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1.7. References
KIwrwI>I. M .... C . _ M I. M.rOO F... aI" Th< """~""., or II"""""",. ,n the ""~ipid· .. ,it>ody.ynd"""" . N r..... J ~ ))20 99)·1. I99S. Ill'<" T ).1 . 11 .11 R O. I G; "'",~""""l>oI" ,ho:"9)' r.. ,...,.. ~_ic dila><. eI,.., 9S·lJ5-5IS. I9i9. ... ,. ,,,,,,,, 1 L 0.5 .... T M. Kumit, F I . .. oIo lie· pori • . i_ _ ]'IOjIO. io_"""m_i. i>
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GEN ERAL CARE
Sl.
SO . 35 . 86. 112. 811.
89.
90. 91.
M,o",,', "'''''or
MAl>l\>JI K A' poUo' a.... poo;lIci· pits "'" ;nv.. i.. ,... tnxo~ M. yo CliD !'ro< 71, • 72-1.1996. CcI..",..,blorOltbrili •. M«I Lelltr 41: 11·2. 1999. 11<111"" S M.Sonolbo".coot J. Z.I"m D. «<>Ie 0;.
'. dd<~ y poOpl< . 8 MJ J 10:. IJ·7 . '99~ . Qtioin< 10< "n,JIro cnmpo'. :.1'" ..... ne. 28: 110.
96.
Choice of b<.rodlaup>nn. M ... Uuo. XI: 26-1.
BiCO>S._)'<"fO. II,..G.« t>I, .... ~ .. Io.. _ p.obk .... 10 odullS . O",ical p", 'kc I" i~tli .. no.14. AHCPR publitalioa ' 0. ~2. A _ lor H«ltlo 1'01;" ond !l<.. "",~ . ""1>1;, 1-Ic.ltIo S..... i« .U.S Dc,..""",., oflt",-,,,, ond Itu",," Scr· "ice • • ~O
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M .... So!:o. !i,., M.W.,IoG : M..... ao.rJY' .. or.lliu
GENERAL CARE
1<,
,
IUS. 91. 98.
99. lOG, 10 1.
ONp IO'1")"C ~ ~ ' ri< di_ " _ ........ 21: 99· 100, 1986. MidaiOlam. M«I un•• 2.: 71... . 1986 , f1u ..... ".I. Mod t.ert.. J
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102,
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1.7. References
2.1.
Dementia
Definition: loas of in~llert ... al sbilit,.,. previl)tlJlly ellai ood (me,,,,,ry, judgement, abo itract Lhought , and other high~r co rneal fuoctiona) severe enough to illt.e rfere with 80<:;111 andlo r occupational fUnclionillg' . Memory deficit i. the cardinal feature. however, the DSM·rv definition reQuires impairment in a~ least Dna other domain (langue!:",. per«ptiM , visuO!Ipatia] function. calcu lation , judgement, abotrodion, problem.Bolving 8kjIl8). Affe.:ta 3· 11% of community-dwelling odull..! '" 61> YI" of age, with II grealer pruenoa among institutionalized reaidetlLs". Ri sk faeta ....: advanced age, family hialor)' ofdementia , II.nd npolipuprot<';in E-'I ,I·
.,.
IMlerium: AKAacut.econfuaional atalf. Di.Gtinctfrom dementia, however, patients with deD1Mtia lI.e at increased ri$~ ofdeveJopingdeJerium"', A prima')' dlSoroerofot1.entiOll thalaubsequently &iI'!'Cl.$ all ~theT aspect.; ofCOjfllition'. Often repre$ents lir..·threBt.enjog illness, e.g. hYl"'xia. SRp.oi •. W'emic enCi!phaJopa thy ralllO;«< poffe 64), eJectrolyta .. bnonnality. drug into"ica tion. MI. 50'l0 ofpatienta die within 2 yr8 of t hisdiagnosia. Unlike dementi., delerium b•• !H'ute Onllet, nlotor slgnJI (tremor. myoelonU5, "". t.erw5). slurred speech. alterEd conscioualles& (hyperalertlagita1.ed o,-lethBrgic. Dr fluctuatioo,), hallucinations may be florid . EEG - proDounced diffusti a\()wi,ng. B rain biopsy fo r d e m e nti a Clinical criteria are .. aually .ufficient for the diagnosis of moet dementiaa. Biopsy shouJd be res..rved for calle!; 01'11. chronIc progreulVe cerebral dillOrder wi t h an UnU!U,] cli nica l coW'se where all other pOOlSible diagnOlitie method$ have ~o eKh ...... ted lind h .. "e failed 1.0 provide adequate diagnostic certainty'. Biopay may d.iscl06e.CJD, low grade 9St.rocytoma, and AD .mOllI: olhen. The high incideme oCCJO among pat.ieoUl.elected for biopsy under the .... criteris ~es5italeflllppropri&te pn!C&utions (IIH Creu~reld'·Jokob dr_if. page 227 ). In .. repon of:;O brain bioplllH p&rformw t o _ progreo;aive neurodegenerative dllJease (If unclear etiology" the diagnC)l;tic yield ........ only 2{1% (6% WeN! only Hugguliv~ of. diagnosis. 66~ wert! abnonDal but nonspecific. B'l> wen! normal ). The yield wa5 lIighHt in thoae"With focal MRI obnonulities. Among \he 10 potienl.i with diagn05ticbiopsies, the biopsy rHult led \0 a meaningful therapeutic in te ...... c!llt;on in on ly 4. Rcoommend a t;on a, B~ M tbe above, the follo .... ing recommendation. are made for patienu; ..... ith an olllerwi5c uneJeplained neu rodeg.merati.-e dis-em;e: 1. thOlle ...·ith a focal abnonnality on MRJ, stereot.llrtic biopi;y :l. t h...... wltlloul foc",] abnormality (pouibly including SPECl'o. p£T ...,.n): b.ai.o bioplly /lhould only be perfonned within an invntigative protocol
2.2.
Headache
Headache (W A) may be broadly categorized as foUows: I. chronlc recurring headache. A, vasculsr typE! (mig.-a.ine)' ... ~ bdo'" B. muscle contraction (U!nrionl headachall 2. hudache due \0 pathology A. systemic p8thol~ B. intracl1lnial pathology; II wide variety nf atiologi811 including: 1. aubarathnoid hemorrhage, Il!J12~Q onlie!, severe. uaually with vomit-ing, apoplexy. focal deficits pos$ible iM'e poga 7ff2lilr diifel"l!lItial diag-
2.1_ Dementia
NEUROLOGY
I'M" of paroxYlmal HlAJ inctewaed intra","n ;.1 preMure linm a ny CII\lItI (tu mor, almmWlica l~
2
In, hydr1lOeph"I\I5, inna mmati on, Jli!IeudoluJ>>ur cereb ri .••) 3 4
irrita t ion!W Inflammlltion ofmeningel! m@ningiti. tumor: with Or without ele~aU!d ICP (no. JXlIt 4!l.S) C. lou! plIlhoiolD' oft~ eye, nuopha ryr\ll:, OT utracn.ni"J tiRuea (;"duding ~U"lt
<ell artenti.,.- pagt. 58)
o following head t ' II"llIa (~tcor>c:uU;Y" ayndrorne}: ~~ PfJ~ 682 E. f.lllowtllg ainlolllmy t".ynd rome 01 th. trephIned"): 1ft pagt 6J2 A ,,,!Yere new HlA. (It. chaos , in the plltI\!m oflllo"8.tarulinl,: Of' rKUrTenl HI" ( in~
eluding developing IInoclllt.l(l NN, Or ~n "lmormal neurologic enm) w.rran~ (.. r1MoT in· Yt$tiption with CTorMR I' ,
2.2.1.
Migraine
Migraine attacks ua uallY'ICCLlr in indiyicillaLt pr.dilpeud IA'l ,h"condition, lind mly
be activated by factorl alleh a.\ urill,lli,ht, Itrfla. diilt ch, npll. trauma, adminiNrillioI'I ofrttdiologic contrast
m~i.
(eJpedaUy 8JlgiOgtIlPhy) and vesodilalol'l ,
CLASSIFICATION See aJfrO indu under HtodQl;/u" e.lI. [or; crash pogr 782, pOIH,.. myeIOjfraUl headache ptJ~ 46".
migr~ine
(thundndap headachel
COMMON MIGRAINE
Ep;Booie HI" with:;.lN and photopl:lobil, w;thoutllu", or fleurologiedelkit, CLASSIC MIGRAIIII£ migrai~ ~Ive comp1t~ly
... aur. , May have HlA with occuional foelll neuro\orlC dt:firiuI ) in .. 24 hra . Over halfofthe Ir""delll neurologie dilturblln~ ~f'It vilUll\' and uluan,. _no!" poIIitiyt phenomena (lpArk photoplil, ,Ian., IXlmplu 1000metrie pIIuema, fortifiCAtion apectra) which mllY leaye negative phenOOltl\a (goloma. hemianopia , monocular or bin. orular yjauplloQ , .. ) in their wake. The HlCond IIIlXt malloon symptOma a,.. .amatoRn.. frOry involving the hand aod lower rac~ ..... fn!quently, defldu may (:QIUI\1t orapha&ia, .... mipa ....,is. or unil,,~ral elu"",;n ...... A slaw mardl·like progrnllion ofdeficit i. Cha rllllVlril'itk. Tho! risk of ~Iroke is probably inatlaMd ;" palientll wilh mign,,""'. Com mon
Ihlll
COMPLIC.'o TEO MIGAAIN£ Occuional IIll.8eU ofdaJ.4jcmigte;ne with minimal or no auociated HlA, and comple~ re&Olution ofneuroJorie deficit ..... 30 dap
M/OAAINE £OU/VAL£f'/T Nelll'Olork 'ymplomf INN. visualaLlrJ.. etc. ) without HlA (_phaJg.e migraine), Seen mc:.11y in childnn UsuIII,. dev&lopll into typical migraiae with I~ Au.. lIIay be ehorVlned by opening and _an_inl contenb of a 10 IIll!I ni(ed,pine capw]e'". HEMIPLEGIC MIGIM/Nf
lilA typiC*lly pr-'eII hemiplegia whkh may penlSt eve"
Il~r
HlA t«olves.
