction of oxygen supply to tissue below physiological levels despite adequate perfusion of the tissue by blood. [EU] Hypoxic: Having too little oxygen. [NIH] Idiopathic: Describes a disease of unknown cause. [NIH] Imaging procedures: Methods of producing pictures of areas inside the body. [NIH] Imidazole: C3H4N2. The ring is present in polybenzimidazoles. [NIH] Immune Complex Diseases: Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides serum sickness and the arthus reaction, evidence supports a pathogenic role for immune complexes in many other systemic immunologic diseases including glomerulonephritis, systemic lupus erythematosus and polyarteritis nodosa. [NIH] Immune response: The activity of the immune system against foreign substances (antigens). [NIH]

Immune system: The organs, cells, and molecules responsible for the recognition and disposal of foreign ("non-self") material which enters the body. [NIH] Immunity: Nonsusceptibility to the invasive or pathogenic microorganisms or to the toxic effect of antigenic substances. [NIH]

effects

of

foreign

Immunodeficiency: The decreased ability of the body to fight infection and disease. [NIH] Immunogenic: Producing immunity; evoking an immune response. [EU] Immunologic: The ability of the antibody-forming system to recall a previous experience with an antigen and to respond to a second exposure with the prompt production of large amounts of antibody. [NIH] Immunology: The study of the body's immune system. [NIH] Immunotherapy: Manipulation of the host's immune system in treatment of disease. It includes both active and passive immunization as well as immunosuppressive therapy to prevent graft rejection. [NIH] Impairment: In the context of health experience, an impairment is any loss or abnormality of psychological, physiological, or anatomical structure or function. [NIH] In situ: In the natural or normal place; confined to the site of origin without invasion of neighbouring tissues. [EU] In Situ Hybridization: A technique that localizes specific nucleic acid sequences within intact chromosomes, eukaryotic cells, or bacterial cells through the use of specific nucleic acid-labeled probes. [NIH] In vitro: In the laboratory (outside the body). The opposite of in vivo (in the body). [NIH] In vivo: In the body. The opposite of in vitro (outside the body or in the laboratory). [NIH]

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Incision: A cut made in the body during surgery. [NIH] Incontinence: Inability to control the flow of urine from the bladder (urinary incontinence) or the escape of stool from the rectum (fecal incontinence). [NIH] Induction: The act or process of inducing or causing to occur, especially the production of a specific morphogenetic effect in the developing embryo through the influence of evocators or organizers, or the production of anaesthesia or unconsciousness by use of appropriate agents. [EU] Infarction: A pathological process consisting of a sudden insufficient blood supply to an area, which results in necrosis of that area. It is usually caused by a thrombus, an embolus, or a vascular torsion. [NIH] Infection: 1. Invasion and multiplication of microorganisms in body tissues, which may be clinically unapparent or result in local cellular injury due to competitive metabolism, toxins, intracellular replication, or antigen-antibody response. The infection may remain localized, subclinical, and temporary if the body's defensive mechanisms are effective. A local infection may persist and spread by extension to become an acute, subacute, or chronic clinical infection or disease state. A local infection may also become systemic when the microorganisms gain access to the lymphatic or vascular system. 2. An infectious disease. [EU]

Infertility: The diminished or absent ability to conceive or produce an offspring while sterility is the complete inability to conceive or produce an offspring. [NIH] Infiltration: The diffusion or accumulation in a tissue or cells of substances not normal to it or in amounts of the normal. Also, the material so accumulated. [EU] Inflammation: A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function. [NIH] Inhalation: The drawing of air or other substances into the lungs. [EU] Initiation: Mutation induced by a chemical reactive substance causing cell changes; being a step in a carcinogenic process. [NIH] Innervation: 1. The distribution or supply of nerves to a part. 2. The supply of nervous energy or of nerve stimulus sent to a part. [EU] Inositol: An isomer of glucose that has traditionally been considered to be a B vitamin although it has an uncertain status as a vitamin and a deficiency syndrome has not been identified in man. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1379) Inositol phospholipids are important in signal transduction. [NIH] Inotropic: Affecting the force or energy of muscular contractions. [EU] Insight: The capacity to understand one's own motives, to be aware of one's own psychodynamics, to appreciate the meaning of symbolic behavior. [NIH] Institutionalization: The caring for individuals in institutions and their adaptation to routines characteristic of the institutional environment, and/or their loss of adaptation to life outside the institution. [NIH] Insulator: Material covering the metal conductor of the lead. It is usually polyurethane or silicone. [NIH] Interferon: A biological response modifier (a substance that can improve the body's natural response to disease). Interferons interfere with the division of cancer cells and can slow tumor growth. There are several types of interferons, including interferon-alpha, -beta, and gamma. These substances are normally produced by the body. They are also made in the laboratory for use in treating cancer and other diseases. [NIH]

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Interferon-alpha: One of the type I interferons produced by peripheral blood leukocytes or lymphoblastoid cells when exposed to live or inactivated virus, double-stranded RNA, or bacterial products. It is the major interferon produced by virus-induced leukocyte cultures and, in addition to its pronounced antiviral activity, it causes activation of NK cells. [NIH] Interneurons: Most generally any neurons which are not motor or sensory. Interneurons may also refer to neurons whose axons remain within a particular brain region as contrasted with projection neurons which have axons projecting to other brain regions. [NIH] Interstitial: Pertaining to or situated between parts or in the interspaces of a tissue. [EU] Intestinal: Having to do with the intestines. [NIH] Intestine: A long, tube-shaped organ in the abdomen that completes the process of digestion. There is both a large intestine and a small intestine. Also called the bowel. [NIH] Intoxication: Poisoning, the state of being poisoned. [EU] Intracellular: Inside a cell. [NIH] Intracellular Membranes: Membranes of subcellular structures. [NIH] Intracranial Hemorrhages: Bleeding within the intracranial cavity, including hemorrhages in the brain and within the cranial epidural, subdural, and subarachnoid spaces. [NIH] Intracranial Hypertension: Increased pressure within the cranial vault. This may result from several conditions, including hydrocephalus; brain edema; intracranial masses; severe systemic hypertension; pseudotumor cerebri; and other disorders. [NIH] Intramuscular: IM. Within or into muscle. [NIH] Intramuscular injection: IM. Injection into a muscle. [NIH] Intrinsic: Situated entirely within or pertaining exclusively to a part. [EU] Invasive: 1. Having the quality of invasiveness. 2. Involving puncture or incision of the skin or insertion of an instrument or foreign material into the body; said of diagnostic techniques. [EU]

Involuntary: Reaction occurring without intention or volition. [NIH] Ion Channels: Gated, ion-selective glycoproteins that traverse membranes. The stimulus for channel gating can be a membrane potential, drug, transmitter, cytoplasmic messenger, or a mechanical deformation. Ion channels which are integral parts of ionotropic neurotransmitter receptors are not included. [NIH] Ions: An atom or group of atoms that have a positive or negative electric charge due to a gain (negative charge) or loss (positive charge) of one or more electrons. Atoms with a positive charge are known as cations; those with a negative charge are anions. [NIH] Ischemia: Deficiency of blood in a part, due to functional constriction or actual obstruction of a blood vessel. [EU] Kainic Acid: (2S-(2 alpha,3 beta,4 beta))-2-Carboxy-4-(1-methylethenyl)-3-pyrrolidineacetic acid. Ascaricide obtained from the red alga Digenea simplex. It is a potent excitatory amino acid agonist at some types of excitatory amino acid receptors and has been used to discriminate among receptor types. Like many excitatory amino acid agonists it can cause neurotoxicity and has been used experimentally for that purpose. [NIH] Kava: Dried rhizome and roots of Piper methysticum, a shrub native to Oceania and known for its anti-anxiety and sedative properties. Heavy usage results in some adverse effects. It contains alkaloids, lactones, kawain, methysticin, mucilage, starch, and yangonin. Kava is also the name of the pungent beverage prepared from the plant's roots. [NIH] Kb: A measure of the length of DNA fragments, 1 Kb = 1000 base pairs. The largest DNA

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fragments are up to 50 kilobases long. [NIH] Kinetic: Pertaining to or producing motion. [EU] Labyrinth: The internal ear; the essential part of the organ of hearing. It consists of an osseous and a membranous portion. [NIH] Laceration: 1. The act of tearing. 2. A torn, ragged, mangled wound. [EU] Lactation: The period of the secretion of milk. [EU] Larynx: An irregularly shaped, musculocartilaginous tubular structure, lined with mucous membrane, located at the top of the trachea and below the root of the tongue and the hyoid bone. It is the essential sphincter guarding the entrance into the trachea and functioning secondarily as the organ of voice. [NIH] Latency: The period of apparent inactivity between the time when a stimulus is presented and the moment a response occurs. [NIH] Lectin: A complex molecule that has both protein and sugars. Lectins are able to bind to the outside of a cell and cause biochemical changes in it. Lectins are made by both animals and plants. [NIH] Lens: The transparent, double convex (outward curve on both sides) structure suspended between the aqueous and vitreous; helps to focus light on the retina. [NIH] Lesion: An area of abnormal tissue change. [NIH] Lethargy: Abnormal drowsiness or stupor; a condition of indifference. [EU] Leukemia: Cancer of blood-forming tissue. [NIH] Levo: It is an experimental treatment for heroin addiction that was developed by German scientists around 1948 as an analgesic. Like methadone, it binds with opioid receptors, but it is longer acting. [NIH] Levodopa: The naturally occurring form of dopa and the immediate precursor of dopamine. Unlike dopamine itself, it can be taken orally and crosses the blood-brain barrier. It is rapidly taken up by dopaminergic neurons and converted to dopamine. It is used for the treatment of parkinsonism and is usually given with agents that inhibit its conversion to dopamine outside of the central nervous system. [NIH] Ligands: A RNA simulation method developed by the MIT. [NIH] Limbic: Pertaining to a limbus, or margin; forming a border around. [EU] Limbic System: A set of forebrain structures common to all mammals that is defined functionally and anatomically. It is implicated in the higher integration of visceral, olfactory, and somatic information as well as homeostatic responses including fundamental survival behaviors (feeding, mating, emotion). For most authors, it includes the amygdala, epithalamus, gyrus cinguli, hippocampal formation (see hippocampus), hypothalamus, parahippocampal gyrus, septal nuclei, anterior nuclear group of thalamus, and portions of the basal ganglia. (Parent, Carpenter's Human Neuroanatomy, 9th ed, p744; NeuroNames, http://rprcsgi.rprc.washington.edu/neuronames/index.html (September 2, 1998)). [NIH] Linkage: The tendency of two or more genes in the same chromosome to remain together from one generation to the next more frequently than expected according to the law of independent assortment. [NIH] Lipid: Fat. [NIH] Lipid A: Lipid A is the biologically active component of lipopolysaccharides. It shows strong endotoxic activity and exhibits immunogenic properties. [NIH] Lipopolysaccharides: Substance consisting of polysaccaride and lipid. [NIH]

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Liver: A large, glandular organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile. [NIH] Liver scan: An image of the liver created on a computer screen or on film. A radioactive substance is injected into a blood vessel and travels through the bloodstream. It collects in the liver, especially in abnormal areas, and can be detected by the scanner. [NIH] Lobe: A portion of an organ such as the liver, lung, breast, or brain. [NIH] Localization: The process of determining or marking the location or site of a lesion or disease. May also refer to the process of keeping a lesion or disease in a specific location or site. [NIH] Localized: Cancer which has not metastasized yet. [NIH] Locomotion: Movement or the ability to move from one place or another. It can refer to humans, vertebrate or invertebrate animals, and microorganisms. [NIH] Locomotor: Of or pertaining to locomotion; pertaining to or affecting the locomotive apparatus of the body. [EU] Longitudinal Studies: Studies in which variables relating to an individual or group of individuals are assessed over a period of time. [NIH] Longitudinal study: Also referred to as a "cohort study" or "prospective study"; the analytic method of epidemiologic study in which subsets of a defined population can be identified who are, have been, or in the future may be exposed or not exposed, or exposed in different degrees, to a factor or factors hypothesized to influence the probability of occurrence of a given disease or other outcome. The main feature of this type of study is to observe large numbers of subjects over an extended time, with comparisons of incidence rates in groups that differ in exposure levels. [NIH] Long-Term Potentiation: A persistent increase in synaptic efficacy, usually induced by appropriate activation of the same synapses. The phenomenological properties of long-term potentiation suggest that it may be a cellular mechanism of learning and memory. [NIH] Lupus: A form of cutaneous tuberculosis. It is seen predominantly in women and typically involves the nasal, buccal, and conjunctival mucosa. [NIH] Lutein Cells: The cells of the corpus luteum which are derived from the granulosa cells and the theca cells of the Graafian follicle. [NIH] Lymphatic: The tissues and organs, including the bone marrow, spleen, thymus, and lymph nodes, that produce and store cells that fight infection and disease. [NIH] Lymphatic system: The tissues and organs that produce, store, and carry white blood cells that fight infection and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes and a network of thin tubes that carry lymph and white blood cells. These tubes branch, like blood vessels, into all the tissues of the body. [NIH] Lymphoblastic: One of the most aggressive types of non-Hodgkin lymphoma. [NIH] Lymphoblasts: Interferon produced predominantly by leucocyte cells. [NIH] Lymphocyte Count: A count of the number of lymphocytes in the blood. [NIH] Lymphocytes: White blood cells formed in the body's lymphoid tissue. The nucleus is round or ovoid with coarse, irregularly clumped chromatin while the cytoplasm is typically pale blue with azurophilic (if any) granules. Most lymphocytes can be classified as either T or B (with subpopulations of each); those with characteristics of neither major class are called null cells. [NIH] Lymphoid: Referring to lymphocytes, a type of white blood cell. Also refers to tissue in which lymphocytes develop. [NIH]

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Lymphoma: A general term for various neoplastic diseases of the lymphoid tissue. [NIH] Lysergic acid: A compound close in chemical structure to LSD-25 but without hallucinogenic effects; one of the direct chemical predecessors of LSD-25. Sometimes LSD-25 is erroneously called by this name. [NIH] Lysergic Acid Diethylamide: Semisynthetic derivative of ergot (Claviceps purpurea). It has complex effects on serotonergic systems including antagonism at some peripheral serotonin receptors, both agonist and antagonist actions at central nervous system serotonin receptors, and possibly effects on serotonin turnover. It is a potent hallucinogen, but the mechanisms of that effect are not well understood. [NIH] Magnetic Resonance Imaging: Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. [NIH] Magnetic Resonance Spectroscopy: Spectroscopic method of measuring the magnetic moment of elementary particles such as atomic nuclei, protons or electrons. It is employed in clinical applications such as NMR Tomography (magnetic resonance imaging). [NIH] Malabsorption: Impaired intestinal absorption of nutrients. [EU] Malformation: A morphologic developmental process. [EU]

defect

resulting

from

an

intrinsically

abnormal

Malignant: Cancerous; a growth with a tendency to invade and destroy nearby tissue and spread to other parts of the body. [NIH] Malnutrition: A condition caused by not eating enough food or not eating a balanced diet. [NIH]

Mammogram: An x-ray of the breast. [NIH] Manganese Poisoning: A chronic neurological disease caused by prolonged exposure to manganese; occurs especially in miners, welders and workers in the primary production of manganese. [NIH] Mania: Excitement of psychotic proportions manifested by mental and physical hyperactivity, disorganization of behaviour, and elevation of mood. [EU] Manic: Affected with mania. [EU] Manic-depressive psychosis: One of a group of psychotic reactions, fundamentally marked by severe mood swings and a tendency to remission and recurrence. [NIH] Manifest: Being the part or aspect of a phenomenon that is directly observable : concretely expressed in behaviour. [EU] Mastication: The act and process of chewing and grinding food in the mouth. [NIH] Masticatory: 1. subserving or pertaining to mastication; affecting the muscles of mastication. 2. a remedy to be chewed but not swallowed. [EU] Meatus: A canal running from the internal auditory foramen through the petrous portion of the temporal bone. It gives passage to the facial and auditory nerves together with the auditory branch of the basilar artery and the internal auditory veins. [NIH] Medial: Lying near the midsaggital plane of the body; opposed to lateral. [NIH] Mediate: Indirect; accomplished by the aid of an intervening medium. [EU] Mediator: An object or substance by which something is mediated, such as (1) a structure of the nervous system that transmits impulses eliciting a specific response; (2) a chemical substance (transmitter substance) that induces activity in an excitable tissue, such as nerve

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or muscle; or (3) a substance released from cells as the result of the interaction of antigen with antibody or by the action of antigen with a sensitized lymphocyte. [EU] MEDLINE: An online database of MEDLARS, the computerized bibliographic Medical Literature Analysis and Retrieval System of the National Library of Medicine. [NIH] Medullary: Pertaining to the marrow or to any medulla; resembling marrow. [EU] Meiosis: A special method of cell division, occurring in maturation of the germ cells, by means of which each daughter nucleus receives half the number of chromosomes characteristic of the somatic cells of the species. [NIH] Membrane: A very thin layer of tissue that covers a surface. [NIH] Membrane Proteins: Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors. [NIH] Memory: Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. [NIH] Meninges: The three membranes that cover and protect the brain and spinal cord. [NIH] Meningitis: Inflammation of the meninges. When it affects the dura mater, the disease is termed pachymeningitis; when the arachnoid and pia mater are involved, it is called leptomeningitis, or meningitis proper. [EU] Menopause: Permanent cessation of menstruation. [NIH] Mental: Pertaining to the mind; psychic. 2. (L. mentum chin) pertaining to the chin. [EU] Mental Disorders: Psychiatric illness or diseases manifested by breakdowns in the adaptational process expressed primarily as abnormalities of thought, feeling, and behavior producing either distress or impairment of function. [NIH] Mental Health: The state wherein the person is well adjusted. [NIH] Mental Retardation: Refers to sub-average general intellectual functioning which originated during the developmental period and is associated with impairment in adaptive behavior. [NIH]