CWSTER HEADACHE
AKA h;'l.8min~ mllTlllIl.. Actually a neUl'OY8ICUIllt ""tnt. di$tincl from true mi. IUrne. RtcutTtnl unila~tll altacks ofseYeN! pa;". Ulually oculoCrontal or IICulolempGfli wilh occuional radiation Intothejaw, ulu_Uy r«Ilnin,on theslme , id"of the head. lpe:ila~ralautonomic: UVl~
(UW)juntliyal injectIon, nau.l
conlle~tion ,
th ino rrhea ,
lacfllUlltion, fad.1 nUllhin,) .... comtll(ln , PBrtial Hamer'aayndrome (ptoai. and ml .... \s) _ellme. o«u,.. Male;f~ale ratio U. ~ 5;1, 25~ of pat ienlll hay" a petllOllal or fami ly hi.tory of m igTIJM. H"adache. ch'''doI'riu~Uy h.... no prodrome. I.t SO-SO minu\.O$, and ~r one or mote limes daily u...al\y (or 4- 12 wHIu, often lit a .imila t time day. following which ther. is typieally, remillion fat an average of 12 month.",
or
NEUROLOGY
2.'2.. Headache
BASILAR ARTERY MIGRAINE
Essentially restr icted to adnlellc~nce. RecurT~nt episod811 lastln!: minutes to hours oftral"llnent neurologic ddidu. in distribution ofverwbrobMilar system , Defioil& iru:.iuu; v~rt.igo \ moat common ), ga;~ ataxia, ..,pual disturbance(acotD,Data, "'Ialoeml blindn_), dysarthria, fo1!owad by ... veu HlA and DC:C8IIjonaLly nausea a nd vomiting". Fami ly his_ tory of mrrrBln~ is present in 86%.
2.2.2.
Post LP (myelogram) HlA
AKA "poatapinai headach e" or "spinal helldath~·. MaY·IIIIG folio .... proc:edure. other than l P/myelogmm, such 811 dunil opt!ninc (..." pa/le 308). Can alro o.....,ur with 5pontane· O1.Ir intratrauial hypotension i!eep"'¥t 178).
CllDi ca1 rea tures Important distinct ive cho.ract.eris t lc: HII'. (Ittt.ITS .. hen patient is e~, and ia corn plelely or partially (but eignifitantly) reliev..:! .. hen recumbent. May be lll!wcisted .... ith nsusea. vomi ting. diuiness, or v;"'uaJ dioturbances. Tim e CO~: MO$t pD6t_LP headachea IPLPllA) hav"-,, delayed OM~1 2" ....8 hl"l after the LP, and although they may octU . ....eek~ poet-LP. mll&tal~o develop within 3 day". The duraliao ofPLPHA varies, with a mean of 4 dBY"''', IUId r eports of dumtion of mon t hs" IlIId even> 1 yeB. '". Pat b o pbY!lio logy Thought to be due \.0 continued CS F leakage IhrQugb the hole in the duro " . which redUCN the CS F ' cuahion" of the brain, 10 the uprigh t po.9i ~iM\. the pull of gravity on II ... bmi n produces t m clicmon lh~ blood veMelaQ nd any .trucluu s tetherinR the brlli n to t he pain-sensitive dura. CSf' may sonleti mu be.delllon~trabl~ in the tl1id.w:a.I.pacc.
Ep id e mi o lo gy ro llowiog LP Reported lnddenct: " ng" is2-40%(typically ~ 2~). bigheraftudiagnoatic LP than for epidural anuthuia". For variobles in LP tl1at impa~t upon the ri$k ofPLPHA, Sef' paMe.617
T REATMENT FOR tvA FOLLOWING LP
Ini(ial 1, 2. 3. 4_ 5. 6.
'cOIlservative" me:saurea include!'.at in bOO for at leat1l24 hI"!! hydm tion (PO or [V) analgeuCii for lilA ~il!:ht abdominal binder de,oxyrortiaone acetate 5 mg 1M q 8 brs" colTeine lodiwn benzoate 500 mg in 2 ~c IV Q B hr. up to:l d mllll (70% of potienlS had relief with 1 or 2 iqj.,dlo~" 7, high-doae .teroi,a, report ofsuccK8ln 8 case orinll1lUao.ial hypoWlls;on auoeis led witl1 "ponllln&OU8 Blit ventricles tapering down from a MArting d05l! "rd",,amethasone 2{j mgldayll 8. blood patch if refractory 1_ below)
E PIDURAL BLOOD PATCH
For refractory PQSt-lumb.o.r punctu r~ or poll-myelogram HlA. W-oru in one a pplica_ tion in over ~ of caul. mil)' ~ repea\.ed ;fine lTective" . Theoret.ital ri8q: inflKlioo, caude equi na com prcasion, failure \.0 relieve HII'.. Tec hn iq u e ACCl!uing epidurall1pace (one ofuvera! techniquu): prDC:etId as rouline LP. When ligame4Ls a re Itavened. and lleedJe tip iB neating apiDel cane!. style!; is renwved. Then. either plllC.l drop of st.erile u line in hub (hanging drop tE<:hnique) ud advance while wa\.chi.ng rOt Jl Ie be. d ......... n iM" n~e at epidural apate if entered, or gtonLly try it\je<:tinK a lr with small !l.Yrinlle(prefera b!y glSMjwhile advlltlclng, wben the epidural space Ii entered, rui.t aoca Ie ir\ie<'tion disa.pp8Q r~, but CSF cannot be aspi rat.ed . A venlpunoture site. i~ prepared a.eptlcally, 10 IIlI oftl1e pollent'. blood is .... ith· dl"lwn. After verifyiog CSf' c:annot be aspitaLed through the ~pin w l needle. the blood i. il\iected i.n\.O the epidurelapa<:e. Afte r 30 minuw supine, patient m~y ambul.te ad lib.
NEUROLOGY
Parkinsonism
2,3,
PArkinaonjsm may be primll ry (irliQpath ie paralytis .gita", (IPAI , cllllllical Pa rki n. \OOn', niUlle) or lW\:onrlaryLO olhu con dillon l. AU lUu lt from I Il!IaUVf; laM of the dOp.llmine mediated in h lbltioo of th .... necL\ C>f acetylch ollne.n Ihl! b. , a! ganIl:1i,.
I DIOPATHIC PAAALVSIS A01TANS
CI .. ¥icn l Patkin!.On'. rlilellae. AKA ! lIaking pa l.,..
Table 2-1 Cla sele triad 01
C lin ica l
Parktnlon'l dlHalll
Aff«tA: -l'*'ofA,mt n canl>apliO)'f'I". M.le:remaJ.
I':
- :"= , ~1'*
1
flItlO i. 3:2. Not clea rly l!:I'I"lronmentllJ!y o. , enedeflUy in. duted, bu\wu)' bo! Infl uenced by th~ f.etart. The donie triad i, shown in TIl"~ 2· 1. Other .igI.. may indude; pos tu ...1 innabill l)', micrographl. , mask-like raae.. CHltl;'On&i.~ or ..... n. J tlUffiin, ate"" l mll ",h", ' per.i lf; p,,) or feuinll!inr i.it ,
C lini uidly di~l ingui8hing IPA from .ceond "..,. padtin..,nitm (I« bfiout): MI.1 be diffi cu lt urly. IP" IIfInflr.Uy uhibia ""dl.l"l on... 1 ofbr&d,yk.inell. with trelnor lNot i. of'Wn lt8ymmetrica l, .nd initi a lly rH porKIl wen too Iev(ldopa. OIM. di_den! are .""",11.
ed with rapid progre..,OJ! of iymptGmll, ",bee the initia l rupome loCI .... odopIl n ~u;vo cal , or "'M!n U",rel. u.rly tnidli,ne 'Y!'lpWlII' (lluo1ia or hnpainrol!nl or ,ai t and blilel>Cf!, &phiocter di!tl.lrb&r>«! ... ) or 1M! pl'l!Hn ce of oUlu fe.tulU .ud'! .. earty demen ti., H n_ 1io01")' findinp. profol,llld orthOli talic h)1lOten!lion. OJ' .bnonnalitin: ofutraocular m",,~nta"' '' .
Pa tb opbys iol ogy Degeneration prim&rlly of pi~nted Ineuromelanin-Iaderl) d opaminel'(ic: netU'on. oflhe pIIn! eompatta of the lub&Ulntia niera,
hllimlrkoflPA. SECONDARY PARKINSONISM
The dift'~r tDt ;' 1 dl~OIiJ loeludell tht' following etiologiH or Htondary pa rkinMlnilln gr ParkinlOn-like «mditi01U{IOme. rmlTt'd to a. -ParlLlnlon plus"): 1- oli vopoDIcx:ereb~Jla. dege.nuel>Oo (OPC) 2. I lriato-ni{fal d~lI4! ration ISND): more .",!!ISi... Ihan parkinsooi.m 3. .-t.enoephaIiLie pIIrkin$oni,m: foliowed.n .. pidemk oft~PNoU lil ~tharg>ca (von £,;onomo di~lI) in tIw 1920'" VIctim, I." no kmll'~r livir\i_ Distinauiahing ruwrea: ClCulOC)'ric niail. In!mllr involv ... not only elItremlli ... butlllso trunk and he.d, uym llletriclll. DO Lt'...,. bodi" ... progreaah'f. IlIpr'lDuciear palsy (PS.W): iropa ired v@rticalgne (eu0600» 6. m"l tiple .,stem IItrophy {SilY-Orager .yndrurne1. '" tHlc>w 6. d"., induced: iDdudn: A. p~riplioB drop (alMdy females _m III~ I UlIOepllb ll!) I . anllptycbolict (AKA neu.roleptict); hal0pen601 (Haldol*) whi<:h work! by blocki", ponayn apuc dopamine receptors
2. phf,nclllillline Inliemeti~: pnxhlorperulne (Com pll1inl4)
3. me!odgpramide t~anll) ... . -rpinl B. MPTP (1-methy l·.... phenyl · I .2,3.6-t.eu.hydrupyri"in~); II commert:illily a ... iLable c:[email protected] illl.gaby-productofthe.ynlheai l or MPPP(I rnependinll "",Ioel tha t w . . .ynthfll.ed lind ae lr·injeo:tl'li by II NEUROLOGY
2 .3 Parlunaonilm
7. S.
9. 10. II.
12. 13. 14.
15.
graduate 6~udent". and later produced by illicit drug manufac~urers to be sold as 'synthetic heroin " and unwittingly injected by some IV drug abusers in northern Cal ifornia in 1983". MPTP was sul»equer.tly diacovered to be a potent neurotoxi n for dopaminergic neuronB. N! a rule , the reBponse to levodopa is dramatic, but short·lived C. there ia an aa yet unproven assertion that metbylenedioxymethamphet· amine (MDMAI AKA Ecstasy ( on the street), may ha"""n the onset ofP ...· kinsoni sm tox.ic: poisoning with carbon monoxide, manganese .. . iscbemic(lacunes in basal ganglia): produces so-called arteriosclerotic parkin· 80nism AKA vaso;u!ar parkinllonism: "lowe... halr pIIrkinllOnism (gait distur· banee predominates"). Also causes paeudobulbar deficits, emotional lability. Tremor is rDre posttraumatic: parkinsonian symptoms may ocCUr in chronic traumatic encepha· lopatby (dementia pugilistica, _ JXJlI~ 68."l). There Dre usuD l1yother features not nonnally present in IPA (e .g. cerebellar findings) nonnal pr~8ure hydrocephalus (NPH): urinary incontinen(e ... (& .... fKlH'l 199) neoplum in the region of the substantiD nigra Riley·Oay (familial dysautonomia) parkinson·dementia complex of Guam: dauic lPA + amyotroph.ic lateral l cleroais (ALS). Pathologically ha l festure. of parkinsonism and Al~heimer'1 disease but no Lewy bodies nor senile plaques Huntington's diMase (lID): whereas adulu typically show morea , wben HO manifesu in a young penon it may resemble IPA (8 pontaneous) intracranial hypotension may present with findings mimicking IPA\.-page178)
MULTIPLE S YSTEM ATROPHY (MSA) AKA Shy· Drager syndrome. Parkinsonism (indisti nguisha ble from IPA), PLUS id· iopa thic orthostatic hypotension, PLUS other , igna of autonomic ne.-voU$ system (ANS) dysfunction (ANS findings may precede parkinsonism and may include urinary aphincter disturbance and bypenenaitivity to noradrenaline or tyramine infusioWl). Degenera· tion ofpregangliQnic latenll>om neurons of thoracic 'pinal cord. NB: classic IPA may eventually produce orth(l8tatic hypot..-naion from inactivity or 8$ a result ofprogresaive autonomic failure. Unlike IPA, mO$t do not respond to dopa therapy. PROGRESSIVE SUPRANUCLEAR PALSY (P$NP)
AKA Steele·Richardson·01alGWski syndrome"". Triad: l. progressive s upranuclear ophthalmoplegia (chiefly ver tical Caze): paresis ofvol· untary vertieal eye movement, but atill moves to vertical doll·s eyes maneuver 2. i>*ludobulbar palsy (mask·like facies with marked dysarthria and dysphagia, hy· peractive jaw jerk, emotional incontinence usually mild) 3. axial dystonia (especially of neck and upper trunk) N!sociated finding" sutCQrtical dementia (inconstant), motor findings of pyramidal, extrapyram id al and cerebellar systems. Average age of onset: 60 yrt!. Males comprise 60'1>. Response to antl.parkinson drugs i, u8ually very short lived. Averagesu ..... ,val after diagnosis: 6:1 yrs . Differelltiating from Parkinson·s disease (IPA): Patienu with PSNP h8ve a pseudo-parkinsonism. They have mask facies, but do not walk bent forward (they walk ered), and they do not have a tremor. They tend to rail backwards. Course I. early: A. mDny fall., due to dysequilibrium + downgaze palsy (can't !lei! noor) B. eye findings may be normal initially, subsequently may develop difficulty looking down (t"Specially to command, l~ to following), calories have nor· mal tonic component but al»ent nystagmus (cortical component) C. alurred spe~h D. personality ch!lllges E. difficulty eating: due to pseudobulbar palsy + ;nabili~ to look down at food on plate
..
2.3. ParkinsoniBUI
NEUROLOGY
2.
I .~
It. eye. fIXed ~utTaUy too response to oxulocepbal ic. or otulovullbular1); OIl1.11 •• ImmotiU ty ill due Lo f. (Hltlll lobe laio n.
8
"""k atiffe ... in "",ten,lon l. etnK'OIllI)
SURG ICAL TREATMENT FOR P AAKINSON'S DISEASE BerGn Ih .. introdudion or t..dGp. in til- I. te 19&0'., .te' f'Otactic tblilemo!.Omy ... n widely used fGr Park ....... diteaae. Tb .. loaItlon ullimll.ely targelN for lel io",;ng"'u the .... nll"Ol.III,...1 o"deu •. Th .. p.-lu...... Q1'ked better for Nlifl.vinr the t~mor tbflll for the bradyllinUla. however It "'1\1 the lillie. lympLom tbllt we.. mOll;t.diubliog. Thi s ~ tedu ", eanoot be done bihllllrfl Uy ... ilhoul .igni licunl rislJ to 'PH\:h function The p.ocedun. f.1I oUt GfflYOr wh.n more effective drop ~lIme IIvIIU.b!e" See SW-I,",lll'WOlm,M 0( f>Qr1ti1lWll', di __ 0"' pap 365 !'or rurther lofonnation.
2.4.
Multiple sclerosis
A derllYlLlin,ti.Q( dlll,",_ (Iffecti", "nly while malto.r) IIfth. al1!brom . optIC neTVl!II, . nd s ploaJ mrd (espec:i.lI". oorti«llp!nal lUlU .nd the pOIIl@rior 'JOJumru). Ptod"Cftmultiple pl.que. of VII no ... ..,;n dilfu ... loxe~1\II in tl!ll CNS. eapec:illily in th" poi"riv~ntri.,. UID r white maUer. LHiolalniti,lIy eYOQ a", innIR'ma~ response with monoqteo and ]ympl»cyllc pl:nvucul., ""mnfi;. bllt with 'g~ fedle down to el ial SClil ri.
EPIDEMIOLOGY Utili] IIp.of onMt ; 10·59 yell,..., " 'i lh lb. gTellli'!lt peAk bet.... een agell20- ~O y,",,~. Ma le 10 femal~ I'Itio: 1.J§,1. Prevalence v.nOM __.th latitude, e.nd is < 1 peer 100,000 rtf:1I r iheequator, lind if - 3Q80 per 100,000 la Ihe north*m U.s. and Canada.
CUNICAL Cauj;eS exac:erb.a tioRa.n d ~rnis T.ble 2-2 Cl1nk:aL MS l ions in variou$locationJ In the eNS (diuemioati~ aDd liIDfl ). Com· mon Iyrnploms! visual utu rbantu (diplopill, bl"rring, fielcl :U1ll1I• .,.,1.0. mil. ,pastic par.pa res;lI. and bJ,ddp... distllrbllntK. N01'llf:11t1l1turfl for tbe. tima 1>'1,,'$11 o( MS if. . h ...... n i", 1'bbh: 1'. 2"1. Re1~P8iDg-rem'lting MS i, the IOOiit com· mon pattem at OMet,.nd hal 1M beat I'f!Sponlie to therapy, b,,~> 5~ of cues ev"ntually become semn~al')' pt'
NEUROI.OCY
2.4 MultIple f('!ll'OIil
..
Signs 80d s ymptoms Visual di s turbances: Disturbances of visual acuity may be csuaed by optieor retrobulbar neuritis ..... hich ia the pTflSenting symptom ofMS in 15%oftllSfls, and ..... hich OCI'UTS at some time;n 50% orMS patients. The percentage ofpatientll ..... ith an attack of optic neu· ritis and no prior attack that will g
Table 2-3 Diagnostic criteria for MS
,
GU SymptOIDll: Urinary frequency, urgency, and incontinence arecommon. lmpotence in males and reduced libido in either sex isoften_n.
,.
D IAGNOSTIC CRITERIA
No single clinical feature ordiagnO$tic test i~ ad· equate for the a<:euratalitiell charae· l4lristie of MS. The presenoe of these MRJ sbnormalitiell increases the risk ofdeveJ· oping MS in 1-3 yellNil (with greal4lr prognostic signifi· cance than CSF-OCB). The mOTe MRJ lesiOl\lj, the higher the risk"'. Criteria for the diagnosis ofMS" follow$. The l4lnns "dinieally ~ definite MS· and -clinically probable MS· are no longer recommended. Preferred terms: MS. possible MS (at ri5k
"
, A.
,. 9 T2Wl lesions on MRI or
C. D.
lId
Q<
CSF tesIs ore dane and ar.
""9"'ive. aPlOl\l e. lreme cautIOn In dlagr<>sing loiS I"I'IU$I ","I cril
d'""-ninaUonTn
~on
t.!FlI
T.,.
U
must mM11hil ttIIeria in r.bleZ·5
aln)<mei Ykuol .....ol
in loiS (IeIay wilh
~I·
p r e _ w..... lo1m)
for MS but diagnosis is equivocal). Or oot MS"'.
2.4. Multiple 5tlernsis
NEUROLOGY
Diagnostic criteria ate shown in Tobie 2·3".