Mercury: A silver metallic element that exists as a liquid at room temperature. It has the atomic symbol Hg (from hydrargyrum, liquid silver), atomic number 80, and atomic weight 200.59. Mercury is used in many industrial applications and its salts have been employed therapeutically as purgatives, antisyphilitics, disinfectants, and astringents. It can be absorbed through the skin and mucous membranes which leads to mercury poisoning. Because of its toxicity, the clinical use of mercury and mercurials is diminishing. [NIH] Meta-Analysis: A quantitative method of combining the results of independent studies (usually drawn from the published literature) and synthesizing summaries and conclusions which may be used to evaluate therapeutic effectiveness, plan new studies, etc., with application chiefly in the areas of research and medicine. [NIH] Metabotropic: A glutamate receptor which triggers an increase in production of 2 intracellular messengers: diacylglycerol and inositol 1, 4, 5-triphosphate. [NIH] Methamphetamine: A central nervous system stimulant and sympathomimetic with actions and uses similar to dextroamphetamine. The smokable form is a drug of abuse and is referred to as crank, crystal, crystal meth, ice, and speed. [NIH] Methysergide: An ergot derivative that is a congener of lysergic acid diethylamide. It antagonizes the effects of serotonin in blood vessels and gastrointestinal smooth muscle, but

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has few of the properties of other ergot alkaloids. Methysergide is used prophylactically in migraine and other vascular headaches and to antagonize serotonin in the carcinoid syndrome. [NIH] Microbe: An organism which cannot be observed with the naked eye; e. g. unicellular animals, lower algae, lower fungi, bacteria. [NIH] Microcalcifications: Tiny deposits of calcium in the breast that cannot be felt but can be detected on a mammogram. A cluster of these very small specks of calcium may indicate that cancer is present. [NIH] Microglia: The third type of glial cell, along with astrocytes and oligodendrocytes (which together form the macroglia). Microglia vary in appearance depending on developmental stage, functional state, and anatomical location; subtype terms include ramified, perivascular, ameboid, resting, and activated. Microglia clearly are capable of phagocytosis and play an important role in a wide spectrum of neuropathologies. They have also been suggested to act in several other roles including in secretion (e.g., of cytokines and neural growth factors), in immunological processing (e.g., antigen presentation), and in central nervous system development and remodeling. [NIH] Microorganism: An organism that can be seen only through a microscope. Microorganisms include bacteria, protozoa, algae, and fungi. Although viruses are not considered living organisms, they are sometimes classified as microorganisms. [NIH] Mineralization: The action of mineralizing; the state of being mineralized. [EU] Mineralocorticoid: 1. Any of the group of C21 corticosteroids, principally aldosterone, predominantly involved in the regulation of electrolyte and water balance through their effect on ion transport in epithelial cells of the renal tubules, resulting in retention of sodium and loss of potassium; some also possess varying degrees of glucocorticoid activity. Their secretion is regulated principally by plasma volume, serum potassium concentration and angiotensin II, and to a lesser extent by anterior pituitary ACTH. 2. Of, pertaining to, having the properties of, or resembling a mineralocorticoid. [EU] Mitochondria: Parts of a cell where aerobic production (also known as cell respiration) takes place. [NIH] Mitosis: A method of indirect cell division by means of which the two daughter nuclei normally receive identical complements of the number of chromosomes of the somatic cells of the species. [NIH] Modification: A change in an organism, or in a process in an organism, that is acquired from its own activity or environment. [NIH] Molecular: Of, pertaining to, or composed of molecules : a very small mass of matter. [EU] Molecule: A chemical made up of two or more atoms. The atoms in a molecule can be the same (an oxygen molecule has two oxygen atoms) or different (a water molecule has two hydrogen atoms and one oxygen atom). Biological molecules, such as proteins and DNA, can be made up of many thousands of atoms. [NIH] Monitor: An apparatus which automatically records such physiological signs as respiration, pulse, and blood pressure in an anesthetized patient or one undergoing surgical or other procedures. [NIH] Monoamine: Enzyme that breaks down dopamine in the astrocytes and microglia. [NIH] Monoclonal: An antibody produced by culturing a single type of cell. It therefore consists of a single species of immunoglobulin molecules. [NIH] Monoclonal antibodies: Laboratory-produced substances that can locate and bind to cancer cells wherever they are in the body. Many monoclonal antibodies are used in cancer

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detection or therapy; each one recognizes a different protein on certain cancer cells. Monoclonal antibodies can be used alone, or they can be used to deliver drugs, toxins, or radioactive material directly to a tumor. [NIH] Morphine: The principal alkaloid in opium and the prototype opiate analgesic and narcotic. Morphine has widespread effects in the central nervous system and on smooth muscle. [NIH] Motility: The ability to move spontaneously. [EU] Motor Activity: The physical activity of an organism as a behavioral phenomenon. [NIH] Motor Cortex: Area of the frontal lobe concerned with primary motor control. It lies anterior to the central sulcus. [NIH] Motor nerve: An efferent nerve conveying an impulse that excites muscular contraction. [NIH]

Movement Disorders: Syndromes which feature dyskinesias as a cardinal manifestation of the disease process. Included in this category are degenerative, hereditary, post-infectious, medication-induced, post-inflammatory, and post-traumatic conditions. [NIH] Mucins: A secretion containing mucopolysaccharides and protein that is the chief constituent of mucus. [NIH] Mucosa: A mucous membrane, or tunica mucosa. [EU] Mucositis: A complication of some cancer therapies in which the lining of the digestive system becomes inflamed. Often seen as sores in the mouth. [NIH] Multiple sclerosis: A disorder of the central nervous system marked by weakness, numbness, a loss of muscle coordination, and problems with vision, speech, and bladder control. Multiple sclerosis is thought to be an autoimmune disease in which the body's immune system destroys myelin. Myelin is a substance that contains both protein and fat (lipid) and serves as a nerve insulator and helps in the transmission of nerve signals. [NIH] Muscarinic Agonists: Drugs that bind to and activate muscarinic cholinergic receptors (receptors, muscarinic). Muscarinic agonists are most commonly used when it is desirable to increase smooth muscle tone, especially in the GI tract, urinary bladder and the eye. They may also be used to reduce heart rate. [NIH] Muscle Contraction: A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments. [NIH] Muscle Fibers: Large single cells, either cylindrical or prismatic in shape, that form the basic unit of muscle tissue. They consist of a soft contractile substance enclosed in a tubular sheath. [NIH] Muscle Proteins: The protein constituents of muscle, the major ones being ACTINS and MYOSIN. More than a dozen accessary proteins exist including troponin, tropomyosin, and dystrophin. [NIH] Muscular Diseases: Acquired, familial, and congenital disorders of skeletal muscle and smooth muscle. [NIH] Musculature: The muscular apparatus of the body, or of any part of it. [EU] Mustard Gas: Severe irritant and vesicant of skin, eyes, and lungs. It may cause blindness and lethal lung edema and was formerly used as a war gas. The substance has been proposed as a cytostatic and for treatment of psoriasis. It has been listed as a known carcinogen in the Fourth Annual Report on Carcinogens (NTP-85-002, 1985) (Merck, 11th ed). [NIH] Mutagen: Any agent, such as X-rays, gamma rays, mustard gas, TCDD, that can cause

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abnormal mutation in living cells; having the power to cause mutations. [NIH] Mutagenesis: Process of generating genetic mutations. It may occur spontaneously or be induced by mutagens. [NIH] Myasthenia: Muscular debility; any constitutional anomaly of muscle. [EU] Myelin: The fatty substance that covers and protects nerves. [NIH] Myocardial infarction: Gross necrosis of the myocardium as a result of interruption of the blood supply to the area; it is almost always caused by atherosclerosis of the coronary arteries, upon which coronary thrombosis is usually superimposed. [NIH] Myocardium: The muscle tissue of the heart composed of striated, involuntary muscle known as cardiac muscle. [NIH] Myoclonus: Involuntary shock-like contractions, irregular in rhythm and amplitude, followed by relaxation, of a muscle or a group of muscles. This condition may be a feature of some central nervous systems diseases (e.g., epilepsy, myoclonic). Nocturnal myoclonus may represent a normal physiologic event or occur as the principal feature of the nocturnal myoclonus syndrome. (From Adams et al., Principles of Neurology, 6th ed, pp102-3). [NIH] Myosin: Chief protein in muscle and the main constituent of the thick filaments of muscle fibers. In conjunction with actin, it is responsible for the contraction and relaxation of muscles. [NIH] Myotonic Dystrophy: A condition presenting muscle weakness and wasting which may be progressive. [NIH] Necrosis: A pathological process caused by the progressive degradative action of enzymes that is generally associated with severe cellular trauma. It is characterized by mitochondrial swelling, nuclear flocculation, uncontrolled cell lysis, and ultimately cell death. [NIH] Neoplasms: New abnormal growth of tissue. Malignant neoplasms show a greater degree of anaplasia and have the properties of invasion and metastasis, compared to benign neoplasms. [NIH] Neostriatum: The phylogenetically newer part of the corpus striatum consisting of the caudate nucleus and putamen. It is often called simply the striatum. [NIH] Nephritis: Inflammation of the kidney; a focal or diffuse proliferative or destructive process which may involve the glomerulus, tubule, or interstitial renal tissue. [EU] Nephron: A tiny part of the kidneys. Each kidney is made up of about 1 million nephrons, which are the working units of the kidneys, removing wastes and extra fluids from the blood. [NIH] Nervous System: The entire nerve apparatus composed of the brain, spinal cord, nerves and ganglia. [NIH] Neural: 1. Pertaining to a nerve or to the nerves. 2. Situated in the region of the spinal axis, as the neutral arch. [EU] Neural tube defects: These defects include problems stemming from fetal development of the spinal cord, spine, brain, and skull, and include birth defects such as spina bifida, anencephaly, and encephalocele. Neural tube defects occur early in pregnancy at about 4 to 6 weeks, usually before a woman knows she is pregnant. Many babies with neural tube defects have difficulty walking and with bladder and bowel control. [NIH] Neurites: In tissue culture, hairlike projections of neurons stimulated by growth factors and other molecules. These projections may go on to form a branched tree of dendrites or a single axon or they may be reabsorbed at a later stage of development. "Neurite" may refer to any filamentous or pointed outgrowth of an embryonal or tissue-culture neural cell. [NIH]

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Neurodegenerative Diseases: Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures. [NIH] Neuroglia: The non-neuronal cells of the nervous system. They are divided into macroglia (astrocytes, oligodendroglia, and schwann cells) and microglia. They not only provide physical support, but also respond to injury, regulate the ionic and chemical composition of the extracellular milieu, participate in the blood-brain and blood-retina barriers, form the myelin insulation of nervous pathways, guide neuronal migration during development, and exchange metabolites with neurons. Neuroglia have high-affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitters, but their role in signaling (as in many other functions) is unclear. [NIH] Neurologic: Having to do with nerves or the nervous system. [NIH] Neurologist: A doctor who specializes in the diagnosis and treatment of disorders of the nervous system. [NIH] Neuromuscular: Pertaining to muscles and nerves. [EU] Neuromuscular Diseases: A general term encompassing lower motor neuron disease; peripheral nervous system diseases; and certain muscular diseases. Manifestations include muscle weakness; fasciculation; muscle atrophy; spasm; myokymia; muscle hypertonia, myalgias, and musclehypotonia. [NIH] Neuromuscular Junction: The synapse between a neuron and a muscle. [NIH] Neuromuscular Junction Diseases: Conditions characterized by impaired transmission of impulses at the neuromuscular junction. This may result from disorders that affect receptor function, pre- or postsynaptic membrane function, or acetylcholinesteraseactivity. The majority of diseases in this category are associated with autoimmune, toxic, or inherited conditions. [NIH] Neuronal: Pertaining to a neuron or neurons (= conducting cells of the nervous system). [EU] Neurons: The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the nervous system. [NIH] Neuropathy: A problem in any part of the nervous system except the brain and spinal cord. Neuropathies can be caused by infection, toxic substances, or disease. [NIH] Neuropeptides: Peptides released by neurons as intercellular messengers. Many neuropeptides are also hormones released by non-neuronal cells. [NIH] Neurophysiology: The scientific discipline concerned with the physiology of the nervous system. [NIH] Neurosis: Functional derangement due to disorders of the nervous system which does not affect the psychic personality of the patient. [NIH] Neurosyphilis: A late form of syphilis that affects the brain and may lead to dementia and death. [NIH] Neurotoxic: Poisonous or destructive to nerve tissue. [EU] Neurotoxicity: The tendency of some treatments to cause damage to the nervous system. [NIH]

Neurotoxin: A substance that is poisonous to nerve tissue. [NIH] Neurotransmitter: Any of a group of substances that are released on excitation from the axon terminal of a presynaptic neuron of the central or peripheral nervous system and travel across the synaptic cleft to either excite or inhibit the target cell. Among the many

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substances that have the properties of a neurotransmitter are acetylcholine, norepinephrine, epinephrine, dopamine, glycine, y-aminobutyrate, glutamic acid, substance P, enkephalins, endorphins, and serotonin. [EU] Nickel: A trace element with the atomic symbol Ni, atomic number 28, and atomic weight 58.69. It is a cofactor of the enzyme urease. [NIH] Nitric Oxide: A free radical gas produced endogenously by a variety of mammalian cells. It is synthesized from arginine by a complex reaction, catalyzed by nitric oxide synthase. Nitric oxide is endothelium-derived relaxing factor. It is released by the vascular endothelium and mediates the relaxation induced by some vasodilators such as acetylcholine and bradykinin. It also inhibits platelet aggregation, induces disaggregation of aggregated platelets, and inhibits platelet adhesion to the vascular endothelium. Nitric oxide activates cytosolic guanylate cyclase and thus elevates intracellular levels of cyclic GMP. [NIH]

Norepinephrine: Precursor of epinephrine that is secreted by the adrenal medulla and is a widespread central and autonomic neurotransmitter. Norepinephrine is the principal transmitter of most postganglionic sympathetic fibers and of the diffuse projection system in the brain arising from the locus ceruleus. It is also found in plants and is used pharmacologically as a sympathomimetic. [NIH] Nuclear: A test of the structure, blood flow, and function of the kidneys. The doctor injects a mildly radioactive solution into an arm vein and uses x-rays to monitor its progress through the kidneys. [NIH] Nuclei: A body of specialized protoplasm found in nearly all cells and containing the chromosomes. [NIH] Nucleic acid: Either of two types of macromolecule (DNA or RNA) formed by polymerization of nucleotides. Nucleic acids are found in all living cells and contain the information (genetic code) for the transfer of genetic information from one generation to the next. [NIH] Nucleus: A body of specialized protoplasm found in nearly all cells and containing the chromosomes. [NIH] Nystagmus: An involuntary, rapid, rhythmic movement of the eyeball, which may be horizontal, vertical, rotatory, or mixed, i.e., of two varieties. [EU] Observational study: An epidemiologic study that does not involve any intervention, experimental or otherwise. Such a study may be one in which nature is allowed to take its course, with changes in one characteristic being studied in relation to changes in other characteristics. Analytical epidemiologic methods, such as case-control and cohort study designs, are properly called observational epidemiology because the investigator is observing without intervention other than to record, classify, count, and statistically analyze results. [NIH] Obsessive-Compulsive Disorder: An anxiety disorder characterized by recurrent, persistent obsessions or compulsions. Obsessions are the intrusive ideas, thoughts, or images that are experienced as senseless or repugnant. Compulsions are repetitive and seemingly purposeful behavior which the individual generally recognizes as senseless and from which the individual does not derive pleasure although it may provide a release from tension. [NIH] Oculi: Globe or ball of the eye. [NIH] Oculomotor: Cranial nerve III. It originate from the lower ventral surface of the midbrain and is classified as a motor nerve. [NIH] Olfactory Bulb: Ovoid body resting on the cribriform plate of the ethmoid bone where the olfactory nerve terminates. The olfactory bulb contains several types of nerve cells including

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the mitral cells, on whose dendrites the olfactory nerve synapses, forming the olfactory glomeruli. The accessory olfactory bulb, which receives the projection from the vomeronasal organ via the vomeronasal nerve, is also included here. [NIH] Olfactory Nerve: The 1st cranial nerve. The olfactory nerve conveys the sense of smell. It is formed by the axons of olfactory receptor neurons which project from the olfactory epithelium (in the nasal epithelium) to the olfactory bulb. [NIH] Oligonucleotide Probes: Synthetic or natural oligonucleotides used in hybridization studies in order to identify and study specific nucleic acid fragments, e.g., DNA segments near or within a specific gene locus or gene. The probe hybridizes with a specific mRNA, if present. Conventional techniques used for testing for the hybridization product include dot blot assays, Southern blot assays, and DNA:RNA hybrid-specific antibody tests. Conventional labels for the probe include the radioisotope labels 32P and 125I and the chemical label biotin. [NIH] Oocytes: Female germ cells in stages between the prophase of the first maturation division and the completion of the second maturation division. [NIH] Opacity: Degree of density (area most dense taken for reading). [NIH] Opiate: A remedy containing or derived from opium; also any drug that induces sleep. [EU] Opportunistic Infections: An infection caused by an organism which becomes pathogenic under certain conditions, e.g., during immunosuppression. [NIH] Optic Chiasm: The X-shaped structure formed by the meeting of the two optic nerves. At the optic chiasm the fibers from the medial part of each retina cross to project to the other side of the brain while the lateral retinal fibers continue on the same side. As a result each half of the brain receives information about the contralateral visual field from both eyes. [NIH]