Ddi"itions" I . attack (exacerbation. relapse): neurologic disturbance lasting> 2:4 hrs" typical of MS when clinicopathological studies de· termine that the cause ill demyelinating or inflammatory lesiaM 2. typical of MS: sigruo &. syrnpwms (SIS) known to occur fn-quently in MS. Thus excludes gray matter lesions, peripheral nervous 6ystem lesions, and non·specific complaints such as HlA, depression, con· vulsive .;eizurea, etc. 3. separate leaions: SIS cannot be explained on bas;' of singI.. lesion (optic neuritis of both eyes simultaneously or within 15 days represents Bingle lesion)
Table 2-4 Brain MAl criteria lor MS
3 althe loIlowIlI9 4 cr~erit· I. 1 gadollnU:!l-enhancing Iasion or, d no gado5nlulI1 enhancing lesions, theo 9 T2WI!esQns 2. ,. 1 intratenloriallesion 3. ,. 1 jultlac:onicalleslon (i.e. lnYoMng subcor1iclll u Jtlers) 4. ,. 3 pefiol .... \rio:uIaf lesions 1 ~ CQf(j lesiDn car!be- subs ~lu'ed 10, 1 bra i n _
Table 2-5 MAl criteria for d ieaemlnaUon ollesillns In time
MRI MRI i. the prefeJTed imaging study in evaluating MSI> and can demOlI9tl'at& dissemination of Ie· sions in time and space. Recommended" brain MRI criteria for diagu08ing MS are . hown ill Table 2·4"'n . Le5ions are normally" 3 rum diameter". M~R[ shows multiple white matter abnormaliti8$ in 80% of patients with MS (compared to 2:9% for CT)'"· ... LesionJI are high signal 011 T2WI, and acute lesions tend to enhance with gadolil!.ium mOre than old Iesion a do. Periventricular lesions may blend in with the tignal from CSF in the ventricles on T2WI. these leaioll8 are shown to better advantege On proton density images as higher intensity than CSF. Spinal ~ming hOI cri1ical. buI 3 mont/I$ b cord lesions normally show little or no .welling, should be:it 3 mm but < 2: vertebral S
2:. 4. Multiple selerosis
"
dictive \l81u8 of tile ebtente oflgG in a potient w;tllauspcctcd MS lias not heeD satisfactorilyelucidated. Recommended ~rilerie llavlI been publi, lIed", m~t of wlli(1I pertain to apecifiea of laboratory analy.it. pertinent cliniClI1 e~wrpta ... e allown in Toblf 2·6.
2.5.
Amyotrophic lateral sclerosis
· I
t Key fealur ..
a mi~ed uppe r an d lower motor neuroo di_an (UMN - mild lpaltici ty in LEI; lMN - atroplly And f.w:icullllion i Ln Ur..) • rw co~itive, .. ruory, nOr autonomic d funct.ion • e/lu.ed by degllneration of ne...,...,n, in t),le tervic,,1 .pi ne end lD&duU. (bulb) In llle U.s. emyotropllic iatflral sclcowi. fALS) i. AKA Lou GeIuig'1 dilellM.
EPIDEMIOLOGY'"
Preva lence: 4·61100,000. Incideote: 0 .8 · 1.21100.000. Familial in 8·10'l00fca_. Familial ca$U Ulually folio ... autDtomal dominant inlier. ita nte. but oecasionally demonstrate a r_ive patl.olm. Onact usually aner 40 years of age. PATHOLOGY Degeneration of anterior 110m alpba·lDotonlluron. (io tho! lpi nal cord IIlli In brain ,tern motor nucllli) and cortioospina! tl1lCI& (lle/"lfle AKA motor neuron diseue). Thi, produces a mixed upper and lower motor DeuTOn discue, ... itll a great deal orv,ri lbi lity de. p"nding on wllicll p""'om;nate, at any given t;n:>fI. The etiology of ALS illtill not known with certainty. CLINICAL
Involvement il ofvoluotary mu..:I.., eparing tile volunury ~ 1Il,,"lee and urinary ephincter. Classically, presents initially with weaueIII and auoplly of the hands (lower motor ncuron) witll l PIIllicity and lIyperunexia of 1M lower extremiti"" (upper motor neuron). However, LEI may be hypon!fleltic if the Io"'e r motor neuron delicita predominate. Dyu.rthria and dysphagia a ... cau..sed by a combination of upp"r and lower motor neuron pathology. Too,gue atroplly and fasciculations may occur. Although eornitive deficits an! generally considered to be abosent m AlS. in aetual· ity 1·2% ofca_ a ... auoc:ialed with dementia. and cognitive changes may occasioo.ally predllte tha ul ual fea t ura of ALS" . DIffERENTIAL DIAGNOSIS
It is important for the neurosu'¥e
EMG: Not abaolutely I1«t'lS&ry to make diagnoei. in moIIt ca.&eI. Pibrillationa and posi· tive I barp .... Vel are fouod in adva nced _ (may be abRnt early, "pedally ;fu pper motor neuron pathology predominatel). LMN findin p in tho! LE in theabRnceon .. mbeor Ipine di.."c. Or fibrillation potenti,l, in t he tonaue are luggestive of ALS. LP (CSF): May have . lightly elevated prol.ein. TRE~ TMENT
Onpng trial. with riluzole (Rilute14), whicll inhibita tile pruyn.ptle ........ of glutamate, indiClitol that dOH' or50-200 mgld inCrf!8MII trach_tortly·free survival al9 &; 12 months, but tile improvemeot it roortlJ>OOkott Of" rnay be non-eJ.ittent by - 18 month," ". At tile tima of thi. writin" the d"., it aVlilabLe onLy for prema:rkl!tln. trials. and cannot be procured comTOCTcially. Much of care i. di~ted tow.rd, minimiain, diubility: I. ..pintion may be treat$;\ witll 2.5. Amyotrophic iat.e!'al lCleroai,
NEUROLOGY
A. Iroch"""IllIllY B. gtostroslllllll' tube III allow cootinued fceding C. voce] eord ill,jedion wit h "(!f1on 2, a paslidty thaL occun wh(!11 upper Illntor neuron defrcit.s predomin~~ may ~ trea~d (usually With ! hort.· UO'ed response ) with: A. bsdafen.: alM! may re~eve t.becommonly oc:currlng cramp. (,el': fX18t 368)
B
dia~epam
PROGNOSIS ~IOlIt patients die within 6 years oranset (median Bu,.".i"al, 3-4 yrsl. "h ... e wIth prominent or(lpharynlfulsymptol1t$ may haO'e 8 . harter lif....span usually due ta compJi. ClI.tio"" q{ IIspinl.lion .
2.6.
Guillain-Barre syndrome
t Key features •
acule onselofpenphet a! neuropathy with pf'Oll"l!Sl ive .... uscle weakne.u (more BeVe~ Drol{imal!~ with arefleria, Teaches mllll'lmum o"e r 3 dllYI t.o 3 waeu cranIal neuropal : 8 180 conunotl, may include fadal diplegia, ophthalrnopltlf;ia little ar nose.uory invo!veml'n t (paru thesiu lire not uncommon) onset often 3 dayo.·6 weeki fo UowI,IlB" "ira! URI. Immunillltion, ar s urgery p&thol~: foeal Rgnlental dem!eli nlltion with endoneuria1 monocyl.ic infll lrat.l! elevated CSF protein wil.baul p eocytosia (albuminocytalogie d,s6Ociation)
AKA BCUU! id iopal.blc pol)'TlIdiculoneuritiB. "he IIlQIlt common acquired demyeli nat.ing neull>\>athy. lncidenoe is _ VIOO,OOO."he lifetime risk forar.y one indlvidulIl gettin!! Guillain·Barri &yndro"" (GBS) is _ VI,GOO. Mild cases of CBS m8y pre&ent only with alllJ
DIAGNOSTIC CRITERIA'" I , fealuree requiNd for diagnosi s: A. progT"'I'Iive motor weaM OlI!s of mor.. \hlln I limb (from minimal weaJrnes>o ,,-ataxia W para]yai~ , lJIIIy include bulba r or facial or EOM palsy ). Unl ike mO&t neurO;lllthieli. proximal muule3 .re affected more then dista l B. arene";. (usually univenal. bul distal Brene~ia WIth d .. finiLe hyporenexl a of blCf!p5l1nd knee je rks suffices if other fellLUrei consisLent ) I!. featun!S strongly 8upporti~e o[diagnosis: A. cHnicol featu res (in order ofi n,l'on.ance) t. progT"e,jon: mowrwe3kness peaks aL2 wksm 60'll>, by S wI\! in 80%, and by 4 wu in ,,9Q
>"
NEURO/..OGY
2.6. Guillain.Bam; syndfOlD8
"
f.
eNS involvl!ment (controversial): e., . ataxia. dy!l8.lhria, Babin .k.i aigna B. CSF !indings: a lb" m ,nocytolog"i<: diSllOCllltion (elevated CSF protein witholll pleocytosi. ) 1. protein: elev'l.o!d I wk or aymptomt, > 55 O'Igidl 2. cell. : 10 or fewer mononuclear le ukocytHIml
ant.
3.
".nanta
no CSF prol.ei n rin 1·10 ... ka afW r oout (rare) b. 11-50 mooocytulmi c, elec t rodiagnolti~.: 8~ heve NeV Ilowing orblock atlOmetime (may take several weeu in lome). NCV \auaUy < 6O'lt of no ....