Oral Health: The optimal state of the mouth and normal functioning of the organs of the mouth without evidence of disease. [NIH] Oral Hygiene: The practice of personal hygiene of the mouth. It includes the maintenance of oral cleanliness, tissue tone, and general preservation of oral health. [NIH] Oral surgeon: A dentist with special training in surgery of the mouth and jaw. [NIH] Orbicularis: A thin layer of fibers that originates at the posterior lacrimal crest and passes outward and forward, dividing into two slips which surround the canaliculi. [NIH] Organ Culture: The growth in aseptic culture of plant organs such as roots or shoots, beginning with organ primordia or segments and maintaining the characteristics of the organ. [NIH] Organelles: Specific particles of membrane-bound organized living substances present in eukaryotic cells, such as the mitochondria; the golgi apparatus; endoplasmic reticulum; lysomomes; plastids; and vacuoles. [NIH] Orofacial: Of or relating to the mouth and face. [EU] Ovum: A female germ cell extruded from the ovary at ovulation. [NIH] Oxidative Phosphorylation: Electron transfer through the cytochrome system liberating free energy which is transformed into high-energy phosphate bonds. [NIH] Oxygenator: An apparatus by which oxygen is introduced into the blood during circulation outside the body, as during open heart surgery. [NIH] Pachymeningitis: Inflammation of the dura mater of the brain, the spinal cord or the optic nerve. [NIH] Palliative: 1. Affording relief, but not cure. 2. An alleviating medicine. [EU]

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Palsy: Disease of the peripheral nervous system occurring usually after many years of increased lead absorption. [NIH] Pancreas: A mixed exocrine and endocrine gland situated transversely across the posterior abdominal wall in the epigastric and hypochondriac regions. The endocrine portion is comprised of the Islets of Langerhans, while the exocrine portion is a compound acinar gland that secretes digestive enzymes. [NIH] Paralysis: Loss of ability to move all or part of the body. [NIH] Paresis: A general term referring to a mild to moderate degree of muscular weakness, occasionally used as a synonym for paralysis (severe or complete loss of motor function). In the older literature, paresis often referred specifically to paretic neurosyphilis. "General paresis" and "general paralysis" may still carry that connotation. Bilateral lower extremity paresis is referred to as paraparesis. [NIH] Parietal: 1. Of or pertaining to the walls of a cavity. 2. Pertaining to or located near the parietal bone, as the parietal lobe. [EU] Parietal Lobe: Upper central part of the cerebral hemisphere. [NIH] Parkinsonism: A group of neurological disorders characterized by hypokinesia, tremor, and muscular rigidity. [EU] Paroxysmal: Recurring in paroxysms (= spasms or seizures). [EU] Partial remission: The shrinking, but not complete disappearance, of a tumor in response to therapy. Also called partial response. [NIH] Particle: A tiny mass of material. [EU] Parturition: The act or process of given birth to a child. [EU] Patch: A piece of material used to cover or protect a wound, an injured part, etc.: a patch over the eye. [NIH] Pathologic: 1. Indicative of or caused by a morbid condition. 2. Pertaining to pathology (= branch of medicine that treats the essential nature of the disease, especially the structural and functional changes in tissues and organs of the body caused by the disease). [EU] Pathologic Processes: The abnormal mechanisms and forms involved in the dysfunctions of tissues and organs. [NIH] Pathophysiology: Altered functions in an individual or an organ due to disease. [NIH] Patient Education: The teaching or training of patients concerning their own health needs. [NIH]

Penicillin: An antibiotic drug used to treat infection. [NIH] Peptide: Any compound consisting of two or more amino acids, the building blocks of proteins. Peptides are combined to make proteins. [NIH] Pergolide: A long-acting dopamine agonist which is effective in the treatment of Parkinson's disease and hyperprolactinemia. It has also been observed to have antihypertensive effects. [NIH]

Pericarditis: Inflammation of the pericardium. [EU] Pericardium: The fibroserous sac surrounding the heart and the roots of the great vessels. [NIH]

Peripheral blood: Blood circulating throughout the body. [NIH] Peripheral Nerves: The nerves outside of the brain and spinal cord, including the autonomic, cranial, and spinal nerves. Peripheral nerves contain non-neuronal cells and connective tissue as well as axons. The connective tissue layers include, from the outside to

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the inside, the epineurium, the perineurium, and the endoneurium. [NIH] Peripheral Nervous System: The nervous system outside of the brain and spinal cord. The peripheral nervous system has autonomic and somatic divisions. The autonomic nervous system includes the enteric, parasympathetic, and sympathetic subdivisions. The somatic nervous system includes the cranial and spinal nerves and their ganglia and the peripheral sensory receptors. [NIH] Peripheral Nervous System Diseases: Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves. [NIH] Petechiae: Pinpoint, unraised, round red spots under the skin caused by bleeding. [NIH] Petroleum: Naturally occurring complex liquid hydrocarbons which, after distillation, yield combustible fuels, petrochemicals, and lubricants. [NIH] Pharmacodynamic: Is concerned with the response of living tissues to chemical stimuli, that is, the action of drugs on the living organism in the absence of disease. [NIH] Pharmacokinetic: The mathematical analysis of the time courses of absorption, distribution, and elimination of drugs. [NIH] Pharmacologic: Pertaining to pharmacology or to the properties and reactions of drugs. [EU] Pharyngitis: Inflammation of the throat. [NIH] Pharynx: The hollow tube about 5 inches long that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes to the stomach). [NIH] Phenotypes: An organism as observed, i. e. as judged by its visually perceptible characters resulting from the interaction of its genotype with the environment. [NIH] Phenytoin: An anticonvulsant that is used in a wide variety of seizures. It is also an antiarrhythmic and a muscle relaxant. The mechanism of therapeutic action is not clear, although several cellular actions have been described including effects on ion channels, active transport, and general membrane stabilization. The mechanism of its muscle relaxant effect appears to involve a reduction in the sensitivity of muscle spindles to stretch. Phenytoin has been proposed for several other therapeutic uses, but its use has been limited by its many adverse effects and interactions with other drugs. [NIH] Phonation: The process of producing vocal sounds by means of vocal cords vibrating in an expiratory blast of air. [NIH] Phospholipases: A class of enzymes that catalyze the hydrolysis of phosphoglycerides or glycerophosphatidates. EC 3.1.-. [NIH] Phospholipids: Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides; glycerophospholipids) or sphingosine (sphingolipids). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system. [NIH] Phosphorus: A non-metallic element that is found in the blood, muscles, nevers, bones, and teeth, and is a component of adenosine triphosphate (ATP; the primary energy source for the body's cells.) [NIH] Phosphorylation: The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety. [NIH] Phrenic Nerve: The motor nerve of the diaphragm. The phrenic nerve fibers originate in the cervical spinal column (mostly C4) and travel through the cervical plexus to the diaphragm. [NIH]

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Physiologic: Having to do with the functions of the body. When used in the phrase "physiologic age," it refers to an age assigned by general health, as opposed to calendar age. [NIH]

Physiology: The science that deals with the life processes and functions of organismus, their cells, tissues, and organs. [NIH] Piloerection: Involuntary erection or bristling of hairs. [NIH] Piracetam: A compound suggested to be both a nootropic and a neuroprotective agent. [NIH] Pitch: The subjective awareness of the frequency or spectral distribution of a sound. [NIH] Plants: Multicellular, eukaryotic life forms of the kingdom Plantae. They are characterized by a mainly photosynthetic mode of nutrition; essentially unlimited growth at localized regions of cell divisions (meristems); cellulose within cells providing rigidity; the absence of organs of locomotion; absense of nervous and sensory systems; and an alteration of haploid and diploid generations. [NIH] Plasma: The clear, yellowish, fluid part of the blood that carries the blood cells. The proteins that form blood clots are in plasma. [NIH] Plasma cells: A type of white blood cell that produces antibodies. [NIH] Plasma Exchange: Removal of plasma and replacement with various fluids, e.g., fresh frozen plasma, plasma protein fractions (PPF), albumin preparations, dextran solutions, saline. Used in treatment of autoimmune diseases, immune complex diseases, diseases of excess plasma factors, and other conditions. [NIH] Plasma protein: One of the hundreds of different proteins present in blood plasma, including carrier proteins ( such albumin, transferrin, and haptoglobin), fibrinogen and other coagulation factors, complement components, immunoglobulins, enzyme inhibitors, precursors of substances such as angiotension and bradykinin, and many other types of proteins. [EU] Plasmapheresis: Procedure whereby plasma is separated and extracted from anticoagulated whole blood and the red cells retransfused to the donor. Plasmapheresis is also employed for therapeutic use. [NIH] Plasmin: A product of the lysis of plasminogen (profibrinolysin) by plasminogen activators. It is composed of two polypeptide chains, light (B) and heavy (A), with a molecular weight of 75,000. It is the major proteolytic enzyme involved in blood clot retraction or the lysis of fibrin and quickly inactivated by antiplasmins. EC 3.4.21.7. [NIH] Plasminogen Activators: A heterogeneous group of proteolytic enzymes that convert plasminogen to plasmin. They are concentrated in the lysosomes of most cells and in the vascular endothelium, particularly in the vessels of the microcirculation. EC 3.4.21.-. [NIH] Platelet Activation: A series of progressive, overlapping events triggered by exposure of the platelets to subendothelial tissue. These events include shape change, adhesiveness, aggregation, and release reactions. When carried through to completion, these events lead to the formation of a stable hemostatic plug. [NIH] Platelet Aggregation: The attachment of platelets to one another. This clumping together can be induced by a number of agents (e.g., thrombin, collagen) and is part of the mechanism leading to the formation of a thrombus. [NIH] Platelets: A type of blood cell that helps prevent bleeding by causing blood clots to form. Also called thrombocytes. [NIH] Pneumonia: Inflammation of the lungs. [NIH] Point Mutation: A mutation caused by the substitution of one nucleotide for another. This

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results in the DNA molecule having a change in a single base pair. [NIH] Poisoning: A condition or physical state produced by the ingestion, injection or inhalation of, or exposure to a deleterious agent. [NIH] Polyarthritis: An inflammation of several joints together. [EU] Polymerase: An enzyme which catalyses the synthesis of DNA using a single DNA strand as a template. The polymerase copies the template in the 5'-3'direction provided that sufficient quantities of free nucleotides, dATP and dTTP are present. [NIH] Polymerase Chain Reaction: In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships. [NIH] Polymorphic: Occurring in several or many forms; appearing in different forms at different stages of development. [EU] Polyneuritis: Inflammation of several peripheral nerves at the same time. [NIH] Polypeptide: A peptide which on hydrolysis yields more than two amino acids; called tripeptides, tetrapeptides, etc. according to the number of amino acids contained. [EU] Polysaccharide: A type of carbohydrate. It contains sugar molecules that are linked together chemically. [NIH] Pons: The part of the central nervous system lying between the medulla oblongata and the mesencephalon, ventral to the cerebellum, and consisting of a pars dorsalis and a pars ventralis. [NIH] Posterior: Situated in back of, or in the back part of, or affecting the back or dorsal surface of the body. In lower animals, it refers to the caudal end of the body. [EU] Postherpetic Neuralgia: Variety of neuralgia associated with migraine in which pain is felt in or behind the eye. [NIH] Postsynaptic: Nerve potential generated by an inhibitory hyperpolarizing stimulation. [NIH] Post-traumatic: Occurring as a result of or after injury. [EU] Potassium: An element that is in the alkali group of metals. It has an atomic symbol K, atomic number 19, and atomic weight 39.10. It is the chief cation in the intracellular fluid of muscle and other cells. Potassium ion is a strong electrolyte and it plays a significant role in the regulation of fluid volume and maintenance of the water-electrolyte balance. [NIH] Potentiates: A degree of synergism which causes the exposure of the organism to a harmful substance to worsen a disease already contracted. [NIH] Potentiation: An overall effect of two drugs taken together which is greater than the sum of the effects of each drug taken alone. [NIH] Practice Guidelines: Directions or principles presenting current or future rules of policy for the health care practitioner to assist him in patient care decisions regarding diagnosis, therapy, or related clinical circumstances. The guidelines may be developed by government agencies at any level, institutions, professional societies, governing boards, or by the convening of expert panels. The guidelines form a basis for the evaluation of all aspects of health care and delivery. [NIH] Precursor: Something that precedes. In biological processes, a substance from which

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another, usually more active or mature substance is formed. In clinical medicine, a sign or symptom that heralds another. [EU] Prednisolone: A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states. [NIH] Prednisone: A synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver. [NIH] Prefrontal Cortex: The rostral part of the frontal lobe, bounded by the inferior precentral fissure in humans, which receives projection fibers from the mediodorsal nucleus of the thalamus. The prefrontal cortex receives afferent fibers from numerous structures of the diencephalon, mesencephalon, and limbic system as well as cortical afferents of visual, auditory, and somatic origin. [NIH] Prenatal: Existing or occurring before birth, with reference to the fetus. [EU] Presynaptic: Situated proximal to a synapse, or occurring before the synapse is crossed. [EU] Probe: An instrument used in exploring cavities, or in the detection and dilatation of strictures, or in demonstrating the potency of channels; an elongated instrument for exploring or sounding body cavities. [NIH] Progesterone: Pregn-4-ene-3,20-dione. The principal progestational hormone of the body, secreted by the corpus luteum, adrenal cortex, and placenta. Its chief function is to prepare the uterus for the reception and development of the fertilized ovum. It acts as an antiovulatory agent when administered on days 5-25 of the menstrual cycle. [NIH] Progression: Increase in the size of a tumor or spread of cancer in the body. [NIH] Progressive: Advancing; going forward; going from bad to worse; increasing in scope or severity. [EU] Projection: A defense mechanism, operating unconsciously, whereby that which is emotionally unacceptable in the self is rejected and attributed (projected) to others. [NIH] Prolactin: Pituitary lactogenic hormone. A polypeptide hormone with a molecular weight of about 23,000. It is essential in the induction of lactation in mammals at parturition and is synergistic with estrogen. The hormone also brings about the release of progesterone from lutein cells, which renders the uterine mucosa suited for the embedding of the ovum should fertilization occur. [NIH] Prophase: The first phase of cell division, in which the chromosomes become visible, the nucleus starts to lose its identity, the spindle appears, and the centrioles migrate toward opposite poles. [NIH] Prophylaxis: An attempt to prevent disease. [NIH] Propranolol: A widely used non-cardioselective beta-adrenergic antagonist. Propranolol is used in the treatment or prevention of many disorders including acute myocardial infarction, arrhythmias, angina pectoris, hypertension, hypertensive emergencies, hyperthyroidism, migraine, pheochromocytoma, menopause, and anxiety. [NIH] Prosencephalon: The part of the brain developed from the most rostral of the three primary vesicles of the embryonic neural tube and consisting of the diencephalon and telencephalon. [NIH]

Prospective study: An epidemiologic study in which a group of individuals (a cohort), all free of a particular disease and varying in their exposure to a possible risk factor, is followed over a specific amount of time to determine the incidence rates of the disease in the exposed and unexposed groups. [NIH]

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Prostate: A gland in males that surrounds the neck of the bladder and the urethra. It secretes a substance that liquifies coagulated semen. It is situated in the pelvic cavity behind the lower part of the pubic symphysis, above the deep layer of the triangular ligament, and rests upon the rectum. [NIH] Prosthesis: An artificial replacement of a part of the body. [NIH] Protein C: A vitamin-K dependent zymogen present in the blood, which, upon activation by thrombin and thrombomodulin exerts anticoagulant properties by inactivating factors Va and VIIIa at the rate-limiting steps of thrombin formation. [NIH] Protein S: The vitamin K-dependent cofactor of activated protein C. Together with protein C, it inhibits the action of factors VIIIa and Va. A deficiency in protein S can lead to recurrent venous and arterial thrombosis. [NIH] Proteins: Polymers of amino acids linked by peptide bonds. The specific sequence of amino acids determines the shape and function of the protein. [NIH] Proteolytic: 1. Pertaining to, characterized by, or promoting proteolysis. 2. An enzyme that promotes proteolysis (= the splitting of proteins by hydrolysis of the peptide bonds with formation of smaller polypeptides). [EU] Protons: Stable elementary particles having the smallest known positive charge, found in the nuclei of all elements. The proton mass is less than that of a neutron. A proton is the nucleus of the light hydrogen atom, i.e., the hydrogen ion. [NIH] Proximal: Nearest; closer to any point of reference; opposed to distal. [EU] Psychiatric: Pertaining to or within the purview of psychiatry. [EU] Psychiatry: The medical science that deals with the origin, diagnosis, prevention, and treatment of mental disorders. [NIH] Psychic: Pertaining to the psyche or to the mind; mental. [EU] Psychoactive: Those drugs which alter sensation, mood, consciousness or other psychological or behavioral functions. [NIH] Psychosis: A mental disorder characterized by gross impairment in reality testing as evidenced by delusions, hallucinations, markedly incoherent speech, or disorganized and agitated behaviour without apparent awareness on the part of the patient of the incomprehensibility of his behaviour; the term is also used in a more general sense to refer to mental disorders in which mental functioning is sufficiently impaired as to interfere grossly with the patient's capacity to meet the ordinary demands of life. Historically, the term has been applied to many conditions, e.g. manic-depressive psychosis, that were first described in psychotic patients, although many patients with the disorder are not judged psychotic. [EU] Psychotomimetic: Psychosis miming. [NIH] Public Health: Branch of medicine concerned with the prevention and control of disease and disability, and the promotion of physical and mental health of the population on the international, national, state, or municipal level. [NIH] Public Policy: A course or method of action selected, usually by a government, from among alternatives to guide and determine present and future decisions. [NIH] Pulmonary: Relating to the lungs. [NIH] Pulse: The rhythmical expansion and contraction of an artery produced by waves of pressure caused by the ejection of blood from the left ventricle of the heart as it contracts. [NIH]

Purines: A series of heterocyclic compounds that are variously substituted in nature and are

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known also as purine bases. They include adenine and guanine, constituents of nucleic acids, as well as many alkaloids such as caffeine and theophylline. Uric acid is the metabolic end product of purine metabolism. [NIH] Purpura: Purplish or brownish red discoloration, easily visible through the epidermis, caused by hemorrhage into the tissues. [NIH] Putamen: The largest and most lateral of the basal ganglia lying between the lateral medullary lamina of the globus pallidus and the external capsule. It is part of the neostriatum and forms part of the lentiform nucleus along with the globus pallidus. [NIH] Pyramidal Tracts: Fibers that arise from cells within the cerebral cortex, pass through the medullary pyramid, and descend in the spinal cord. Many authorities say the pyramidal tracts include both the corticospinal and corticobulbar tracts. [NIH] Pyrimidines: A family of 6-membered heterocyclic compounds occurring in nature in a wide variety of forms. They include several nucleic acid constituents (cytosine, thymine, and uracil) and form the basic structure of the barbiturates. [NIH] Pyrogenic: Inducing fever. [EU] Quadriplegia: Severe or complete loss of motor function in all four limbs which may result from brain diseases; spinal cord diseases; peripheral nervous system diseases; neuromuscular diseases; or rarely muscular diseases. The locked-in syndrome is characterized by quadriplegia in combination with cranial muscle paralysis. Consciousness is spared and the only retained voluntary motor activity may be limited eye movements. This condition is usually caused by a lesion in the upper brain stem which injures the descending cortico-spinal and cortico-bulbar tracts. [NIH] Quality of Life: A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral and social environment. [NIH] Race: A population within a species which exhibits general similarities within itself, but is both discontinuous and distinct from other populations of that species, though not sufficiently so as to achieve the status of a taxon. [NIH] Racemic: Optically inactive but resolvable in the way of all racemic compounds. [NIH] Radiation: Emission or propagation of electromagnetic energy (waves/rays), or the waves/rays themselves; a stream of electromagnetic particles (electrons, neutrons, protons, alpha particles) or a mixture of these. The most common source is the sun. [NIH] Radiation therapy: The use of high-energy radiation from x-rays, gamma rays, neutrons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body in the area near cancer cells (internal radiation therapy, implant radiation, or brachytherapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that circulates throughout the body. Also called radiotherapy. [NIH] Radioactive: Giving off radiation. [NIH] Radioisotope: An unstable element that releases radiation as it breaks down. Radioisotopes can be used in imaging tests or as a treatment for cancer. [NIH] Radiolabeled: Any compound that has been joined with a radioactive substance. [NIH] Randomized: Describes an experiment or clinical trial in which animal or human subjects are assigned by chance to separate groups that compare different treatments. [NIH] Ranitidine: A non-imidazole blocker of those histamine receptors that mediate gastric secretion (H2 receptors). It is used to treat gastrointestinal ulcers. [NIH]

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Reality Testing: The individual's objective evaluation of the external world and the ability to differentiate adequately between it and the internal world; considered to be a primary ego function. [NIH] Receptor: A molecule inside or on the surface of a cell that binds to a specific substance and causes a specific physiologic effect in the cell. [NIH] Receptors, Serotonin: Cell-surface proteins that bind serotonin and trigger intracellular changes which influence the behavior of cells. Several types of serotonin receptors have been recognized which differ in their pharmacology, molecular biology, and mode of action. [NIH] Recombinant: A cell or an individual with a new combination of genes not found together in either parent; usually applied to linked genes. [EU] Recombinant Proteins: Proteins prepared by recombinant DNA technology. [NIH] Recombination: The formation of new combinations of genes as a result of segregation in crosses between genetically different parents; also the rearrangement of linked genes due to crossing-over. [NIH] Recur: To occur again. Recurrence is the return of cancer, at the same site as the original (primary) tumor or in another location, after the tumor had disappeared. [NIH] Recurrence: The return of a sign, symptom, or disease after a remission. [NIH] Red blood cells: RBCs. Cells that carry oxygen to all parts of the body. Also called erythrocytes. [NIH] Red Nucleus: A pinkish-yellow portion of the midbrain situated in the rostral mesencephalic tegmentum. It receives a large projection from the contralateral half of the cerebellum via the superior cerebellar peduncle and a projection from the ipsilateral motor cortex. [NIH] Refer: To send or direct for treatment, aid, information, de decision. [NIH] Reflex: An involuntary movement or exercise of function in a part, excited in response to a stimulus applied to the periphery and transmitted to the brain or spinal cord. [NIH] Refraction: A test to determine the best eyeglasses or contact lenses to correct a refractive error (myopia, hyperopia, or astigmatism). [NIH] Regimen: A treatment plan that specifies the dosage, the schedule, and the duration of treatment. [NIH] Remission: A decrease in or disappearance of signs and symptoms of cancer. In partial remission, some, but not all, signs and symptoms of cancer have disappeared. In complete remission, all signs and symptoms of cancer have disappeared, although there still may be cancer in the body. [NIH] Renal failure: Progressive renal insufficiency and uremia, due to irreversible and progressive renal glomerular tubular or interstitial disease. [NIH] Research Design: A plan for collecting and utilizing data so that desired information can be obtained with sufficient precision or so that an hypothesis can be tested properly. [NIH] Resorption: The loss of substance through physiologic or pathologic means, such as loss of dentin and cementum of a tooth, or of the alveolar process of the mandible or maxilla. [EU] Respiration: The act of breathing with the lungs, consisting of inspiration, or the taking into the lungs of the ambient air, and of expiration, or the expelling of the modified air which contains more carbon dioxide than the air taken in (Blakiston's Gould Medical Dictionary, 4th ed.). This does not include tissue respiration (= oxygen consumption) or cell respiration (= cell respiration). [NIH] Respiratory distress syndrome: A lung disease that occurs primarily in premature infants;

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the newborn must struggle for each breath and blueing of its skin reflects the baby's inability to get enough oxygen. [NIH] Respiratory Paralysis: Complete or severe weakness of the muscles of respiration. This condition may be associated with motor neuron diseases; peripheral nerve disorders; neuromuscular junction diseases; spinal cord diseases; injury to the phrenic nerve; and other disorders. [NIH] Retina: The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outer-most layer is pigmented, whereas the inner nine layers are transparent. [NIH] Retinoid: Vitamin A or a vitamin A-like compound. [NIH] Retinopathy: 1. Retinitis (= inflammation of the retina). 2. Retinosis (= degenerative, noninflammatory condition of the retina). [EU] Retrograde: 1. Moving backward or against the usual direction of flow. 2. Degenerating, deteriorating, or catabolic. [EU] Rheumatic Heart Disease: Disease of the heart resulting from rheumatic fever and characterized by inflammatory changes in the myocardium or scarring of the valves. [NIH] Ribose: A pentose active in biological systems usually in its D-form. [NIH] Ribosome: A granule of protein and RNA, synthesized in the nucleolus and found in the cytoplasm of cells. Ribosomes are the main sites of protein synthesis. Messenger RNA attaches to them and there receives molecules of transfer RNA bearing amino acids. [NIH] Rigidity: Stiffness or inflexibility, chiefly that which is abnormal or morbid; rigor. [EU] Risk factor: A habit, trait, condition, or genetic alteration that increases a person's chance of developing a disease. [NIH] Risperidone: A selective blocker of dopamine D2 and serotonin-5-HT-2 receptors that acts as an atypical antipsychotic agent. It has been shown to improve both positive and negative symptoms in the treatment of schizophrenia. [NIH] Rod: A reception for vision, located in the retina. [NIH] Saccule: The smaller of the 2 sacs within the vestibule of the ear. [NIH] Saline: A solution of salt and water. [NIH] Saliva: The clear, viscous fluid secreted by the salivary glands and mucous glands of the mouth. It contains mucins, water, organic salts, and ptylin. [NIH] Salivary: The duct that convey saliva to the mouth. [NIH] Salivary glands: Glands in the mouth that produce saliva. [NIH] Saponins: Sapogenin glycosides. A type of glycoside widely distributed in plants. Each consists of a sapogenin as the aglycon moiety, and a sugar. The sapogenin may be a steroid or a triterpene and the sugar may be glucose, galactose, a pentose, or a methylpentose. Sapogenins are poisonous towards the lower forms of life and are powerful hemolytics when injected into the blood stream able to dissolve red blood cells at even extreme dilutions. [NIH] Sarcoplasmic Reticulum: A network of tubules and sacs in the cytoplasm of skeletal muscles that assist with muscle contraction and relaxation by releasing and storing calcium ions. [NIH] Scans: Pictures of structures inside the body. Scans often used in diagnosing, staging, and monitoring disease include liver scans, bone scans, and computed tomography (CT) or

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computerized axial tomography (CAT) scans and magnetic resonance imaging (MRI) scans. In liver scanning and bone scanning, radioactive substances that are injected into the bloodstream collect in these organs. A scanner that detects the radiation is used to create pictures. In CT scanning, an x-ray machine linked to a computer is used to produce detailed pictures of organs inside the body. MRI scans use a large magnet connected to a computer to create pictures of areas inside the body. [NIH] Schizoid: Having qualities resembling those found in greater degree in schizophrenics; a person of schizoid personality. [NIH] Schizophrenia: A mental disorder characterized by a special type of disintegration of the personality. [NIH] Schizotypal Personality Disorder: A personality disorder in which there are oddities of thought (magical thinking, paranoid ideation, suspiciousness), perception (illusions, depersonalization), speech (digressive, vague, overelaborate), and behavior (inappropriate affect in social interactions, frequently social isolation) that are not severe enough to characterize schizophrenia. [NIH] Sclerosis: A pathological process consisting of hardening or fibrosis of an anatomical structure, often a vessel or a nerve. [NIH] Screening: Checking for disease when there are no symptoms. [NIH] Second Messenger Systems: Systems in which an intracellular signal is generated in response to an intercellular primary messenger such as a hormone or neurotransmitter. They are intermediate signals in cellular processes such as metabolism, secretion, contraction, phototransduction, and cell growth. Examples of second messenger systems are the adenyl cyclase-cyclic AMP system, the phosphatidylinositol diphosphate-inositol triphosphate system, and the cyclic GMP system. [NIH] Secretion: 1. The process of elaborating a specific product as a result of the activity of a gland; this activity may range from separating a specific substance of the blood to the elaboration of a new chemical substance. 2. Any substance produced by secretion. [EU] Secretory: Secreting; relating to or influencing secretion or the secretions. [NIH] Sedative: 1. Allaying activity and excitement. 2. An agent that allays excitement. [EU] Segmentation: The process by which muscles in the intestines move food and wastes through the body. [NIH] Segregation: The separation in meiotic cell division of homologous chromosome pairs and their contained allelomorphic gene pairs. [NIH] Seizures: Clinical or subclinical disturbances of cortical function due to a sudden, abnormal, excessive, and disorganized discharge of brain cells. Clinical manifestations include abnormal motor, sensory and psychic phenomena. Recurrent seizures are usually referred to as epilepsy or "seizure disorder." [NIH] Selegiline: A selective, irreversible inhibitor of Type B monoamine oxidase. It is used in newly diagnosed patients with Parkinson's disease. It may slow progression of the clinical disease and delay the requirement for levodopa therapy. It also may be given with levodopa upon onset of disability. (From AMA Drug Evaluations Annual, 1994, p385) The compound without isomeric designation is Deprenyl. [NIH] Semisynthetic: Produced by chemical manipulation of naturally occurring substances. [EU] Senile: Relating or belonging to old age; characteristic of old age; resulting from infirmity of old age. [NIH] Sensibility: The ability to receive, feel and appreciate sensations and impressions; the

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quality of being sensitive; the extend to which a method gives results that are free from false negatives. [NIH] Sensory loss: A disease of the nerves whereby the myelin or insulating sheath of myelin on the nerves does not stay intact and the messages from the brain to the muscles through the nerves are not carried properly. [NIH] Sequela: Any lesion or affection following or caused by an attack of disease. [EU] Sequencing: The determination of the order of nucleotides in a DNA or RNA chain. [NIH] Serine: A non-essential amino acid occurring in natural form as the L-isomer. It is synthesized from glycine or threonine. It is involved in the biosynthesis of purines, pyrimidines, and other amino acids. [NIH] Serotonin: A biochemical messenger and regulator, synthesized from the essential amino acid L-tryptophan. In humans it is found primarily in the central nervous system, gastrointestinal tract, and blood platelets. Serotonin mediates several important physiological functions including neurotransmission, gastrointestinal motility, hemostasis, and cardiovascular integrity. Multiple receptor families (receptors, serotonin) explain the broad physiological actions and distribution of this biochemical mediator. [NIH] Serum: The clear liquid part of the blood that remains after blood cells and clotting proteins have been removed. [NIH] Shock: The general bodily disturbance following a severe injury; an emotional or moral upset occasioned by some disturbing or unexpected experience; disruption of the circulation, which can upset all body functions: sometimes referred to as circulatory shock. [NIH]

Side effect: A consequence other than the one(s) for which an agent or measure is used, as the adverse effects produced by a drug, especially on a tissue or organ system other than the one sought to be benefited by its administration. [EU] Signal Transduction: The intercellular or intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GABA-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptormediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway. [NIH] Signs and Symptoms: Clinical manifestations that can be either objective when observed by a physician, or subjective when perceived by the patient. [NIH] Skeletal: Having to do with the skeleton (boney part of the body). [NIH] Skeleton: The framework that supports the soft tissues of vertebrate animals and protects many of their internal organs. The skeletons of vertebrates are made of bone and/or cartilage. [NIH] Skull: The skeleton of the head including the bones of the face and the bones enclosing the brain. [NIH] Small intestine: The part of the digestive tract that is located between the stomach and the

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large intestine. [NIH] Smooth muscle: Muscle that performs automatic tasks, such as constricting blood vessels. [NIH]

Social Environment: The aggregate of social and cultural institutions, forms, patterns, and processes that influence the life of an individual or community. [NIH] Sodium: An element that is a member of the alkali group of metals. It has the atomic symbol Na, atomic number 11, and atomic weight 23. With a valence of 1, it has a strong affinity for oxygen and other nonmetallic elements. Sodium provides the chief cation of the extracellular body fluids. Its salts are the most widely used in medicine. (From Dorland, 27th ed) Physiologically the sodium ion plays a major role in blood pressure regulation, maintenance of fluid volume, and electrolyte balance. [NIH] Sodium Channels: Cell membrane glycoproteins selective for sodium ions. Fast sodium current is associated with the action potential in neural membranes. [NIH] Soft tissue: Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body. [NIH] Solitary Nucleus: Gray matter located in the dorsomedial part of the medulla oblongata associated with the solitary tract. The solitary nucleus receives inputs from most organ systems including the terminations of the facial, glossopharyngeal, and vagus nerves. It is a major coordinator of autonomic nervous system regulation of cardiovascular, respiratory, gustatory, gastrointestinal, and chemoreceptive aspects of homeostasis. The solitary nucleus is also notable for the large number of neurotransmitters which are found therein. [NIH] Somatic: 1. Pertaining to or characteristic of the soma or body. 2. Pertaining to the body wall in contrast to the viscera. [EU] Somatosensory Cortex: Area of the parietal lobe concerned with receiving general sensations. It lies posterior to the central sulcus. [NIH] Spasm: An involuntary contraction of a muscle or group of muscles. Spasms may involve skeletal muscle or smooth muscle. [NIH] Spasmodic: Of the nature of a spasm. [EU] Spatial disorientation: Loss of orientation in space where person does not know which way is up. [NIH] Specialist: In medicine, one who concentrates on 1 special branch of medical science. [NIH] Species: A taxonomic category subordinate to a genus (or subgenus) and superior to a subspecies or variety, composed of individuals possessing common characters distinguishing them from other categories of individuals of the same taxonomic level. In taxonomic nomenclature, species are designated by the genus name followed by a Latin or Latinized adjective or noun. [EU] Specificity: Degree of selectivity shown by an antibody with respect to the number and types of antigens with which the antibody combines, as well as with respect to the rates and the extents of these reactions. [NIH] Spectrum: A charted band of wavelengths of electromagnetic vibrations obtained by refraction and diffraction. By extension, a measurable range of activity, such as the range of bacteria affected by an antibiotic (antibacterial s.) or the complete range of manifestations of a disease. [EU] Sperm: The fecundating fluid of the male. [NIH] Spermidine: A polyamine formed from putrescine. It is found in almost all tissues in association with nucleic acids. It is found as a cation at all pH values, and is thought to help

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stabilize some membranes and nucleic acid structures. It is a precursor of spermine. [NIH] Spermine: A biogenic polyamine formed from spermidine. It is found in a wide variety of organisms and tissues and is an essential growth factor in some bacteria. It is found as a polycation at all pH values. Spermine is associated with nucleic acids, particularly in viruses, and is thought to stabilize the helical structure. [NIH] Spherocytes: Small, abnormal spherical red blood cells with more than the normal amount of hemoglobin. [NIH] Spherocytosis: A condition in which there are abnormally thick, almost spherical, red blood cells or spherocytes in the blood. [NIH] Spina bifida: A defect in development of the vertebral column in which there is a central deficiency of the vertebral lamina. [NIH] Spinal cord: The main trunk or bundle of nerves running down the spine through holes in the spinal bone (the vertebrae) from the brain to the level of the lower back. [NIH] Spinal Cord Diseases: Pathologic conditions which feature spinal cord damage or dysfunction, including disorders involving the meninges and perimeningeal spaces surrounding the spinal cord. Traumatic injuries, vascular diseases, infections, and inflammatory/autoimmune processes may affect the spinal cord. [NIH] Spiperone: A spiro butyrophenone analog similar to haloperidol and other related compounds. It has been recommended in the treatment of schizophrenia. [NIH] Spirochete: Lyme disease. [NIH] Sporadic: Neither endemic nor epidemic; occurring occasionally in a random or isolated manner. [EU] Staging: Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. [NIH]