•.
l,Ilal, but not in all nerve-
3. fealu," cu tin, doubt on di egno. i.: A. marked. peraiat.t!nt, lI)'lllrMtry of_aknna B. pertiaUlnt bowel or bladder dy.funttion C. > 150 monocytesltDi CSF D. PMNa in CSF E. _hBrp sensory level 4. features that rule out diagnoei l (findinga that . uggeat 1M! preHnoe orone ortha conditionll in the dj([ert0tjl l d jlR'm!Ja. e., . ' " Myck>pq.llly. pap 902): A. current huacarboo use: volatile IiOlvenw In·hu.ne. methyln·butyl ketone), glue anilftng D. .cut.. In te.rmita .. n l porphyria (AlP): a diliOrder of porphyrin metabolism. CS t' protein is not elevated 10 AlP. Recu rrent painfUl.bdomln. 1 eri. &ei ...... eommon . Ch~k urine delta ·aminolevulinic scid or porphobilinogen C. r~ent diphtheritic infection: diphtheritic polyneuropathy bu a lonCCr la· \.ency Dnd •• Iower cresa!odo of . ymptomt O. lead neuropathy: UE weakneu with wrin drop. May be uymmelrical E. purely sensory syndrome f '. poliomyelitis: u,ually uymmet.rn:, hat meningeal irritation C. hypophosphatemia (may OCCUr in ch ronic IV hyperalimentation) H. botuJi~m: diffi culttodis tioi'Jish clinically from CBS. Norm. l NCV . od . f• • cilitating .-.sponse to repetitive nerve stimulation on electrodiagnoaliCl I. toxic neuropathy (e.g. from nitrofU."ntoin, dapsone, thallium or arsenic) J . tick paralysis: may ca. ...e an ucending motor neuropathy without sensory impainneoL Careful eumination oft.he scalp for tickC.) K. long time coune: mey indicate chronic im ... Wlc d .... yeli n . tmg polyra· d icu lon .. uropatby (Cl OP) AKA chronic relapsing CBS, chronic rela psing polyneuritis" . Similar w C BS. however symptoms must be present> 2 mOl. CIDP produces progrellllive, symroetric:al, proximal &. diua l weaknes.s, depre.. ion of muscle atretch noflex .... and ,·.riable soellSOry laq. Cranial ne""es are usually spared (faci al muades may be involved). Balance diffi· cullin are common. NHd for respiraWry IUpport i. rare. I'l!ak incidence: age 40-60 )'TI. ElectrodiagnOltjCl lllId nerve biopsy findings an!' indicative of dem~lination. CS t' findings .r. aimilar w CBS (_ obow). Most respond to immun""uppnuive therapy (npeciaUy predniliOlone &: plasmaphereaia) but relapaea arecommDn. Refractorycuea may be Ina~ with IV gamma. globulin , cyd"" porin ." ", tolal body lymphoid irradiation or inu.rferon-<>." The MiIle, · t ·ilher v.nant of CBS indoo... ataxia. an!nexia and ophthalmoplegia. TREATM£ftIT' LoununoclobuliNi m.ybe helpfUl. lnKIWc.....,ea rlyplaamaph ..... is haa\.eM!.he recovery and reduaoa the ....id""l defici L Its role in mild c:aaea Is l,In«rtain. S\.erOids . re not. helpful". Mecba\1.ical venlilationlJ.>d meuu,"" to preven t aa piration , ... used .. a ppropriate , l n e. 1IQ of raci. 1 dipleril , the eyn muSl be protected from eXl""'ure keratitis.
CNTCOME Recovery may not be complete !'or H veMl I montlu:. 35'" of untre,led patienla have .... id""l wealtn .... and atrophy. Reairreneeor CBS .lUr achievina: maximal recovery DCronl in . 2...
2.6. Cuillain·a. ... . yndrome
NEUROLOGY
2.7.
Myelitis
AKA acute transverse myelitis (ATM). The terminology is confusing: myelitis over· laps with "myelopathy". Both are pathologic conditions of the spinal eord. Myelitis indicot
Er/Ol..OGY Many so-called "causes" remain uoproven. ImmWlologic response against the e NS (most likely via cell mediated corolX'Dent ) il the probab le COmmon mechanism. An.iroaJ model : experimental allergic encephalomyeliti s (requ iN!8 myeli n basic protein areNS. not peripheral). Ger"~rally
1.
accepted etiologies include: infectious and post-infectious A , primary infectious myelitis I. viral: poliomyelitis, myelitis with viral encephalomyelitis, herpes ~OIIter • • abie!<
2. bacterial: including tuberculoma of spinal rord 3. !pi.ro<:hetal: AKA ~yphHitit myelitia. Causes syphilitic endarteritia 4. fungal (aspergillosis. blastomytosis, tryptococcosis) 5. parasitic (Echinococcus, cystiterl;Ollis, paragonimiasis. schistQf!omia· sis)
2.
3.
4. 5.
6.
7. 8. 9.
B. post-infectious: including posl-
~". connective tissue B. mixed disesse I~", .
_ .ted . .
WIth .. u ~ti.k "' .1 be ""Or'
properly ro th. ,
u... o>y.M;.
ilb. my.lopothy _
CLINICA L
A: 34
~nIj ";'~
ATM'"
W/lf$ B: 52 ~nts .,ijh OCU"' OI subaCUlfI tran ... _myeli ~s""
PRESENTATION
34 patients with ATM ": age of On· set ra nged 15-55 yrs, with 66'l1>occurTing in 3rd 8nd 4th d"",ade. 12 patienUl (35%) had s
Nt'UROLOGY
2.7. Myelitis
"
"iral ·like prodrome. Pruent;ng symptoms are shown in Tabk 2·1, with otherpresenting . ymptoma of unspecified frequency including"': fev .. r and rash . Pr elHlDling leve l The level . at pretentation in 62 pationu with ATM aro shown in Table 2·8". The thoradc le"el is the molt common ten · sory le~ l . ATM i. rarely the pre.enting.ymptom of MS I_ 306'11 of patiMtI with AT M de"elop MS).
Table 2-8 Level of
MnSOl'Y deficit
PROGRESSION Pro8l'usion i. usually rapid, with 66% reaching maximal deficit by 24 hn, however the interval between fint . ymptom lind mlllUmal deficit "aries from 2 hn·14 days-. Finding. It th e time of maximal deficit a re .hown in rabk 2·9.
EVALUATION
MY.florram CT &..M.SI: nO characteri.· tic finding. One pape r reparts 2 patients with fusiform cord enlargement". High ...... olution MRI with thin CUtl may be able to demonstrate al'8S ofinvol~ment within the cord . Patient.hould hIve imagi ng to IUO compressive lesion. C5.E: nonna l d ur ing acute phase in 38'11 ofLPs. &roaioder 162%) had elevated protein (usually:> 40 mg%) or ploocytOS i$ (Iym. phocyte$. PMNs, or both ) or both.
Tabl.2·9 Symptom' " time 01 maJlk mald.lell (62 patieotl..-i l h ATM"')
,
EVALUATION SCHEME
10 a patient developing acute myelopa· thy/paraplegia. espc(:ia lly when ATM is considered likely. the fint uslof choice is an emergency MRL Iffl()t readily available, a royelogTam (with CT to foI!ow ) direc:ted at the region of illesensory If!>'e l is perfonned ICSF may be sent in this ci rcumstan« once bloek is ruled Dull.
T REATME NT
Suggested efficacy ofhigh..:lose s teroid treatmen t in 1 patient .... ith ATMG (methyl· prednisolone 250 mg IV q 6 h,.. X 24 h ,.. . 125 Ing IV q 6 h,.. .. 24 h,.., 125 mg rv q 12 h1'$ I. 48 hrs. then 30 mg PO q 6 hno. Ulperilld gradually. Rc>&imen sllould probably be individ· ua liud baaed On ..... ponsel.
P ROGNOSIS
In a $eries of34 ATM palien U willi" 5 yrs follow·up (FlU)"": 9 pal iellUl 126%' had good rltCOYery (amb",1ale well mild urinary symp&o ....., minimal $eraory and UMN l ign l ): 9 (26'110) lIad fai r recov ....y (functional g. il with $Orne degM!! of $p8sticily. urioary u.rgency , obvioua .. ntory '[1"5, par' pereaill; 11 (aN) poor (paraplecic. a btent sphiDcter fIOIItro\); 5 (15%) died wilhi n 4 ..- or illn ...... 18 palientl (62':1. of survivorsl becama am· bulatory (in th_ cues,.1l c:ould walk with support by 3-6 ..-). In a Hri " 01'59 pllienU-{FIU period unl pl!('ified 1: 22 (37'11) ....d ,ood recovery; 14 (24") poor; 3 died in K U\.a stage (ru pinotoTy inl ufficiency in 2, Hpli5 in U. R«overy oc· curred between 4 weeki and 3 _ after on$et IfI() improvement oc:curred .fter 3 rnos).
2.8.
Neurosarcoidosis
SI.rcoidosi, i • • gran",lon,atou. di _ that il ulu,Uy I y, terni.:, and may indude the CNS (_ lied neuroaan;:oidotl ia). Only 39& or cue. have CNS fiodinp without IY.' temic: manifut.alion. ... The ('8uHorthe diM_ i, ",nknow n. An infeo:tiou l Igeol il pot. NEUROLOGY
.ible. Organ~C(jmmon ly in~(Ilved indude \ulllP. ~k1n , lymph nole.. bones, eyn, mUliClu. IlIld parolid glands'".
P ATHOLOGV
CNS lall:oid().9ia primanly InV()lvl!Ii the lep~omeningea, however parenchyma l invpaion often occu.rtI. Adhesive a rachnoiditia wilb nooule form.t.iOD may al-o oco.:u r llloduJ~ have" predil~iOll rOT the pc., nerior £(658). Oiffuse. meningitis ~r roellmgoeneephalitis may occur, and may be 1II011! pronounced al the balll.! oft bl! brain {bR8IIl meningitia l Bnd in the aubependymal region of the third ventricle (including the hypotbalamwo ). Cons tan t min"Oll~pic featW"1!5 of neurO$&TCoidNi. in clude n~a;,eati ng JlTllllulomilS with Iymph
E PIOEMIOLOGV
Ineide ",:., ofs.ar(Jlidosi6 Is - 3·50 "'IsesllOO,OOO popu lation; neu rOlla ra)idotlb O«UTI in _ 5% Or(;llM1l (reporud ran Ke: 1_27'1» . In one • .,ries, the median 1ge monset of neuro. l"(ie symptoms was 44 ~arl.
CLI NICAL FIN01 NGS
Clinlcallindings incl ude m ultiple cranial lIe .... e pal&iea, pl!flphe ral neuropathy, and myopathy". Ottasional1y the le8ionl roIlY prOOuce mauelfe-c:l1'. IUld byd rocephalW! roIlY res ult from adhe,,;vB basal ara~hnoidili$. Patiant>lUlay have 10.... grade r.. ve r. lntr&CrBoi. al hypertension i~ common and UlAy be dangerou s. HypolhalaJDie invol vero!';n t may produce dillorder~ or AD" fdiabetl>5 tru.ipidU6", disordered thirst).