Steel: A tough, malleable, iron-based alloy containing up to, but no more than, two percent carbon and often other metals. It is used in medicine and dentistry in implants and instrumentation. [NIH] Stenosis: Narrowing or stricture of a duct or canal. [EU] Stereotypy: Unvarying repetition or unvarying persistence. [NIH] Steroid: A group name for lipids that contain a hydrogenated cyclopentanoperhydrophenanthrene ring system. Some of the substances included in this group are progesterone, adrenocortical hormones, the gonadal hormones, cardiac aglycones, bile acids, sterols (such as cholesterol), toad poisons, saponins, and some of the carcinogenic hydrocarbons. [EU] Stimulant: 1. Producing stimulation; especially producing stimulation by causing tension on muscle fibre through the nervous tissue. 2. An agent or remedy that produces stimulation. [EU]

Stimulus: That which can elicit or evoke action (response) in a muscle, nerve, gland or other excitable issue, or cause an augmenting action upon any function or metabolic process. [NIH] Stomach: An organ of digestion situated in the left upper quadrant of the abdomen between the termination of the esophagus and the beginning of the duodenum. [NIH] Strand: DNA normally exists in the bacterial nucleus in a helix, in which two strands are coiled together. [NIH] Streptococcal: Caused by infection due to any species of streptococcus. [NIH] Streptococcal Infections: Infections with bacteria of the genus Streptococcus. [NIH]

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Streptococci: A genus of spherical Gram-positive bacteria occurring in chains or pairs. They are widely distributed in nature, being important pathogens but often found as normal commensals in the mouth, skin, and intestine of humans and other animals. [NIH] Streptococcus: A genus of gram-positive, coccoid bacteria whose organisms occur in pairs or chains. No endospores are produced. Many species exist as commensals or parasites on man or animals with some being highly pathogenic. A few species are saprophytes and occur in the natural environment. [NIH] Streptokinase: Streptococcal fibrinolysin . An enzyme produced by hemolytic streptococci. It hydrolyzes amide linkages and serves as an activator of plasminogen. It is used in thrombolytic therapy and is used also in mixtures with streptodornase (streptodornase and streptokinase). EC 3.4.-. [NIH] Stress: Forcibly exerted influence; pressure. Any condition or situation that causes strain or tension. Stress may be either physical or psychologic, or both. [NIH] Striatum: A higher brain's domain thus called because of its stripes. [NIH] Stricture: The abnormal narrowing of a body opening. Also called stenosis. [NIH] Stroke: Sudden loss of function of part of the brain because of loss of blood flow. Stroke may be caused by a clot (thrombosis) or rupture (hemorrhage) of a blood vessel to the brain. [NIH] Subacute: Somewhat acute; between acute and chronic. [EU] Subarachnoid: Situated or occurring between the arachnoid and the pia mater. [EU] Subclinical: Without clinical manifestations; said of the early stage(s) of an infection or other disease or abnormality before symptoms and signs become apparent or detectable by clinical examination or laboratory tests, or of a very mild form of an infection or other disease or abnormality. [EU] Subcutaneous: Beneath the skin. [NIH] Subiculum: A region of the hippocampus that projects to other areas of the brain. [NIH] Substance P: An eleven-amino acid neurotransmitter that appears in both the central and peripheral nervous systems. It is involved in transmission of pain, causes rapid contractions of the gastrointestinal smooth muscle, and modulates inflammatory and immune responses. [NIH]

Superantigens: Microbial antigens that have in common an extremely potent activating effect on T-cells that bear a specific variable region. Superantigens cross-link the variable region with class II MHC proteins regardless of the peptide binding in the T-cell receptor's pocket. The result is a transient expansion and subsequent death and anergy of the T-cells with the appropriate variable regions. [NIH] Suprachiasmatic Nucleus: An ovoid densely packed collection of small cells of the anterior hypothalamus lying close to the midline in a shallow impression of the optic chiasm. [NIH] Sweat: The fluid excreted by the sweat glands. It consists of water containing sodium chloride, phosphate, urea, ammonia, and other waste products. [NIH] Sympathetic Nervous System: The thoracolumbar division of the autonomic nervous system. Sympathetic preganglionic fibers originate in neurons of the intermediolateral column of the spinal cord and project to the paravertebral and prevertebral ganglia, which in turn project to target organs. The sympathetic nervous system mediates the body's response to stressful situations, i.e., the fight or flight reactions. It often acts reciprocally to the parasympathetic system. [NIH] Sympathomimetic: 1. Mimicking the effects of impulses conveyed by adrenergic postganglionic fibres of the sympathetic nervous system. 2. An agent that produces effects

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similar to those of impulses conveyed by adrenergic postganglionic fibres of the sympathetic nervous system. Called also adrenergic. [EU] Symptomatic: Having to do with symptoms, which are signs of a condition or disease. [NIH] Symptomatic treatment: Therapy that eases symptoms without addressing the cause of disease. [NIH] Synapse: The region where the processes of two neurons come into close contiguity, and the nervous impulse passes from one to the other; the fibers of the two are intermeshed, but, according to the general view, there is no direct contiguity. [NIH] Synapsis: The pairing between homologous chromosomes of maternal and paternal origin during the prophase of meiosis, leading to the formation of gametes. [NIH] Synaptic: Pertaining to or affecting a synapse (= site of functional apposition between neurons, at which an impulse is transmitted from one neuron to another by electrical or chemical means); pertaining to synapsis (= pairing off in point-for-point association of homologous chromosomes from the male and female pronuclei during the early prophase of meiosis). [EU] Synaptic Transmission: The communication from a neuron to a target (neuron, muscle, or secretory cell) across a synapse. In chemical synaptic transmission, the presynaptic neuron releases a neurotransmitter that diffuses across the synaptic cleft and binds to specific synaptic receptors. These activated receptors modulate ion channels and/or secondmessenger systems to influence the postsynaptic cell. Electrical transmission is less common in the nervous system, and, as in other tissues, is mediated by gap junctions. [NIH] Syncope: A temporary suspension of consciousness due to generalized cerebral schemia, a faint or swoon. [EU] Synergistic: Acting together; enhancing the effect of another force or agent. [EU] Syphilis: A contagious venereal disease caused by the spirochete Treponema pallidum. [NIH]

Syringomyelia: The presence in the spinal cord of elongated central fluid containing cavities surrounded by gliosis. [NIH] Systemic: Affecting the entire body. [NIH] Systemic lupus erythematosus: SLE. A chronic inflammatory connective tissue disease marked by skin rashes, joint pain and swelling, inflammation of the kidneys, inflammation of the fibrous tissue surrounding the heart (i.e., the pericardium), as well as other problems. Not all affected individuals display all of these problems. May be referred to as lupus. [NIH] Telangiectasia: The permanent enlargement of blood vessels, causing redness in the skin or mucous membranes. [NIH] Telencephalon: Paired anteriolateral evaginations of the prosencephalon plus the lamina terminalis. The cerebral hemispheres are derived from it. Many authors consider cerebrum a synonymous term to telencephalon, though a minority include diencephalon as part of the cerebrum (Anthoney, 1994). [NIH] Temporal: One of the two irregular bones forming part of the lateral surfaces and base of the skull, and containing the organs of hearing. [NIH] Terminalis: A groove on the lateral surface of the right atrium. [NIH] Tetani: Causal agent of tetanus. [NIH] Tetanic: Having the characteristics of, or relating to tetanus. [NIH] Tetanus: A disease caused by tetanospasmin, a powerful protein toxin produced by Clostridium tetani. Tetanus usually occurs after an acute injury, such as a puncture wound

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or laceration. Generalized tetanus, the most common form, is characterized by tetanic muscular contractions and hyperreflexia. Localized tetanus presents itself as a mild condition with manifestations restricted to muscles near the wound. It may progress to the generalized form. [NIH] Tetanus Toxin: The toxin elaborated by Clostridium tetani. It is a protein with a molecular weight of about 150,000, probably consisting of two fragments, tetanolysin being the hemolytic and tetanospasmin the neurotoxic principle. The toxin causes disruption of the inhibitory mechanisms of the CNS, thus permitting uncontrolled nervous activity, leading to fatal convulsions. [NIH] Thalamic: Cell that reaches the lateral nucleus of amygdala. [NIH] Thalamic Diseases: Disorders of the centrally located thalamus, which integrates a wide range of cortical and subcortical information. Manifestations include sensory loss, movement disorders; ataxia, pain syndromes, visual disorders, a variety of neuropsychological conditions, and coma. Relatively common etiologies include cerebrovascular disorders; craniocerebral trauma; brain neoplasms; brain hypoxia; intracranial hemorrhages; and infectious processes. [NIH] Thalamus: Paired bodies containing mostly gray substance and forming part of the lateral wall of the third ventricle of the brain. The thalamus represents the major portion of the diencephalon and is commonly divided into cellular aggregates known as nuclear groups. [NIH]

Therapeutics: The branch of medicine which is concerned with the treatment of diseases, palliative or curative. [NIH] Thermal: Pertaining to or characterized by heat. [EU] Thiamine: 3-((4-Amino-2-methyl-5-pyrimidinyl)methyl)-5-(2methylthiazolium chloride. [NIH]

hydroxyethyl)-4-

Third Ventricle: A narrow cleft inferior to the corpus callosum, within the diencephalon, between the paired thalami. Its floor is formed by the hypothalamus, its anterior wall by the lamina terminalis, and its roof by ependyma. It communicates with the fourth ventricle by the cerebral aqueduct, and with the lateral ventricles by the interventricular foramina. [NIH] Thoracic: Having to do with the chest. [NIH] Threonine: An essential amino acid occurring naturally in the L-form, which is the active form. It is found in eggs, milk, gelatin, and other proteins. [NIH] Threshold: For a specified sensory modality (e. g. light, sound, vibration), the lowest level (absolute threshold) or smallest difference (difference threshold, difference limen) or intensity of the stimulus discernible in prescribed conditions of stimulation. [NIH] Thrombin: An enzyme formed from prothrombin that converts fibrinogen to fibrin. (Dorland, 27th ed) EC 3.4.21.5. [NIH] Thrombocytes: Blood cells that help prevent bleeding by causing blood clots to form. Also called platelets. [NIH] Thrombolytic: 1. Dissolving or splitting up a thrombus. 2. A thrombolytic agent. [EU] Thrombolytic Therapy: Use of infusions of fibrinolytic agents to destroy or dissolve thrombi in blood vessels or bypass grafts. [NIH] Thrombomodulin: A cell surface glycoprotein of endothelial cells that binds thrombin and serves as a cofactor in the activation of protein C and its regulation of blood coagulation. [NIH]

Thrombopenia: Reduction in the number of platelets in the blood. [NIH]

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Thromboses: The formation or presence of a blood clot within a blood vessel during life. [NIH]

Thrombosis: The formation or presence of a blood clot inside a blood vessel. [NIH] Thymidine: A chemical compound found in DNA. Also used as treatment for mucositis. [NIH]

Thymoma: A tumor of the thymus, an organ that is part of the lymphatic system and is located in the chest, behind the breastbone. [NIH] Thymus: An organ that is part of the lymphatic system, in which T lymphocytes grow and multiply. The thymus is in the chest behind the breastbone. [NIH] Thyroid: A gland located near the windpipe (trachea) that produces thyroid hormone, which helps regulate growth and metabolism. [NIH] Tic: An involuntary compulsive, repetitive, stereotyped movement, resembling a purposeful movement because it is coordinated and involves muscles in their normal synergistic relationships; tics usually involve the face and shoulders. [EU] Tissue: A group or layer of cells that are alike in type and work together to perform a specific function. [NIH] Tissue Culture: Maintaining or growing of tissue, organ primordia, or the whole or part of an organ in vitro so as to preserve its architecture and/or function (Dorland, 28th ed). Tissue culture includes both organ culture and cell culture. [NIH] Tomography: Imaging methods that result in sharp images of objects located on a chosen plane and blurred images located above or below the plane. [NIH] Tone: 1. The normal degree of vigour and tension; in muscle, the resistance to passive elongation or stretch; tonus. 2. A particular quality of sound or of voice. 3. To make permanent, or to change, the colour of silver stain by chemical treatment, usually with a heavy metal. [EU] Tonic: 1. Producing and restoring the normal tone. 2. Characterized by continuous tension. 3. A term formerly used for a class of medicinal preparations believed to have the power of restoring normal tone to tissue. [EU] Tonicity: The normal state of muscular tension. [NIH] Torsion: A twisting or rotation of a bodily part or member on its axis. [NIH] Torticollis: Wryneck; a contracted state of the cervical muscles, producing twisting of the neck and an unnatural position of the head. [EU] Toxic: Having to do with poison or something harmful to the body. Toxic substances usually cause unwanted side effects. [NIH] Toxicity: The quality of being poisonous, especially the degree of virulence of a toxic microbe or of a poison. [EU] Toxicokinetics: Study of the absorption, distribution, metabolism, and excretion of test substances. [NIH] Toxicology: The science concerned with the detection, chemical composition, and pharmacologic action of toxic substances or poisons and the treatment and prevention of toxic manifestations. [NIH] Toxin: A poison; frequently used to refer specifically to a protein produced by some higher plants, certain animals, and pathogenic bacteria, which is highly toxic for other living organisms. Such substances are differentiated from the simple chemical poisons and the vegetable alkaloids by their high molecular weight and antigenicity. [EU] Trace element: Substance or element essential to plant or animal life, but present in

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extremely small amounts. [NIH] Tracer: A substance (such as a radioisotope) used in imaging procedures. [NIH] Trachea: The cartilaginous and membranous tube descending from the larynx and branching into the right and left main bronchi. [NIH] Traction: The act of pulling. [NIH] Transduction: The transfer of genes from one cell to another by means of a viral (in the case of bacteria, a bacteriophage) vector or a vector which is similar to a virus particle (pseudovirion). [NIH] Transfection: The uptake of naked or purified DNA into cells, usually eukaryotic. It is analogous to bacterial transformation. [NIH] Translation: The process whereby the genetic information present in the linear sequence of ribonucleotides in mRNA is converted into a corresponding sequence of amino acids in a protein. It occurs on the ribosome and is unidirectional. [NIH] Translational: The cleavage of signal sequence that directs the passage of the protein through a cell or organelle membrane. [NIH] Translocate: The attachment of a fragment of one chromosome to a non-homologous chromosome. [NIH] Translocation: The movement of material in solution inside the body of the plant. [NIH] Transmitter: A chemical substance which effects the passage of nerve impulses from one cell to the other at the synapse. [NIH] Transplantation: Transference of a tissue or organ, alive or dead, within an individual, between individuals of the same species, or between individuals of different species. [NIH] Trauma: Any injury, wound, or shock, must frequently physical or structural shock, producing a disturbance. [NIH] Tremor: Cyclical movement of a body part that can represent either a physiologic process or a manifestation of disease. Intention or action tremor, a common manifestation of cerebellar diseases, is aggravated by movement. In contrast, resting tremor is maximal when there is no attempt at voluntary movement, and occurs as a relatively frequent manifestation of Parkinson disease. [NIH] Trigeminal: Cranial nerve V. It is sensory for the eyeball, the conjunctiva, the eyebrow, the skin of face and scalp, the teeth, the mucous membranes in the mouth and nose, and is motor to the muscles of mastication. [NIH] Trinucleotide Repeat Expansion: DNA region comprised of a variable number of repetitive, contiguous trinucleotide sequences. The presence of these regions is associated with diseases such as Fragile X Syndrome and myotonic dystrophy. Many chromosome fragile sites (chromosome fragility) contain expanded trinucleotide repeats. [NIH] Trinucleotide Repeats: Microsatellite repeats consisting of three nucleotides dispersed in the euchromatic arms of chromosomes. [NIH] Tropomyosin: A protein found in the thin filaments of muscle fibers. It inhibits contraction of the muscle unless its position is modified by troponin. [NIH] Troponin: One of the minor protein components of skeletal muscle. Its function is to serve as the calcium-binding component in the troponin-tropomyosin B-actin-myosin complex by conferring calcium sensitivity to the cross-linked actin and myosin filaments. [NIH] Tryptophan: An essential amino acid that is necessary for normal growth in infants and for nitrogen balance in adults. It is a precursor serotonin and niacin. [NIH]

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Tuberculosis: Any of the infectious diseases of man and other animals caused by species of Mycobacterium. [NIH] Tumor marker: A substance sometimes found in an increased amount in the blood, other body fluids, or tissues and which may mean that a certain type of cancer is in the body. Examples of tumor markers include CA 125 (ovarian cancer), CA 15-3 (breast cancer), CEA (ovarian, lung, breast, pancreas, and gastrointestinal tract cancers), and PSA (prostate cancer). Also called biomarker. [NIH] Tyrosine: A non-essential amino acid. In animals it is synthesized from phenylalanine. It is also the precursor of epinephrine, thyroid hormones, and melanin. [NIH] Urease: An enzyme that catalyzes the conversion of urea and water to carbon dioxide and ammonia. EC 3.5.1.5. [NIH] Uremia: The illness associated with the buildup of urea in the blood because the kidneys are not working effectively. Symptoms include nausea, vomiting, loss of appetite, weakness, and mental confusion. [NIH] Urinary: Having to do with urine or the organs of the body that produce and get rid of urine. [NIH] Vaccination: Administration of vaccines to stimulate the host's immune response. This includes any preparation intended for active immunological prophylaxis. [NIH] Vaccines: Suspensions of killed or attenuated microorganisms (bacteria, viruses, fungi, protozoa, or rickettsiae), antigenic proteins derived from them, or synthetic constructs, administered for the prevention, amelioration, or treatment of infectious and other diseases. [NIH]