LABORATOR Y
CBC: m ild
leuk~i.
IltId ",,,lnoph.llla may o<:cu r.
Se r u.D;l ang"iotenai n·co nvertiPi eo~ym" fACE): abnormally elevlto!d in 83% or patienu with active pulmo",,!), aar<:oid08is, but io only II % with in8ctivD diJlellse"'_F~llle
posi tive ral.e: 2-S'IIio: ma) ..180 be eleval.ed in primary biliary cir:h08;s. CSF : similar w any 8ubacuto! menlogitis: elevated p,et;SUN!, mild pleocyt.o8"ia ( 10200 oe:llslmllll ) IIIO$tJr.lymphoq'tu, elevated protein Cup to 2.0!lO mgfdl), mild Il.ypoglyc· orrha~hia ( 15·40 mgldl), CSF ACE is eleva ted in . Or CaI" with n eU'0$8rcnidoai. (nonnal in patienu .... ith lIa",oidOllis not involving th6 C NS)"' . No organislllS are /"IIWV· e red on clll t uTto, gram 'tain.
5-5"
DIAGNOSIS
Oifferentiati.nij ¥NnulolllatoUll8ngiitil (GA )
Table 2-10 Oltfe. enllal diagno-
froUl rlellTosarroido&is that invo]"1!!1 only the CNS ClIn be dane 011 histologic &riteril: the in n ommatory rail¢> lion in l,rt"Oid08i.t! il nat limited to Wle region imroed.i·
sis 01neurosareoldosla
au.ly ~uN"Ound;n G bl~ V_18 "" It;8 in CA, .... he ro ""len6;v8 disruption Qrrne vueel wall mayo<:cur. Making the diap"";5 is relatively BII$y when systemic involvement occurs: chara cteristic ti nd",~ on CXR. bioPIY of ak.iu Or live. n<>dulei . U\ LI.iIele biopey, leTVUI ACE a""y . holato!d neur~lra)idosiI Ul'y be Ulore diffi cult to diagorn.e, alld IOsy requi re hioP")' ($o!"t' bd(!w ).
BIOPS.,. In UOCfrt8iO~aH', biopsy mQy beindltated. Wheneverpo$aible, MRl a hou ld be used to localize a SUpl"1ltentorial region ofinV(llvernen~. and biopsy should include alileye" or IJIf!ninges .lJld QBrebro l cortex. Cul tlLr1!S and ~t/lin.s for fungus Ind acid·fMt bacteria ('1'8 ) 8hould be performed in sddiUon to m icrwcopit exalUinMion.
NEUROLOGY
2.8, NeUrMOrcoidoo;$
TREATMENT
AntibioliCll hB~t not been proven!.O ~ofbentfit. Steroid. ore bc!n!':fidal for 'YI!.emie u WtU •• nellrolo,ic involvemen t. Thtr. py with cycIOlpori". m.y allow a redoclion in lteroiddos"e in r.fn ctoryuIQ". Otbtr I..., well ~~IId.ied trutman tll : methotl'i! "~' eytaun. CSF shunting Ui indk,~ if hydroceph,III,dtvelopa,
PROO NOSIS UIu.Jly I bftlign disease. Periphuflllllld CtlUlilll nervli palsl" l"itWVer,lowly.
Vasculitis and vasculopathy
2.9.
Th. vlkUlitides ora . IJ"lIp of diaordtrl cho ... ~~riud by inflammation and MCrolia of blood v_la. VaKul;ti. ma, be prilllllt)' or IeQ)OOlry. Those thOL n'ay affect lhe CNS Ire lilted in Table 2· 11 , all ofthel6CflUJe WIUt 'Khemla (even ,,/\ar the inflarnma tion it! quitlCl!nt) thlt mlY raup iJI elT'eo:t from nauroprllXi, tQ infa rction
Ta ble 2-11 Yueulillde. lhal
!(£Y:O."""""""'''' ....._
the eNS'"
: •• _''''''''''''''': >-t.'''''''''''''''ICI1 . onIr~_~
SAI-I.~~
_HCIIon"..
~I
Iot_tDIlia
J>AN"
IfIOUI>"'_~......,-...ybt~
2.9.1 .
Giant cell arteritis (GCA)
f
TKey fuw ....
formetll D~" ~fured ta al l.emporal arteritis d ' romc vallCUht it!oflac]{e and ~um celibtr v_la, primlri l, LDvolvlnSeTl' nill b",ncl;>ea of Ole Imnft trisioS from the .0000e...::h age,. 50 yean: atrKU WOme
EPIOEIdIOl OO 'r
Stan ,lm Ollt e.:dU&i~1y in CauCII.;allt:> 60 '"' &Ie (mean'~ of o....,t it! 701. Ind· denc:.; 17.8 ~r 100,000 paople" M1yearaold" (r.np:OA9·231 Pr .... a1enc.: _ 223 (aulOp., incidence may be much hiKrlIl"rl" MoreCODlOMlll in n-orthtm litiludea Ind lmoll, 2.9 VpeulitiJ and visculopalhy
NEUROI.OGr
individuals ofScandintwiaa descent". Female:male ratio is _ 2:1 (reported raage: 1.05· 7.4:1). 50% ofCCA pat ients 11180 hllve polymyalgia rhellmatica (PMR) (."" PQHe 61).
P ATHOLOGY
Discontinuous (so..clliled "skip lesions") inJlammlitory reaction oflymphocytell. pl asma cell s, macro phages, :t giant cells u fabsent , intimal proliferation may he prominent); predominantly in n,edia ofirlVolved arterie~. Aruries plllferentially involved include the ophthalmic and posterior ciliary branches and the entilll distribution of the eKternal ca · rotid system (ofwhith the STA i8 a terroinal branch). Other arter ies in the body mal' be involved (reported invot-'ement ofabdominal aorta. femoral , brachial and mesenteric arteries a .... rarely symptomatic). Unlike PAN. CCA generally s pares the renal arteries.
C LINICAL
Various combinations of Table 2· 12 Signs and aymploms 01 GCA". T7 symptoms of giant cell arteritis are FreqU8fl1 listed in Tabl~ 2· 12. Onset is urual· j> SO'4 01 ca_) jll).5(l'!{,ol c_) « 1,"- of cases) Iy insidious. although OCCWlliOflaliy -.i$Ua1 sympIQm$ it may be abrupt"". ' ~'-~~ ~alMIlly ~11o$S exlremi!y daudicalion Details afso me findings \Dng~ claudica1ion ,-~ !8'/e( (law gllOe) 1. HlA: the most common pre· prounalmyalgi3$ earpai:l senting symptom. May be jaw claudicalion nonspeciIicor located in One I ~a:ial pa;., or both temporal areaa, scalp lenderlleSS angina forehead . or occiput. Mal' be superficial or burning with parolC)'smBI hmcin ating pain 2. symplQllul relating to ECA blood supply (Itrongly sugge~tive orCCA. bu t not pathognomonic"):jaw claudication, tongue. or pharyngul muscles 3. ophthalmologic sym ptoms: due to arteritis and oodusion ofbranch.es of ophthalm· ic artery Or posterior ciliary arteries A. 6YI"PtomS inclllde: amaurosis rugax (pre<:edes permanent visual loss in 44%), bUnMa$!;. visual field cUtl. diplopia. plOt;'. o<:ullir pain. oomelll edema. chemosis B. blindneu: incidence is - 7%. and once itoccutll, reeovery of'ight is unlikely 4. systemic aymptoms A. nonspecifk constitutional symptom!: fever (may present as FUO in 15% of cases). anorexia. weight 1088, fatigue, walaise 8 . 30'l0 have neurologic manifestations. 14% are neu..... pathies including mononeuropathies and peripheral polyneu ropathies of the annS Or I~ C. musculoskeletal symptoms 1. PMR is the most common (o<:curs in 40'l0 ofpatienta): I« page 61 2 . periphersl arthriti s. swelling &. piltiog edelIlli of bands &. feet in 25% 3. arm wllldicatioo from ,tellOSi. of subclavian and 8lIillary arteries D_ thoracic aortic aneurysms: 17 times as likely in GCA. Annual CXRs are adequate for screening 5. lel3tpo
"".....,
"'" '"""'"
.-. ''''''
&""
NEUROLOGY
2.9. Vasculitis and vaaculopathy
"
E VALUATION
Laborlltory . tudi e. I. ESR > 40 mrnlhr (usuIlIy > 50) II)' Wnwr,,"," method llf > 80 IIIm1hr with .bcJo,·e CtUII<:8J ')'IIdromu, highly '''8I!el tlVl! orecAl. ESR i. norlllal in up to 22.5"" 2. C·rellCuVi protein: 8uDther ftcul.ol phllse reactant thlt" n,Ore iII" S;t ;V!!. \hln ES R. Hu th e .dvllnta84' thllt it o:.vo he perfon ned on frozen Olen :1. c a c : ,,'.y I how tn.ild ao,moc hrom;e 8uemi"~ ot . rhe umatoid f.~tor. ANA . and Il!rum eomple.tnent ..... ally oo rmal 6. LIT. I bnomuiJ in 3O~ (usua lly elevated !llk.,li"e phoeiphalll.te.) Ii. 1.e1.1I1"ot rheuma toid ractor and AN A "'" usually "w,lll;v. 7. to!mpor818rtery allglollTlPtr.y not tr.elpfuillngi0lrtlphy elsewMrl! Indicated if Sui· pldofl "flll rgt! aI"Wry ili vo lv,",,<,nleltlllll 8. CT' uI"ally 00\ helpful. oce report dutriberl c..lci6ed area colTlllponrline to the temporal ''''I",riu tJ 9. U:mporal arl.lry biopsy: '"' ~1f>W TEM PORAl AItTE AY BIOPSY Sensi tivity and ~pl!l:Itidty I re . ho... n in Tab/I
,./.1.
Indication, and timini
rlblcl2·'3 . ~.mpo,''''''''Y
Current reo;;ommend8Do .....: IOlmpwII artery biop.sy ill 8IL pa t.ient-J , UBpecloPd af I\avHl8 GCA". Preferllbly. biopsy ahould bto dane bl!fore t retltmenl is inlti nt..,d ... Ho... """• • pAlhologie cha",,,, bI! .....an after mOre tha" "2 weeu ~fthn-I I'Y"', th ....efor-e do " ot withhol d steroid& to await biopIy. Technique of tempora l a rtery hiopsy Bioplly of the coutra lawral .ide if til<" n ..:n side 11 ~~tl.-e III tun when!! ciiDlul lIuijplcioo is high innullell the yield by 5- 10'A-.
TREATMENT
No known cure . StMnids o;an produ .... ~ pl.o"'atic n!!lidand ueuIUy pr....ent blind. (proc,..:t5ion of DC ul • • prohlenlllU--4a btl! _flu i""titution af . deq"ataateroid.ls rare). Tot.llilyblind pauenUl or t110fie witb louenaodine partial VlJwoJ 10M ani! ulllikely to nI!~pond to any tRUmeot. t. for mo,n. ~.; A. IItllrt with ~n.91tt:. 4().6Q mlfd PO divided BlD-QlD tqild doli", is "I,,· I lly HOt ef'ff!d ive in Initillmanag",meut) B. ifno reapoulI! aI\u TI! bn:. and diap108i. teTtlin, Ito 10·25 nlj QJD C. OnCO! ""'POOBe ....:un (" suaUy ... ithin 3·7 days). giv," ,",,:..n. doH 81 q AM dole. for:l-8 wet!u unlil l ympwoos relOlved and ESR oo""aliz... t~1,U"!! in 87~ oflNltienu with",_ ~ wftOka)or . u.bili:es at < oto..;.G.-nnVbr D. once quiHc.mt., a if1Io\Iual u.per is performed lO preye n: encerbe.tions:- re-d\lee by 10 mgldq 2.4 w"klI to 40 mild. ~n by5 mgId 02--4 wb to20mgld. then by 2.5 mgld q 2""", wu lO 5-7.5 metd ... b~b i& IDIIUll.ained for MYn-1I1 manu, •. fol lowed by I ",gld de(:l':'menllq 1-3 "'1111 (... ulIl .... n~h af tre31""",1 il &-2 4 m05: do ACll 00 llto.roida "~n ES R normaliau) E. If Iym ptomt recur during trtaltn.tnt, prednisone d_ is tf:mporarily in· Cl"tllaed until tympl.OmJ reMlv, (]JQ1.ted riM in ES R is lIot lumcie n~renon to inc..... ltf:.-.idl") F JUlUlflUlhou ld be foUowed clOHly for . 2 ~atl 2. '" .e~ertly III pltittnu: methylpredn1'lOlone, 15-20 mg IV QlD 3. II1ti_l\lllnt thenop,.; cont.l"OVeni.J ... .Cllte blind"... (on51': witbin 24·36 h,..) in Il*tien l with giant ",,1I11rt.eriUI: 11.. colI.lder up lO 500 me aleth,l'tpndnBollllIe IV over SO-flO ",I"" (no eontrolled studi" thow I"e\WNI ar bhndnelMl) B. lOme bave used intf:nnitwnt inhlllatlOn " r MII CII rbon dio,,,d~ and o~an n eolll
OUTCOME.
Complication. of IW:roid tilen py occur in _ ~ or p&t ientl\mOlltare not lif. Ihlll!at· erunl. Ind loclud. "eneoral DOllI plll!"t5lOn f"'cture.ln _ ~. pepUc "I.... diM... ln
"
NEUROLOGY
~ 12~,
proximal myopathy. cSUlrscUl. exacerbstion ordiobetes: also see Po.9iblt cld~je
'''''''!litLt tI~t.of,teroids, P~i:" WI. 30-50% of pauenta will ha"e sJ.I(In\.
yea ... ) .... gam.lus ofth tonicosleroid I"
Polymyalgia rheumatlca
2.9.2.
PolymyaJgia Theumat;c:a (J' MR) and giant ",,\I arteritio (GeN i_fXJR£1i8) may be diffe rUlj pointl! on /I condnuum orche ume disease.
Epidemiolo gy)' Both GCA & PMR occur in peopl .. ~ 50 years old . The incidence increaaes with age and peMa betw~n 70-80 yeat8 and is higher a~ higher latilud"".
Polymyalgia rb e umati cs (PMR) " an inflammatory cond ition afunknow n etiology clinical rna",clenst\a A. aching and morning stiff/ieM in the c"rvical f' IlDOnth. The pain llstUllly increases with movement I . sh oulder pIl;n: present in 7<1·95% of plltients. Radi~ te& t.oward elbow 2. hip & neck pain: :;O·70'!l>. Hlp pain radlat@$ eoward$ 1Lo_ 8. age,. 50 yN1"8 C. ESR., '10 m.mI"hr (7-20% ha ve normal ESR") D. W!ull1!y respond" rapidly t.o low dO!le corth:ost./!roid. I. 20 rng- predn',,'meiday) utlHlow E. IYlt.eIl\lC lympt.olTl5lpl'e6t'nt in . 33
A. 5001HlO.OOO'" B. I case per 133 people .. age50'" favorable prognoais
TTeat m ec t PMR TespGn
2.9.3.
Other vasculitides
PERIIIRTERmS NOOOSA
AKA polyarteritil! nodol8 . Actually a gl"Oup ofllecrotidnK v~ulitjdes. ineludine; dauic periarterili! rwd0811 (PAN): II multil!yn.em disease with infl:unmatory ne· 0I"0Il;&, thrQm ......,~ ( ocdu.ion ). Dnd hCnlorrh"so or Drt<:riDll and arteriDI"" in ow~ry olgan except lung & ~pleen. Nodules may ~ palpated along medium sized mu&cular art~e •. Commonly produces monooeu';t!. multiple,.. weigbt!""' f"wer, Bnd tachyca rdia . Peripberal nerve mllrUfntatioO!l Ol"l! attributed 10 arterilicocelusion ofvo"" OOrvonirn. C!,;S rnBnifestatlOlIS are uncommon . nd indude HlA . $.ilUTt!! . SAH. felfnal hemorrhages, . nd eVA In - l~ • aJ.Iergic angiit", and granulomlltosi s (Churg-5trn uS!l syndrome) systemic n""rolizing YII8Cnlit;s
These pa t;enl.ll do better when t~lIt..ed with cyclophospbllmide rather tban "erai""
WEGENER 'S GRANULOMATOSIS
)\, systemic n
OWl" - cough/nemopt)'lli., snd/or n_1 airwal'l' - laTosanguinou! no""J d rainage ='"'p. Ull perforation - characteri~lie "i8ddle DO'" de.formity") lind freq"ently the kidney. (no ref"Jrted caBej; of kJdney inVOlvement without 1'f5pirotory.... .
NBUROLOcr
2.9. VRllCuliti! and
vB5fillop~thJ
"
Na .... obttruetion and e,ulting ... the ulual initial findill8l1. Arthralgia (nOt true ill present in > 50'1&. Neurologic involvement .... uaUy eon$isU of cl"lUlial oerve d,..runetion ( UIIUIUy II, 111, IV, &. VI; 1_ often V, VlI, &. VI II ; and leal t commonly IX. X, XI, &. Xli) and peripheral neuropathiU, wi t h diabetn illlipi du'(OOX.lionally pt«edingothet8)'ll1ptom. by up to 9 montha). Foealle.ionl of th e Drain lind spinal «Iro occur In. frequently.
.rthrit~)
Differenti.l diagnosis indudu! "Ia tbal m idline I/Tanu lo ma" (may be similar or identical to Jl<)Iymorphie relic>ulosis) may evolve into lymphoma. ~boy cauu r... lminant loca l dutrud;on ofth. nasal tiu ue. Dift'erenlilltion il crucial II thill cODdilion ;1 treated by radiation; one l hould avoid immune.uppruaion (a.,. eyciophOllph,mide). Problblydoet: not involve troe granulom... Reoal and tTacb •• 1 involvement do nOl. occur fungal dille..e: SpOrothriz fChfncitii &. Cocci dioidet may ca\IM identicallyn. drome other vasculitidu: up«i~lIy Ch ul'1-Stnu.. I yndrome (uthma and peripheral eosinophi lia usua lly _ n), and PAN (,,"anulomas ulvally IACki.ng)
LYMPHOMATOID GRA NULOt.'-"TOSIS
Rare; alfecu mainly the IUlISI, akin (erythemalOUs maeulu Or .. ndurat.ed plaequet in 40%) and nervOuS Iy,tem (CNS in 20 .... p"'ripberal neuropathiell in 15'1» , Si nuee" lymph nodn, Ind spleen are usually lpa red.
B EH(:ET'S SVNOROME
Relap!;ingoculaf lesion. and recu!l'entoraJ I nd genital ulcer., with occ.a.ional . k.in les;ons, thrombophlebitis, and arthriti' .., H/A ooeur in,. 50". Neurologic involvement includes peeudoturnor, cerebellar ataxia, paraplegia, aeilurea, and dural linus thrombo.i•. Only 5% have neurologic aymptoma as the presenting c:omplainL 86% bave CSP pleocyto&s and protein elevation . Cerebral Ingio(raphy is ul uaUy nonnal. CT may show focal areal of enhancing low deMity. Steroi
I SOLATED eNS VASCUUTIS
AKA i!lOIAt...:! IniP ili. o f the CNS. Rare (~ 20caaa repol'l.ed" &II of l 983); limited \.Ov_11 ofCNS. Small veeel vuculiti. i. ~ alwlYI praent - segmental inflammation and necrosis OflmlUleptomeningeallnd parenchymal blood v_18 witb lurrou.ndi ng ti.,,,e itchemil or heO'lOrmlp". PRESENTATION
Combinltion. ortYA, eoor"lion, dfmentia, I nd lethargy. OccuionIlly WilU' H . Poell and mullifocal brain dilturbanceotCurs in,. 8O'JI.. VifllalIympt.llllllIre frequ.,nt (se.:ondary either to involvemt nt of choroid.land retinllarteriH. or to involvement of villlal cortex - vil ual hlllucinatio.-.). EVALUATION
ESR &. W8C COWlt Ire usually DOrm. l. CSF DIlly be normal or hl~ pleocyta.il andlor elevated protein. CT may ahow enhancin( areas of low denai ty. AngiOCrlphy hequ.ind br di'enOl;a>: characteri.mally . ho,.... multiple area' of Iymme\.rieal nlfTOwinll ("ltting ofp"'.rlt" cooflguration). If nannal, it don notuc1ude dilgnotil. Hiltolop:.l diagn06i1 (recommended>: III bioJMY materi.l.hould be cultured. Brain parenchyma biapey ;nf~uently , how. vl.ICuliti .. LeptOmt ninpal bioJMY invariably involvement.