Vagal: Pertaining to the vagus nerve. [EU] Vagina: The muscular canal extending from the uterus to the exterior of the body. Also called the birth canal. [NIH] Vagus Nerve: The 10th cranial nerve. The vagus is a mixed nerve which contains somatic afferents (from skin in back of the ear and the external auditory meatus), visceral afferents (from the pharynx, larynx, thorax, and abdomen), parasympathetic efferents (to the thorax and abdomen), and efferents to striated muscle (of the larynx and pharynx). [NIH] Valproic Acid: A fatty acid with anticonvulsant properties used in the treatment of epilepsy. The mechanisms of its therapeutic actions are not well understood. It may act by increasing GABA levels in the brain or by altering the properties of voltage dependent sodium channels. [NIH] Valves: Flap-like structures that control the direction of blood flow through the heart. [NIH] Vascular: Pertaining to blood vessels or indicative of a copious blood supply. [EU] Vascular Headaches: A group of disorders characterized by recurrent headaches associated with abnormal dilation and constriction of cerebral blood vessels. Representative disorders from this category include migraine, cluster headache, and paroxysmal hemicrania. [NIH] Vasodilator: An agent that widens blood vessels. [NIH] Vector: Plasmid or other self-replicating DNA molecule that transfers DNA between cells in nature or in recombinant DNA technology. [NIH] Vein: Vessel-carrying blood from various parts of the body to the heart. [NIH] Venereal: Pertaining or related to or transmitted by sexual contact. [EU] Venous: Of or pertaining to the veins. [EU] Venous blood: Blood that has given up its oxygen to the tissues and carries carbon dioxide

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back for gas exchange. [NIH] Venter: Belly. [NIH] Ventilation: 1. In respiratory physiology, the process of exchange of air between the lungs and the ambient air. Pulmonary ventilation (usually measured in litres per minute) refers to the total exchange, whereas alveolar ventilation refers to the effective ventilation of the alveoli, in which gas exchange with the blood takes place. 2. In psychiatry, verbalization of one's emotional problems. [EU] Ventral: 1. Pertaining to the belly or to any venter. 2. Denoting a position more toward the belly surface than some other object of reference; same as anterior in human anatomy. [EU] Ventricle: One of the two pumping chambers of the heart. The right ventricle receives oxygen-poor blood from the right atrium and pumps it to the lungs through the pulmonary artery. The left ventricle receives oxygen-rich blood from the left atrium and pumps it to the body through the aorta. [NIH] Ventricular: Pertaining to a ventricle. [EU] Vertebral: Of or pertaining to a vertebra. [EU] Vestibule: A small, oval, bony chamber of the labyrinth. The vestibule contains the utricle and saccule, organs which are part of the balancing apparatus of the ear. [NIH] Veterinary Medicine: The medical science concerned with the prevention, diagnosis, and treatment of diseases in animals. [NIH] Villi: The tiny, fingerlike projections on the surface of the small intestine. Villi help absorb nutrients. [NIH] Viral: Pertaining to, caused by, or of the nature of virus. [EU] Virulence: The degree of pathogenicity within a group or species of microorganisms or viruses as indicated by case fatality rates and/or the ability of the organism to invade the tissues of the host. [NIH] Virus: Submicroscopic organism that causes infectious disease. In cancer therapy, some viruses may be made into vaccines that help the body build an immune response to, and kill, tumor cells. [NIH] Visceral: , from viscus a viscus) pertaining to a viscus. [EU] Visceral Afferents: The sensory fibers innervating the viscera. [NIH] Vitro: Descriptive of an event or enzyme reaction under experimental investigation occurring outside a living organism. Parts of an organism or microorganism are used together with artificial substrates and/or conditions. [NIH] Vivo: Outside of or removed from the body of a living organism. [NIH] Vocal cord: The vocal folds of the larynx. [NIH] Voice Disorders: Disorders of voice pitch, loudness, or quality. Dysphonia refers to impaired utterance of sounds by the vocal folds. [NIH] Volition: Voluntary activity without external compulsion. [NIH] Voltage-gated: It is opened by the altered charge distribution across the cell membrane. [NIH]

Vomeronasal Organ: A specialized part of the olfactory system located anteriorly in the nasal cavity within the nasal septum. Chemosensitive cells of the vomeronasal organ project via the vomeronasal nerve to the accessory olfactory bulb. The primary function of this organ appears to be in sensing pheromones which regulate reproductive and other social behaviors. While the structure has been thought absent in higher primate adults, data now

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suggests it may be present in adult humans. [NIH] Wakefulness: A state in which there is an enhanced potential for sensitivity and an efficient responsiveness to external stimuli. [NIH] White blood cell: A type of cell in the immune system that helps the body fight infection and disease. White blood cells include lymphocytes, granulocytes, macrophages, and others. [NIH]

Windpipe: A rigid tube, 10 cm long, extending from the cricoid cartilage to the upper border of the fifth thoracic vertebra. [NIH] Withdrawal: 1. A pathological retreat from interpersonal contact and social involvement, as may occur in schizophrenia, depression, or schizoid avoidant and schizotypal personality disorders. 2. (DSM III-R) A substance-specific organic brain syndrome that follows the cessation of use or reduction in intake of a psychoactive substance that had been regularly used to induce a state of intoxication. [EU] Xenograft: The cells of one species transplanted to another species. [NIH] Xerostomia: Decreased salivary flow. [NIH] X-ray: High-energy radiation used in low doses to diagnose diseases and in high doses to treat cancer. [NIH] Zymogen: Inactive form of an enzyme which can then be converted to the active form, usually by excision of a polypeptide, e. g. trypsinogen is the zymogen of trypsin. [NIH]

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INDEX 3 3-dimensional, 22, 121 A Abdomen, 121, 129, 152, 154, 175, 181 Abdominal, 89, 121, 139, 163 Abdominal Pain, 89, 121 Abulia, 82, 121 Acanthocytes, 51, 60, 121 Acetylcholine, 7, 8, 121, 133, 161 Acetylglucosamine, 36, 121 Acquired Immunodeficiency Syndrome, 5, 83, 121 Actin, 24, 121, 158, 159, 180 Acute lymphoblastic leukemia, 74, 121 Acute lymphocytic leukemia, 121 Acute renal, 121, 148 Adaptability, 121, 131, 132 Adaptation, 121, 151 Adenine, 122, 169 Adenosine, 7, 122, 164 Adrenergic, 30, 122, 125, 139, 142, 167, 176 Adverse Effect, 122, 125, 152, 164, 173 Aerobic, 122, 157 Afferent, 122, 137, 167 Affinity, 10, 11, 17, 122, 126, 160, 174 Agonist, 12, 17, 38, 122, 125, 129, 139, 152, 155, 163 Alanine, 23, 122 Albumin, 122, 165 Alexia, 122, 140 Algorithms, 122, 128 Alkaline, 122, 130 Alkaloid, 122, 129, 134, 158 Alleles, 19, 122 Allergen, 123, 138 Alternative medicine, 94, 123 Amantadine, 50, 83, 89, 123 Ameliorated, 82, 123 Amenorrhea, 123, 129 Amine, 123, 148 Amino Acid Sequence, 123, 124, 143 Amino Acids, 21, 123, 142, 163, 166, 168, 171, 173, 180 Amphetamine, 12, 51, 123, 138 Amyotrophy, 34, 60, 123 Anaesthesia, 46, 61, 123, 151 Anal, 123, 154 Analog, 123, 175

Anatomical, 12, 123, 126, 132, 150, 157, 172 Anergy, 123, 176 Aneurysm, 28, 124 Angina, 124, 167 Angina Pectoris, 124, 167 Animal model, 18, 50, 53, 124 Annealing, 124, 166 Anophthalmia, 124, 148 Antecedent, 21, 124 Anterograde, 9, 124 Antibacterial, 124, 174 Antibiotic, 89, 90, 124, 142, 163, 174 Antibiotic Prophylaxis, 89, 124 Antibodies, 14, 18, 27, 28, 59, 74, 124, 125, 127, 147, 148, 157, 165 Antibodies, Anticardiolipin, 124, 125 Antibodies, Antiphospholipid, 124, 125 Antibody, 14, 18, 27, 59, 90, 122, 124, 125, 134, 147, 148, 150, 151, 156, 157, 162, 169, 174 Anticholinergic, 89, 124 Anticoagulant, 35, 53, 62, 74, 124, 125, 168 Anticonvulsant, 124, 130, 164, 181 Antifungal, 124, 146 Antigen, 14, 122, 124, 125, 131, 134, 148, 149, 150, 151, 156, 157 Antihypertensive, 125, 163 Anti-inflammatory, 125, 126, 144, 146, 167 Anti-Inflammatory Agents, 125, 126 Antimetabolite, 125, 129 Antimicrobial, 19, 125 Antineoplastic, 125, 129 Antiphospholipid Syndrome, 32, 61, 124, 125 Antipsychotic, 89, 125, 171 Antiviral, 123, 125, 129, 146, 152 Anxiety, 77, 125, 152, 161, 167 Aorta, 125, 131, 182 Apathy, 82, 125 Apomorphine, 51, 125 Apoptosis, 15, 125 Aqueous, 126, 127, 137, 141, 153 Arginine, 126, 161 Arterial, 125, 126, 129, 132, 149, 168 Artery, 124, 126, 128, 132, 141, 143, 155, 168, 182 Articulation, 90, 126, 140 Aspirin, 5, 90, 126

186

Chorea

Assay, 10, 26, 126 Astringents, 126, 156 Astrocytes, 126, 145, 157, 160 Ataxia, 16, 27, 28, 41, 43, 60, 74, 85, 87, 109, 126, 132, 149, 178 Athetosis, 89, 126 Atrium, 126, 131, 177, 182 Atrophy, 3, 59, 110, 126, 160 Atypical, 28, 126, 171 Auditory, 31, 126, 155, 167, 181 Auscultation, 126, 147 Autoantibodies, 18, 124, 127 Autoantigens, 7, 127 Autoimmune disease, 22, 124, 127, 158, 165 Autoimmunity, 18, 127 Autonomic Nervous System, 89, 127, 164, 174, 176 Autopsy, 59, 127 Autoradiography, 12, 127 Axons, 127, 138, 152, 162, 163 B Bacteria, 90, 124, 125, 127, 138, 141, 146, 148, 149, 157, 174, 175, 176, 179, 180, 181 Bacteriostatic, 127, 142 Bacterium, 127, 148 Basal Ganglia Diseases, 126, 127, 150 Base, 85, 122, 127, 138, 152, 166, 177 Benign, 20, 26, 28, 29, 30, 33, 40, 47, 56, 57, 127, 129, 130, 147, 159 Bifida, 127 Bilateral, 30, 42, 48, 83, 127, 163 Bile, 127, 144, 154, 175 Biochemical, 8, 22, 36, 50, 84, 123, 125, 128, 144, 153, 173 Biological response modifier, 128, 151 Biological therapy, 128, 146 Biomarkers, 13, 128 Biosynthesis, 128, 173 Biotechnology, 25, 94, 103, 128 Biotin, 128, 162 Biotransformation, 128 Bipolar Disorder, 6, 128 Bladder, 128, 151, 158, 159, 168 Blepharospasm, 89, 128 Blood Coagulation, 128, 130, 178 Blood Platelets, 65, 128, 173 Blood pressure, 125, 128, 149, 157, 174 Blood-Brain Barrier, 82, 128, 153 Blot, 129, 162 Body Fluids, 128, 129, 140, 174, 181 Bone Marrow, 121, 129, 154

Bone scan, 129, 171 Bowel, 61, 123, 129, 152, 159 Bradykinin, 129, 161, 165 Brain Diseases, 129, 169 Brain Infarction, 3, 129 Brain Neoplasms, 129, 149, 178 Brain Stem, 129, 132, 169 Brain Stem Infarctions, 129 Bromocriptine, 31, 38, 44, 89, 129 Bromodeoxyuridine, 11, 129 Bronchial, 129, 148 Bronchopulmonary, 49, 129, 130 Bronchopulmonary Dysplasia, 49, 130 Buccal, 130, 154 Bulbar, 130, 169 Butyric Acid, 82, 130 C Calcification, 60, 130 Calcium, 21, 130, 134, 157, 171, 173, 180 Carbamazepine, 34, 63, 130 Carbohydrates, 130, 131 Carbon Dioxide, 89, 130, 137, 170, 181 Carbon Monoxide Poisoning, 75, 130 Carbonate Dehydratase, 130 Carbonic Anhydrase Inhibitors, 16, 130 Carcinogenic, 130, 151, 175 Carcinoid, 130, 157 Carcinoma, 59, 130 Cardiac, 5, 18, 21, 130, 142, 143, 147, 159, 175 Cardiolipins, 125, 131 Cardiomegaly, 90, 131 Cardiomyopathy, 131 Cardiopulmonary, 22, 31, 131 Cardiopulmonary Bypass, 22, 31, 131 Cardioselective, 131, 167 Cardiovascular, 123, 131, 173, 174 Case report, 4, 29, 30, 32, 35, 37, 38, 41, 62, 66, 131, 133 Cataracts, 40, 131 Catecholamine, 131, 139 Caudal, 131, 150, 166 Caudate Nucleus, 14, 74, 127, 131, 136, 159 Causal, 9, 131, 148, 177 Cell Death, 15, 20, 125, 131, 159 Cell Differentiation, 131, 173 Cell Division, 127, 131, 146, 156, 157, 165, 167, 172 Cell Extracts, 15, 131 Cell membrane, 10, 131, 138, 144, 164, 174, 182 Cell proliferation, 11, 131, 173

187

Cell Respiration, 131, 157, 170 Cell Size, 131, 144 Cell Survival, 132, 146 Central Nervous System Infections, 132, 147, 149 Cerebellar, 16, 60, 74, 109, 126, 132, 170, 180 Cerebellar Diseases, 126, 132, 180 Cerebellum, 16, 90, 129, 132, 136, 166, 170 Cerebral, 8, 9, 11, 22, 23, 31, 35, 42, 65, 82, 83, 87, 126, 127, 128, 129, 132, 136, 138, 141, 142, 143, 144, 147, 149, 163, 169, 177, 178, 181 Cerebral Cortex, 9, 23, 65, 82, 83, 126, 129, 132, 142, 143, 144, 169 Cerebral hemispheres, 127, 129, 132, 177 Cerebral Infarction, 129, 132, 149 Cerebral Palsy, 22, 87, 132 Cerebrospinal, 35, 60, 132, 149 Cerebrospinal fluid, 35, 60, 132, 149 Cerebrovascular, 88, 90, 127, 132, 178 Cerebrum, 132, 136, 177 Cervical, 132, 147, 164, 179 Character, 124, 132, 137 Chin, 83, 132, 156 Cholesterol, 127, 133, 175 Choline, 82, 133 Cholinergic, 4, 8, 125, 133, 158 Cholinergic Agents, 4, 133 Choreatic Disorders, 133 Chromatin, 126, 133, 142, 154 Chromosome, 27, 29, 64, 133, 148, 153, 172, 180 Chromosome Abnormalities, 133, 148 Chromosome Fragility, 133, 180 Chronic, 3, 4, 5, 12, 19, 42, 130, 133, 151, 155, 176, 177 Clamp, 8, 17, 133 Clear cell carcinoma, 133, 138 Cleft Lip, 133, 148 Cleft Palate, 133, 148 Clinical study, 133, 136 Clinical trial, 6, 13, 103, 133, 136, 139, 169 Clonic, 128, 133 Cloning, 20, 128, 133 Clot Retraction, 134, 165 Coagulation, 125, 128, 134, 148, 165 Coca, 134 Cocaine, 12, 134 Cofactor, 134, 161, 168, 178 Cognition, 23, 134 Collagen, 134, 136, 144, 165

Coloboma, 134, 148 Combinatorial, 21, 134 Complement, 134, 135, 145, 165 Complementary and alternative medicine, 73, 79, 135 Complementary medicine, 73, 135 Complete remission, 135, 170 Compulsions, 135, 161 Computational Biology, 103, 135 Computed tomography, 135, 171 Computerized axial tomography, 135, 172 Conception, 135, 136, 144 Confounding, 6, 135 Confusion, 135, 139, 149, 181 Congestion, 125, 135, 142 Congestive heart failure, 5, 90, 135 Conjugated, 41, 135, 137, 146 Conjunctiva, 135, 180 Connective Tissue, 5, 125, 129, 134, 136, 144, 163, 177 Connective Tissue Cells, 136 Connective Tissue Diseases, 125, 136 Consciousness, 136, 138, 139, 168, 169, 177 Constitutional, 136, 159 Constriction, 136, 152, 181 Contraceptive, 59, 136 Contraindications, ii, 136 Contralateral, 35, 136, 162, 170 Controlled clinical trial, 53, 136 Controlled study, 50, 136 Convulsions, 124, 136, 149, 178 Coordination, 4, 16, 89, 132, 136, 158 Corpus, 136, 145, 154, 159, 167, 178 Corpus Striatum, 136, 145, 159 Corpuscle, 136, 142 Cortex, 9, 23, 25, 73, 136, 142, 167 Cortical, 9, 25, 136, 143, 167, 172, 178 Cortices, 25, 136 Corticosteroids, 5, 65, 136, 146, 157, 167 Cortisone, 136, 167 Cranial, 87, 132, 136, 137, 142, 145, 147, 148, 149, 152, 161, 162, 163, 164, 169, 180, 181 Cranial Nerves, 87, 137 Craniocerebral Trauma, 127, 137, 147, 149, 178 Cribriform, 137, 161 Crossing-over, 137, 170 Crowns, 4, 137 Curative, 78, 137, 178 Cutaneous, 5, 9, 137, 154 Cyclic, 137, 146, 161, 172

188

Chorea

Cyclopia, 137, 148 Cytochrome, 137, 162 Cytokine, 18, 137 Cytoplasm, 13, 126, 131, 137, 142, 146, 154, 171 Cytoskeleton, 24, 137 Cytotoxic, 18, 137, 173 D Decarboxylation, 137, 148 Degenerative, 11, 22, 84, 89, 108, 110, 137, 145, 158, 171 Deletion, 8, 41, 126, 137 Delirium, 88, 125, 137 Delusions, 138, 168 Dementia, 3, 4, 5, 9, 13, 21, 23, 37, 45, 61, 62, 65, 82, 83, 88, 121, 125, 138, 160 Denaturation, 138, 166 Dendrites, 138, 159, 160, 162 Dendritic, 24, 138 Dental Caries, 138, 144 Dentate Gyrus, 138, 148 Depolarization, 138, 173 DES, 41, 138 Desensitization, 15, 138 Dextroamphetamine, 123, 138, 156 Diabetes Mellitus, 48, 139, 146, 147 Diagnostic procedure, 81, 94, 139 Diaphragm, 89, 139, 164 Digestion, 127, 129, 139, 152, 154, 175 Dilated cardiomyopathy, 28, 139 Dilation, 129, 139, 149, 181 Direct, iii, 19, 97, 139, 155, 170, 177 Disease Susceptibility, 14, 139 Disorientation, 135, 138, 139 Dissociation, 122, 139 Dizziness, 90, 139 Dopa, 37, 52, 66, 139, 153 Dopamine Agonists, 11, 139 Dorsal, 23, 139, 166 Dorsum, 139 Double-blind, 41, 139 Drug Interactions, 98, 140 Duct, 140, 171, 175 Dura mater, 140, 156, 162 Dysarthria, 90, 140 Dyskinesia, 4, 125, 140 Dyslexia, 8, 140 Dysphonia, 89, 140, 182 Dystonia, 7, 29, 32, 35, 37, 42, 49, 54, 60, 61, 70, 89, 125, 140 Dystrophin, 140, 158

E Echocardiography, 66, 140 Effector, 121, 134, 140 Efferent, 137, 140, 158 Efficacy, 16, 34, 38, 140, 154 Electrolyte, 138, 140, 157, 166, 174 Electromyography, 108, 140 Electrons, 127, 140, 152, 155, 169 Elementary Particles, 140, 155, 168 Emaciation, 121, 140 Embolus, 141, 151 Emetic, 125, 141 Emulsion, 127, 141 Encephalitis, 45, 59, 89, 141 Encephalitis, Viral, 141 Encephalocele, 141, 159 Encephalopathy, 17, 47, 76, 88, 141 Endemic, 141, 175 Endogenous, 13, 127, 139, 141 Endorphins, 141, 161 Endothelial cell, 128, 141, 178 Endothelium, 141, 161, 165 Endothelium-derived, 141, 161 Endotoxic, 141, 153 Enkephalin, 13, 141 Entorhinal Cortex, 142, 148 Environmental Health, 102, 104, 142 Enzymatic, 130, 135, 138, 142, 148, 166 Enzyme, 130, 140, 142, 146, 157, 161, 165, 166, 168, 173, 176, 178, 181, 182, 183 Eosinophilia, 74, 142 Eosinophils, 142, 146 Epidemic, 142, 175 Epinephrine, 122, 139, 142, 161, 181 Epistaxis, 89, 142 Ergot, 129, 142, 155, 156 Ergot Alkaloids, 142, 157 Erythema, 5, 88, 90, 142 Erythrocyte Membrane, 65, 142 Erythrocytes, 26, 121, 129, 130, 142, 148, 170 Erythromycin, 5, 142 Estrogen, 142, 167 Ethmoid, 142, 161 Eukaryotic Cells, 142, 150, 162 Evoke, 142, 175 Excitability, 15, 143 Excitation, 143, 144, 160 Excitatory, 24, 143, 146, 152 Excitatory Amino Acid Agonists, 143, 152 Exogenous, 128, 141, 143, 145 Exon, 17, 143

189

Exotoxin, 14, 143 Expiratory, 143, 164 Exploratory Behavior, 83, 143 Extracellular, 8, 12, 126, 136, 143, 144, 160, 174 Extracellular Matrix, 136, 143, 144 Extrapyramidal, 12, 22, 123, 125, 139, 143 Extravasation, 143, 147 Eye Movements, 36, 143, 169 F Facial, 36, 76, 87, 90, 143, 148, 155, 174 Facial Pain, 87, 143 Facial Paralysis, 87, 90, 143 Family Planning, 103, 143 Famotidine, 82, 143 Fat, 129, 130, 141, 143, 153, 158, 174 Femoral, 131, 143 Femoral Artery, 131, 143 Fetus, 144, 167 Fibrin, 128, 134, 144, 165, 178 Fibroblasts, 36, 136, 144 Fibrosis, 144, 172 Fissure, 133, 134, 138, 144, 167 Flow Cytometry, 18, 144 Fludrocortisone, 90, 144 Fluorescence, 144 Fluorescent Dyes, 144 Fluorine, 11, 144 Fossa, 132, 144 Frontal Lobe, 82, 83, 132, 144, 158, 167 G Gait, 4, 132, 144 Gallbladder, 121, 144 Gamma Rays, 144, 158, 169 Gamma-hydroxybutyrate, 51, 144 Ganglia, 7, 9, 15, 18, 22, 27, 30, 32, 38, 42, 46, 48, 59, 60, 74, 82, 121, 125, 127, 129, 144, 153, 159, 164, 169, 176 Gap Junctions, 144, 177 Gas, 130, 144, 149, 158, 161, 182 Gasoline, 76, 145 Gastric, 143, 145, 148, 169 Gastrin, 145, 148 Gastrointestinal, 129, 130, 142, 145, 156, 169, 173, 174, 176, 181 Gastrointestinal tract, 145, 173, 181 Gene, 6, 11, 12, 13, 16, 20, 23, 24, 40, 43, 47, 56, 57, 122, 128, 140, 145, 149, 162, 172 Gene Expression, 6, 13, 145 Gene Expression Profiling, 6, 145 Gene Targeting, 24, 145 Genetic Engineering, 128, 133, 145

Genetic testing, 145, 166 Genetics, 12, 27, 29, 30, 38, 40, 43, 44, 47, 56, 57, 59, 64, 88, 145 Genotype, 145, 164 Germ Cells, 145, 156, 162 Gland, 136, 145, 149, 163, 168, 172, 175, 179 Gliosis, 61, 145, 177 Globus Pallidus, 15, 22, 57, 83, 127, 136, 145, 169 Glomerular, 145, 170 Glomeruli, 145, 162 Glomerulonephritis, 14, 145, 150 Glomerulus, 14, 145, 159 Glossopharyngeal Nerve, 143, 145 Glottis, 89, 146 Glucocorticoid, 146, 157, 167 Glucose, 48, 139, 146, 147, 149, 151, 171 Glucose Intolerance, 139, 146 Glutamate, 15, 17, 22, 24, 146, 156 Glutamic Acid, 146, 161 Glycerol, 130, 131, 146, 164 Glycine, 17, 146, 161, 173 Glycoproteins, 146, 152, 174 Gonadal, 146, 175 Governing Board, 146, 166 Gram-positive, 146, 176 Granulocytes, 146, 173, 183 Gravis, 88, 90, 146 Growth factors, 20, 146, 157, 159 Guanidines, 10, 146 Guanylate Cyclase, 146, 161 H Haematoma, 147 Haemorrhage, 60, 147 Haloperidol, 34, 98, 147, 175 Haptens, 122, 147 Headache, 90, 147, 149, 181 Headache Disorders, 147 Heart failure, 147 Heart Murmurs, 90, 147 Heart Sounds, 147 Hematoma, 3, 147 Hemiplegia, 126, 147 Hemoglobin, 142, 147, 175 Hemolysis, 142, 148 Hemolytic, 18, 21, 148, 176, 178 Hemorrhage, 137, 147, 148, 169, 176 Hemostasis, 148, 173 Hepatic, 122, 138, 148 Hereditary, 4, 26, 28, 29, 30, 33, 40, 44, 45, 47, 56, 89, 133, 136, 148, 158, 160

190

Chorea

Heredity, 5, 145, 148 Hermetic, 4, 148 Heterogeneity, 33, 39, 122, 148 Hippocampus, 17, 83, 138, 148, 153, 176 Histamine, 53, 82, 83, 125, 143, 148, 169 Histidine, 148 Holoprosencephaly, 42, 148 Homeobox, 23, 148 Homologous, 16, 123, 137, 145, 148, 172, 177, 180 Hormonal, 126, 148 Hormone, 44, 45, 136, 138, 142, 145, 148, 156, 167, 172, 173, 179 Human growth hormone, 30, 148 Hybrid, 23, 149, 162 Hybridization, 149, 162 Hydrocephalus, 3, 149, 152 Hydrogen, 123, 127, 130, 138, 149, 157, 168 Hydrolysis, 128, 149, 164, 166, 168 Hyperglycaemia, 35, 149 Hyperglycemia, 32, 34, 43, 46, 149 Hyperkinesia, 4, 35, 149 Hyperplasia, 8, 149 Hyperreflexia, 149, 178 Hypersensitivity, 123, 138, 149 Hypertension, 149, 152, 167 Hyperthyroidism, 47, 149, 167 Hypertrophy, 131, 149 Hypnotherapy, 77, 149 Hypoglossal Nerve, 87, 149 Hypoglycaemia, 49, 138, 149 Hypokinesia, 149, 163 Hypothalamic, 8, 150 Hypothalamus, 30, 83, 127, 129, 141, 150, 153, 176, 178 Hypothermia, 149, 150 Hypoxia, 138, 150, 178 Hypoxic, 88, 150 I Idiopathic, 29, 60, 88, 89, 150 Imaging procedures, 150, 180 Imidazole, 128, 148, 150, 169 Immune Complex Diseases, 150, 165 Immune response, 19, 34, 123, 125, 127, 136, 147, 150, 176, 181, 182 Immune system, 127, 128, 150, 158, 183 Immunity, 121, 122, 150 Immunodeficiency, 121, 150 Immunogenic, 150, 153 Immunologic, 19, 150 Immunology, 17, 19, 122, 144, 150 Immunotherapy, 128, 138, 150

Impairment, 8, 60, 88, 121, 126, 138, 140, 150, 156, 168 In situ, 17, 150 In Situ Hybridization, 17, 150 In vitro, 10, 12, 15, 55, 150, 166, 179 In vivo, 10, 15, 18, 48, 150 Incision, 151, 152 Incontinence, 149, 151 Induction, 70, 125, 151, 167 Infarction, 35, 57, 132, 151 Infection, 5, 8, 18, 19, 21, 90, 109, 121, 128, 138, 141, 150, 151, 154, 160, 162, 163, 175, 176, 183 Infertility, 129, 151 Infiltration, 145, 151 Inflammation, 122, 125, 126, 141, 144, 151, 156, 159, 162, 163, 164, 165, 166, 171, 177 Inhalation, 76, 151, 166 Initiation, 15, 90, 151 Innervation, 149, 151 Inositol, 151, 156, 172 Inotropic, 139, 151 Insight, 19, 151 Institutionalization, 6, 151 Insulator, 151, 158 Interferon, 35, 61, 151, 152, 154 Interferon-alpha, 61, 151, 152 Interneurons, 16, 23, 152 Interstitial, 152, 159, 170 Intestinal, 152, 155 Intestine, 129, 152, 174, 176 Intoxication, 4, 75, 88, 138, 152, 183 Intracellular, 151, 152, 156, 161, 166, 170, 172, 173 Intracellular Membranes, 152, 156 Intracranial Hemorrhages, 149, 152, 178 Intracranial Hypertension, 147, 149, 152 Intramuscular, 5, 152 Intramuscular injection, 5, 152 Intrinsic, 15, 122, 152 Invasive, 10, 22, 150, 152, 155 Involuntary, 11, 22, 49, 75, 89, 110, 127, 152, 159, 161, 165, 170, 174, 179 Ion Channels, 24, 126, 152, 160, 164, 177 Ions, 127, 130, 139, 140, 149, 152, 171, 174 Ischemia, 17, 20, 22, 124, 126, 152 K Kainic Acid, 21, 45, 49, 152 Kava, 75, 77, 152 Kb, 102, 152 Kinetic, 153

191

L Labyrinth, 153, 182 Laceration, 153, 178 Lactation, 153, 167 Larynx, 146, 153, 180, 181, 182 Latency, 53, 153 Lectin, 153, 156 Lens, 131, 153 Lesion, 32, 145, 153, 154, 169, 173 Lethargy, 149, 153 Leukemia, 153 Levo, 139, 153 Levodopa, 29, 51, 52, 53, 89, 139, 153, 172 Ligands, 10, 11, 153 Limbic, 9, 59, 153, 167 Limbic System, 153, 167 Linkage, 43, 75, 153 Lipid, 39, 133, 146, 153, 158 Lipid A, 39, 153 Lipopolysaccharides, 153 Liver, 4, 84, 88, 121, 122, 127, 128, 141, 144, 148, 154, 167, 171 Liver scan, 154, 171 Lobe, 83, 132, 148, 154 Localization, 13, 23, 24, 154 Localized, 16, 138, 140, 147, 151, 154, 165, 178 Locomotion, 154, 165 Locomotor, 12, 154 Longitudinal Studies, 12, 154 Longitudinal study, 18, 154 Long-Term Potentiation, 17, 154 Lupus, 26, 31, 35, 46, 53, 54, 62, 74, 124, 125, 154, 177 Lutein Cells, 154, 167 Lymphatic, 141, 151, 154, 179 Lymphatic system, 154, 179 Lymphoblastic, 154 Lymphoblasts, 121, 154 Lymphocyte Count, 121, 154 Lymphocytes, 121, 125, 154, 179, 183 Lymphoid, 124, 136, 154, 155 Lymphoma, 61, 154, 155 Lysergic acid, 155, 156 Lysergic Acid Diethylamide, 155, 156 M Magnetic Resonance Imaging, 19, 155, 172 Magnetic Resonance Spectroscopy, 60, 155 Malabsorption, 47, 155 Malformation, 76, 155 Malignant, 121, 125, 129, 155, 159

Malnutrition, 122, 126, 155 Mammogram, 130, 155, 157 Manganese Poisoning, 54, 155 Mania, 76, 155 Manic, 125, 128, 155, 168 Manic-depressive psychosis, 155, 168 Manifest, 88, 147, 155 Mastication, 155, 180 Masticatory, 54, 155 Meatus, 155, 181 Medial, 133, 142, 145, 155, 162 Mediate, 9, 139, 155, 169 Mediator, 139, 155, 173 MEDLINE, 103, 156 Medullary, 156, 169 Meiosis, 156, 177 Membrane Proteins, 39, 156 Memory, 8, 17, 25, 88, 138, 154, 156 Meninges, 132, 137, 140, 156, 175 Meningitis, 3, 156 Menopause, 156, 167 Mental Disorders, 88, 150, 156, 168 Mental Health, iv, 5, 88, 102, 104, 156, 168 Mental Retardation, 148, 156 Mercury, 88, 144, 156 Meta-Analysis, 32, 156 Metabotropic, 15, 156 Methamphetamine, 12, 156 Methysergide, 58, 156 Microbe, 157, 179 Microcalcifications, 130, 157 Microglia, 126, 157, 160 Microorganism, 134, 157, 182 Mineralization, 38, 157 Mineralocorticoid, 144, 157 Mitochondria, 85, 157, 162 Mitosis, 126, 157 Modification, 145, 157, 169 Molecule, 14, 21, 125, 127, 131, 134, 139, 140, 141, 143, 149, 153, 157, 166, 170, 173, 181 Monitor, 10, 19, 157, 161 Monoamine, 11, 54, 123, 138, 157, 172 Monoclonal, 18, 157, 169 Monoclonal antibodies, 18, 157 Morphine, 125, 158 Motility, 158, 173 Motor Activity, 136, 158, 169 Motor Cortex, 49, 158, 170 Motor nerve, 158, 161, 164 Mucins, 146, 158, 171 Mucosa, 154, 158, 167

192

Chorea

Mucositis, 158, 179 Multiple sclerosis, 82, 83, 88, 90, 158 Muscarinic Agonists, 133, 158 Muscle Contraction, 22, 140, 158, 171 Muscle Fibers, 158, 159, 180 Muscle Proteins, 27, 158 Muscular Diseases, 143, 158, 160, 169 Musculature, 4, 149, 158 Mustard Gas, 158 Mutagen, 64, 158 Mutagenesis, 17, 24, 159 Myasthenia, 85, 88, 90, 159 Myelin, 158, 159, 160, 173 Myocardial infarction, 159, 167 Myocardium, 124, 159, 171 Myoclonus, 40, 41, 89, 159 Myosin, 18, 158, 159, 180 Myotonic Dystrophy, 159, 180 N Necrosis, 126, 129, 132, 151, 159 Neoplasms, 121, 125, 129, 159 Neostriatum, 9, 22, 131, 136, 159, 169 Nephritis, 14, 159 Nephron, 145, 159 Nervous System, 3, 8, 13, 21, 46, 77, 88, 89, 110, 121, 122, 123, 127, 129, 132, 134, 138, 140, 144, 146, 153, 155, 156, 157, 158, 159, 160, 164, 166, 173, 176, 177 Neural, 9, 17, 27, 38, 58, 87, 122, 141, 157, 159, 167, 174 Neural tube defects, 87, 159 Neurites, 65, 159 Neurodegenerative Diseases, 20, 127, 160 Neuroglia, 145, 160 Neurologic, 5, 7, 87, 90, 141, 149, 160 Neurologist, 4, 33, 160 Neuromuscular, 108, 121, 143, 160, 169, 171 Neuromuscular Diseases, 160, 169 Neuromuscular Junction, 121, 160, 171 Neuromuscular Junction Diseases, 160, 171 Neuronal, 8, 9, 17, 21, 28, 54, 57, 58, 59, 160, 163 Neuropathy, 88, 123, 160 Neuropeptides, 54, 160 Neurophysiology, 54, 67, 74, 76, 90, 138, 160 Neurosis, 77, 160 Neurosyphilis, 5, 160, 163 Neurotoxic, 160, 178 Neurotoxicity, 152, 160

Neurotoxin, 50, 160 Neurotransmitter, 10, 121, 122, 129, 139, 146, 148, 152, 160, 161, 172, 173, 176, 177 Nickel, 20, 161 Nitric Oxide, 20, 161 Norepinephrine, 122, 139, 161 Nuclear, 27, 28, 127, 140, 142, 144, 153, 159, 161, 178 Nuclei, 7, 15, 22, 30, 57, 140, 143, 145, 153, 155, 157, 161, 168 Nucleic acid, 149, 150, 161, 162, 169, 174, 175 Nystagmus, 40, 161 O Observational study, 54, 161 Obsessive-Compulsive Disorder, 14, 18, 21, 161 Oculi, 128, 161 Oculomotor, 9, 12, 37, 161 Olfactory Bulb, 23, 161, 162, 182 Olfactory Nerve, 161, 162 Oligonucleotide Probes, 17, 162 Oocytes, 17, 162 Opacity, 131, 162 Opiate, 141, 158, 162 Opportunistic Infections, 121, 162 Optic Chiasm, 150, 162, 176 Oral Health, 87, 162 Oral Hygiene, 4, 162 Oral surgeon, 4, 162 Orbicularis, 128, 162 Organ Culture, 162, 179 Organelles, 137, 162 Orofacial, 143, 162 Ovum, 162, 167 Oxidative Phosphorylation, 85, 162 Oxygenator, 131, 162 P Pachymeningitis, 156, 162 Palliative, 162, 178 Palsy, 3, 5, 88, 89, 163 Pancreas, 121, 128, 163, 181 Paralysis, 84, 90, 130, 143, 163, 169 Paresis, 143, 147, 163 Parietal, 25, 76, 163, 174 Parietal Lobe, 76, 163, 174 Parkinsonism, 37, 42, 45, 47, 62, 125, 153, 163 Paroxysmal, 60, 75, 76, 124, 147, 163, 181 Partial remission, 163, 170 Particle, 163, 180 Parturition, 163, 167

193

Patch, 8, 17, 62, 163 Pathologic, 126, 129, 149, 163, 170, 175 Pathologic Processes, 126, 163 Pathophysiology, 5, 10, 11, 22, 163 Patient Education, 109, 114, 116, 119, 163 Penicillin, 5, 53, 63, 90, 163 Peptide, 163, 166, 168, 176 Pergolide, 89, 163 Pericarditis, 90, 163 Pericardium, 163, 177 Peripheral blood, 152, 163 Peripheral Nerves, 163, 164, 166 Peripheral Nervous System, 140, 147, 160, 163, 164, 169, 176 Peripheral Nervous System Diseases, 147, 160, 164, 169 Petechiae, 147, 164 Petroleum, 145, 164 Pharmacodynamic, 27, 143, 164 Pharmacokinetic, 164 Pharmacologic, 22, 164, 179 Pharyngitis, 5, 91, 164 Pharynx, 164, 181 Phenotypes, 19, 164 Phenytoin, 130, 164 Phonation, 90, 164 Phospholipases, 164, 173 Phospholipids, 124, 125, 131, 143, 151, 164 Phosphorus, 130, 164 Phosphorylation, 15, 22, 48, 164 Phrenic Nerve, 164, 171 Physiologic, 9, 122, 128, 139, 150, 159, 165, 170, 180 Physiology, 9, 122, 160, 165, 182 Piloerection, 149, 165 Piracetam, 37, 165 Pitch, 165, 182 Plants, 122, 130, 133, 134, 146, 153, 161, 165, 171, 179 Plasma, 21, 29, 41, 122, 124, 131, 140, 146, 147, 148, 157, 165 Plasma cells, 124, 165 Plasma Exchange, 21, 165 Plasma protein, 122, 165 Plasmapheresis, 19, 55, 165 Plasmin, 14, 165 Plasminogen Activators, 165 Platelet Activation, 165, 173 Platelet Aggregation, 161, 165 Platelets, 36, 65, 66, 161, 165, 178 Pneumonia, 89, 136, 165 Point Mutation, 16, 85, 165

Poisoning, 125, 138, 142, 152, 156, 166 Polyarthritis, 66, 166 Polymerase, 17, 26, 35, 166 Polymerase Chain Reaction, 17, 26, 35, 166 Polymorphic, 27, 138, 166 Polyneuritis, 88, 166 Polypeptide, 21, 123, 134, 149, 165, 166, 167, 183 Polysaccharide, 125, 166 Pons, 129, 143, 166 Posterior, 23, 25, 123, 126, 132, 139, 146, 162, 163, 166, 174 Postherpetic Neuralgia, 123, 166 Postsynaptic, 15, 16, 24, 160, 166, 173, 177 Post-traumatic, 147, 158, 166 Potassium, 16, 157, 166 Potentiates, 13, 166 Potentiation, 17, 154, 166, 173 Practice Guidelines, 104, 166 Precursor, 133, 139, 140, 141, 142, 153, 161, 166, 175, 180, 181 Prednisolone, 167 Prednisone, 90, 167 Prefrontal Cortex, 25, 167 Prenatal, 11, 167 Presynaptic, 16, 160, 167, 177 Probe, 10, 162, 167 Progesterone, 167, 175 Progression, 13, 88, 124, 167, 172 Progressive, 3, 4, 5, 57, 60, 89, 131, 138, 159, 160, 165, 167, 170 Projection, 23, 152, 161, 162, 167, 170 Prolactin, 44, 129, 167 Prophase, 162, 167, 177 Prophylaxis, 19, 91, 167, 181 Propranolol, 89, 167 Prosencephalon, 148, 167, 177 Prospective study, 154, 167 Prostate, 128, 168, 181 Prosthesis, 4, 168 Protein C, 13, 14, 122, 123, 158, 168, 180 Protein S, 15, 24, 128, 142, 148, 168, 171 Proteins, 14, 15, 20, 23, 24, 27, 85, 123, 125, 128, 129, 131, 133, 134, 140, 142, 144, 149, 156, 157, 158, 163, 165, 168, 170, 173, 176, 178, 181 Proteolytic, 134, 165, 168 Protons, 149, 155, 168, 169 Proximal, 130, 167, 168 Psychiatric, 7, 8, 19, 25, 34, 57, 63, 82, 83, 88, 109, 156, 168 Psychic, 156, 160, 168, 172

194

Chorea

Psychoactive, 168, 183 Psychosis, 62, 125, 168 Psychotomimetic, 123, 139, 168 Public Health, 21, 104, 168 Public Policy, 103, 168 Pulmonary, 128, 130, 147, 168, 182 Pulse, 157, 168 Purines, 168, 173 Purpura, 147, 169 Putamen, 22, 23, 46, 127, 136, 159, 169 Pyramidal Tracts, 143, 169 Pyrimidines, 169, 173 Pyrogenic, 14, 169 Q Quadriplegia, 90, 169 Quality of Life, 20, 169 R Race, 139, 169 Racemic, 139, 169 Radiation, 121, 124, 127, 140, 144, 169, 172, 183 Radiation therapy, 121, 169 Radioactive, 127, 129, 149, 154, 158, 161, 169, 172 Radioisotope, 162, 169, 180 Radiolabeled, 10, 169 Randomized, 50, 140, 169 Ranitidine, 82, 169 Reality Testing, 168, 170 Receptors, Serotonin, 170, 173 Recombinant, 14, 15, 17, 61, 170, 181 Recombinant Proteins, 15, 170 Recombination, 16, 145, 170 Recur, 5, 170 Recurrence, 63, 128, 155, 170 Red blood cells, 142, 148, 170, 171, 175 Red Nucleus, 126, 170 Refer, 1, 130, 134, 139, 141, 152, 154, 159, 168, 170, 179 Reflex, 54, 143, 170 Refraction, 170, 174 Regimen, 140, 170 Remission, 55, 128, 155, 170 Renal failure, 56, 138, 170 Research Design, 19, 170 Resorption, 149, 170 Respiration, 130, 157, 170, 171 Respiratory distress syndrome, 130, 170 Respiratory Paralysis, 88, 171 Retina, 153, 160, 162, 171 Retinoid, 24, 171 Retinopathy, 77, 171

Retrograde, 9, 171 Rheumatic Heart Disease, 88, 171 Ribose, 122, 171 Ribosome, 171, 180 Rigidity, 35, 89, 163, 165, 171 Risk factor, 62, 63, 167, 171 Risperidone, 37, 62, 171 Rod, 60, 127, 133, 171 S Saccule, 171, 182 Saline, 165, 171 Saliva, 4, 171 Salivary, 171, 183 Salivary glands, 171 Saponins, 171, 175 Sarcoplasmic Reticulum, 21, 171 Scans, 19, 171 Schizoid, 172, 183 Schizophrenia, 6, 7, 8, 9, 10, 12, 22, 23, 25, 42, 61, 82, 83, 171, 172, 175, 183 Schizotypal Personality Disorder, 172, 183 Sclerosis, 20, 83, 90, 158, 172 Screening, 14, 133, 172 Second Messenger Systems, 9, 20, 172 Secretion, 129, 130, 143, 148, 153, 157, 158, 169, 172 Secretory, 172, 177 Sedative, 152, 172 Segmentation, 148, 172 Segregation, 170, 172 Seizures, 130, 138, 148, 163, 164, 172 Selegiline, 89, 172 Semisynthetic, 129, 155, 172 Senile, 9, 21, 26, 89, 172 Sensibility, 123, 172 Sensory loss, 87, 173, 178 Sequela, 18, 90, 173 Sequencing, 166, 173 Serine, 17, 173 Serotonin, 7, 125, 155, 156, 161, 170, 171, 173, 180 Serum, 14, 34, 122, 134, 150, 157, 173 Shock, 159, 173, 180 Side effect, 12, 22, 97, 122, 125, 128, 142, 173, 179 Signal Transduction, 9, 151, 173 Signs and Symptoms, 170, 173 Skeletal, 133, 158, 171, 173, 174, 180 Skeleton, 21, 121, 173 Skull, 137, 141, 159, 173, 177 Small intestine, 148, 152, 173, 182

195

Smooth muscle, 136, 142, 148, 156, 158, 174, 176 Social Environment, 169, 174 Sodium, 37, 63, 70, 157, 174, 176, 181 Sodium Channels, 174, 181 Soft tissue, 129, 173, 174 Solitary Nucleus, 127, 174 Somatic, 137, 145, 153, 156, 157, 164, 167, 174, 181 Somatosensory Cortex, 9, 174 Spasm, 54, 126, 128, 160, 174 Spasmodic, 89, 174 Spatial disorientation, 139, 174 Specialist, 111, 139, 174 Species, 142, 149, 156, 157, 169, 174, 175, 176, 180, 181, 182, 183 Specificity, 14, 122, 174 Spectrum, 21, 37, 42, 56, 157, 174 Sperm, 133, 174 Spermidine, 174, 175 Spermine, 17, 175 Spherocytes, 175 Spherocytosis, 34, 175 Spina bifida, 87, 159, 175 Spinal cord, 126, 129, 132, 133, 140, 147, 156, 159, 160, 162, 163, 164, 169, 170, 171, 175, 176, 177 Spinal Cord Diseases, 147, 169, 171, 175 Spiperone, 36, 175 Spirochete, 175, 177 Sporadic, 31, 160, 175 Staging, 171, 175 Steel, 133, 175 Stenosis, 33, 175, 176 Stereotypy, 51, 175 Steroid, 62, 136, 171, 175 Stimulant, 29, 123, 138, 148, 156, 175 Stimulus, 89, 143, 151, 152, 153, 170, 175, 178 Stomach, 121, 145, 148, 164, 173, 175 Strand, 166, 175 Streptococcal, 5, 8, 14, 18, 19, 21, 27, 76, 90, 175, 176 Streptococcal Infections, 5, 14, 76, 175 Streptococci, 18, 90, 176 Streptococcus, 18, 88, 175, 176 Streptokinase, 14, 176 Stress, 40, 88, 127, 131, 176 Striatum, 7, 11, 22, 23, 24, 51, 82, 83, 159, 176 Stricture, 175, 176 Stroke, 10, 31, 83, 88, 90, 102, 109, 176

Subacute, 3, 151, 176 Subarachnoid, 147, 152, 176 Subclinical, 13, 66, 151, 172, 176 Subcutaneous, 5, 88, 90, 176 Subiculum, 148, 176 Substance P, 142, 172, 176 Superantigens, 18, 176 Suprachiasmatic Nucleus, 8, 176 Sweat, 149, 176 Sympathetic Nervous System, 127, 176 Sympathomimetic, 123, 138, 139, 142, 156, 161, 176 Symptomatic, 11, 13, 110, 123, 177 Symptomatic treatment, 123, 177 Synapse, 24, 27, 30, 53, 122, 160, 167, 177, 180 Synapsis, 177 Synaptic, 15, 16, 154, 160, 173, 177 Synaptic Transmission, 15, 177 Syncope, 88, 177 Synergistic, 167, 177, 179 Syphilis, 3, 88, 131, 160, 177 Syringomyelia, 87, 177 Systemic, 8, 37, 98, 124, 125, 128, 138, 142, 150, 151, 152, 167, 169, 177 Systemic lupus erythematosus, 124, 125, 150, 177 T Telangiectasia, 28, 177 Telencephalon, 23, 127, 132, 167, 177 Temporal, 22, 24, 25, 83, 147, 148, 155, 177 Terminalis, 177, 178 Tetani, 177, 178 Tetanic, 177, 178 Tetanus, 49, 177, 178 Tetanus Toxin, 49, 178 Thalamic, 126, 178 Thalamic Diseases, 126, 178 Thalamus, 35, 48, 54, 129, 136, 153, 167, 178 Therapeutics, 70, 98, 178 Thermal, 139, 166, 178 Thiamine, 4, 178 Third Ventricle, 150, 178 Thoracic, 139, 178, 183 Threonine, 24, 173, 178 Threshold, 143, 149, 178 Thrombin, 144, 165, 168, 178 Thrombocytes, 165, 178 Thrombolytic, 176, 178 Thrombolytic Therapy, 176, 178 Thrombomodulin, 168, 178

196

Chorea

Thrombopenia, 125, 178 Thromboses, 125, 179 Thrombosis, 159, 168, 176, 179 Thymidine, 11, 129, 179 Thymoma, 55, 179 Thymus, 154, 179 Thyroid, 4, 149, 179, 181 Tic, 19, 70, 179 Tissue Culture, 15, 159, 179 Tomography, 10, 36, 135, 155, 179 Tone, 158, 162, 179 Tonic, 128, 179 Tonicity, 140, 148, 179 Torsion, 7, 54, 89, 151, 179 Torticollis, 89, 179 Toxic, iv, 20, 22, 130, 143, 150, 160, 179 Toxicity, 17, 20, 42, 140, 156, 179 Toxicokinetics, 179 Toxicology, 49, 104, 179 Toxin, 108, 141, 177, 178, 179 Trace element, 144, 161, 179 Tracer, 10, 180 Trachea, 153, 164, 179, 180 Traction, 133, 180 Transduction, 9, 173, 180 Transfection, 128, 180 Translation, 15, 19, 142, 180 Translational, 15, 180 Translocate, 13, 180 Translocation, 13, 133, 142, 180 Transmitter, 121, 126, 139, 152, 155, 160, 161, 180 Transplantation, 24, 57, 180 Trauma, 20, 88, 138, 159, 180 Tremor, 40, 89, 90, 108, 163, 180 Trigeminal, 87, 143, 180 Trinucleotide Repeat Expansion, 26, 43, 180 Trinucleotide Repeats, 180 Tropomyosin, 158, 180 Troponin, 158, 180 Tryptophan, 134, 173, 180 Tuberculosis, 154, 181 Tumor marker, 128, 181 Tyrosine, 139, 181 U Urease, 161, 181 Uremia, 170, 181 Urinary, 66, 149, 151, 158, 181 V Vaccination, 19, 181

Vaccines, 181, 182 Vagal, 87, 181 Vagina, 138, 181 Vagus Nerve, 89, 174, 181 Valproic Acid, 34, 38, 62, 181 Valves, 18, 147, 171, 181 Vascular, 66, 141, 147, 151, 157, 161, 165, 175, 181 Vascular Headaches, 157, 181 Vasodilator, 129, 139, 148, 181 Vector, 180, 181 Vein, 124, 161, 181 Venereal, 177, 181 Venous, 76, 125, 129, 132, 168, 181 Venous blood, 129, 132, 181 Venter, 182 Ventilation, 130, 182 Ventral, 23, 150, 161, 166, 182 Ventricle, 131, 148, 168, 178, 182 Ventricular, 147, 149, 182 Vertebral, 127, 175, 182 Vestibule, 4, 171, 182 Veterinary Medicine, 103, 182 Villi, 149, 182 Viral, 15, 67, 89, 141, 180, 182 Virulence, 179, 182 Virus, 35, 121, 132, 145, 152, 180, 182 Visceral, 127, 137, 146, 153, 181, 182 Visceral Afferents, 127, 146, 181, 182 Vitro, 10, 15, 182 Vivo, 10, 48, 182 Vocal cord, 90, 146, 164, 182 Voice Disorders, 90, 182 Volition, 152, 182 Voltage-gated, 16, 182 Vomeronasal Organ, 162, 182 W Wakefulness, 138, 183 White blood cell, 121, 124, 154, 165, 183 Windpipe, 164, 179, 183 Withdrawal, 49, 88, 138, 183 X Xenograft, 124, 183 Xerostomia, 4, 183 X-ray, 135, 144, 155, 158, 161, 169, 172, 183 Z

Zymogen, 168, 183