.mow.
"
2.9. Vllculiti. and vllculopathy
NEUROLOGY
H YPERSENSITIVITY VASCULITIS Neurol~c involv.ment if not I prominent fe~tl.Ire ofthis gl'j)up of"8MUlitidl!$, whIch include: drug induU:d enel'l1c V.~uli li. cul.neo .... V8KUlill. HrulTl siwOII': rna)' - ancep/llllopolh)" Mbu ...., coma, p!!nphe'!ll l>~urol"'Lhy .nd br8th.ial pluopa tb, H~noch-&:h6nlein purpura
D Rua INDUCED VI, SCULms A numlHlrord",i' !l.""".lIOCieied with th"d~elopment of~".ebTl.I vlUlCUlit ia. ,odude metfulfllphellolfline. (".peed"), tOeIIine.{fl'llok vI\SCuliti. ClCCU ..... but is nlJ1!). he ..
n,ne
oln and ephedrine.
2.9.4.
Fibromuscular dysplasia
._i.ted
A vnculopa thy (oogiop.thylatrectilll pnm anly brllndMs o(!.he eoTta, wltll reo.1 nrtery involvement ill S5'i11 of caH. (thl. motl IlOmll"llln .Itel.nd eIImmonly wIth hypertM5101>_no" di_1e hI' an i.otldenee of ~ 1'11> •• nd .esulls in mui t ifoaolll ...... rial cO"'l"lrietions ,"d i,rne"""nirlc regiol'l5 of.oeu~,n.l dilatallon, The le
ETIOlOG Y The ItCtual etlolo1O' remains unknown, alt.hout:h«lncenital df~ of the media (muscula r layer) and ir.lem&i ~a.tk layer oftbe sr\eria bar bee.;> identified which mIoy prediSpoII8 the arteries to injwy from otlll!T"o
1'be reported incidenNof aneuJYSln' with Fr.to'" I'8Dg.. from 20-50'11>.
TabS. 2· 14 PrevIOU .
CI",
.ymplOAW III 37 of aQfloc:rlflll' FMD'"
PRESENTATICJN M <>It f)l.lielll.f hn,·. recurrent, multiple ftym plOml Ihown in Tabl~ 2-14 , Up 10 so.. of patienta p......,nt witll epiaode. of transient «rIb' ll itdlemia or itlr.,.ctlon, Haw"ver, FMD IDAy also be an incidontal ttndi"l and lOme o:a...Hhaye beenJolkrwed for IS ye.1'II WIthout recurTt"nte oflachemic .ymploml i~tinll thll F MO a,sy be. ",1~livel)' benifn condition. Hud lche. are ronunollly unilat..nllnd may be m;'" taken for lypical mignllne. SynllOpe may be cauoed by illvotw.men~ofth" carol.od .;nUl_ Homet'••yndtvme Ottul'll in _ N ofCII ..... T.waw ehanen on EKe DUly be seen ill up .... on.thlrd olca.sn, and m.y be dUI to i.nyolvemtlllto! the roroo"y arteries.
OW;NOS<S The °l6ld·.tandll\l" for the disl_1I orFMO i. the Ingiogram. TIwo th ... a nJiognphic I)'pes of FM D'" INI .hown In ThM. 'J-16.
TREATMENT Medical t hHIPY Inc:luding entiplat..11I medicatloll (e." 45pmnl " .. been TeCOmCMndecl.
NEUROl.OCY
Dil'«1 5urgic~1 trealment;. JW'oblem ridden due to the diffi. tultl_uon (hith ca rotid . rury. nU. the ban of th. al
T. bl. 2·15
OlfMO
t,.
2.9.5.
Miscellaneous vasculopathies
" I
CADASIL
t Key futures clinical. mignllnl!a, deJ;1anua, TIAs. ptyeh,uric d iilurbanc:eI • MRl: wllilt ~t"'r abnormahtiu • autosomal dOI1l",an~ ir.!w.ritllr>Ce • anticoaglliantl controversl.l. ,tneBUy di$CO\l.~ged An a,,",nym enr ClTebrlll .... ulO$Omyl Dominant Artuiapll1hy with Subeorticallnf· a r~.nd Lomkoe,""'phalnpath,.. . .... f.mililll d;~ with onnt in ea r;y ad ... 1thood (meDn .ge lit omet: 45 '" II )'nil, mapped to chroflllMOfDt 19. Clinical and neuroradiglogic fca IU ' " IN!limilarlOlhoIe aeen wilh mullipielubcorticul mro~U from HTN, ucepl t here II no eviden~ofH1'N. The vlISCUloplltlly i5 disti nct "om thot Ieiln in lipO/lyal'nasis. ar1.eri000erosi••nd amyloid lI.IIgiopal hy. and cause.s thiek. ning of the ~ia of lepl.ol!leniniNl ""d penOI1lI,,,,, anerie, m-.uring 11)0.400 "," ," dia.nw!Li!r. Clinical inYillvell>ent: rerurnontsubmnkat io rlln:~ 184~), prawns;v"' .... u"pW'5e dtmentia (3 1"), l1IigTaine "'ito'! li' UI"lI (2'~), a nd depress.on {\tOOIi-). AlIl}'mptomaU( Dnd IS'l\ofUympt.omalie patlentil had prommenuu bcort.ieal whit..·matterand basal ,anlll ill hypennten$it;u on 1'2",1 M Rl. Trutment: Cownadinf>is ua.ed byaome.
2.10.
Vascular dysauloregulalory encephalopalhy
This H<:tion encol1lpa$5fta IrouP Dftnn!phalopathiet that ma,y be I1'lati!d to dl.". dered vai<'u lar autorqulatioc-. Et.ologies ami find il'll' iodud.: 1. tho~e due IO ,~ ...10Dd prfIMure .~vationa: imqilli "Udlellhow I)Im metric (onnuentiesionl with mild ",a", ~tfect Ind pII t.(:hy "",ha_t primarily in the , ,,beort.iCIII wtul.e matllt" of the awwJJl!!la"' {pouiblJl baaitule of limited .ym· plthetk innervltion in the ~rjJlLcir:tuJ1IJ.lwl} whie'" may "rod"," coniCll1 bhndMM .... hype rlf'n.;"", ""'c"ph.lop,thy j " may Ott'" w.th IIUIl ipant h~TUn.ionl 8 peripartu.,,; lluociauod wit h ferebrlll ed'm.'", on"", t'rnponlry, but (pi"manen~) infarctiOM IllIG OttUr L may p,_n l (' .1. with blindnus) du tio ~ pre",. lIty complicated by p,.edlm pua Or eclampsia" 2 rn.y develop 4.9 dlY" pO:lt.ptt rtum . nd ,n',)' be e_l~tt-d with
"._pMm"
2.
..
C cyc1Ot1porine lO~idty uremic tllH phalopoth,e!o: imalJl ng iI"ditsshow multiple ..... or.ym~trieede,. rna in the bani ""'glie. with Ie\'ert _ _ devdop11\i (oaollnfam. WIth or ",i lh. 0"1. hemoITha~ 'rh_ diNOl'derf lire asliOCiated w,th elevate:l 6tJN .nd im-tud ..: A. u•• miD
2.10, Vlllnl iar dYliulOregu latol") eneephalopathy
Ni:UROLOGY
B. glomerulonephritis C. hemo!ytic. u,..,mic . yndmme (HUS) O. thmmbotic thmmbocytic purpura (TTP) These pIItienUl may present with headache, seizures, mental status change.s and f.,. cal neurologic deficit. Intracerebral hemorrhage (le U) may occur.
Tre a tm e n t Disordered autoregulation mandates tight control of blood pressure to avoid hyper· tension to reduce the risk ofICH.
2.11 . I. 1. l.
•• S. 6. 1.
S.
References
en.u......
Cool... """, o.ff.~.'i.1 di,,,,,,,i, of ...• "'<0'; .... di,.,.,. •. JAMA 1": 30111.6. 1917 FI.ml.. I(C. A"' ... AC.i'd
1o.""..
9.
W.l
10.
~J
II .
''''
W, T.... ""' .. otmi'.-l ... LMn1 JJ9: IlOJ·9.lm . ~ m"'lk I P.(;_,,< DS .5<)'lxIkI M E:a.".. 1.0<" .0000000io .born •• Mell
h<Mk1lo:
N _ '2: _
Il. ll. I'.
"
•. 1915.
q..,".
L..opIti.M L.GoI
\>I,,.., i,,,,,h<Mk""""',
n.
".
.." • .. n.
».
".
".
poo.I"')'tIQl"'" Ioe>d",,""- lu"
".
'I.
M.m>s ~ • R. i".oc...;'1 bypOltn>_ ...... ,Ii, "<."""In. J N",,01 S"' . ... .." ,,"y
n.
19.
Mil
ClwtUc
I(olk, II.! C.SOI"", 0 E. u"b."" ...... .... . llorit~ foe.1>< m. ",,,,,,,, .. oIhrlil,,*,', di
NEUROLOGY
.Y.,""....
..,.·.d.......
m ,.
F'oo<. C M. ""'1 0 W. Sch
• ...... i< .. Hoe ... I", <e$
ptOID
Ntutol 'J' Ill·) I. 19n S... "'",. I W: Mg" iplc .
17
,-
UnJ"""J w. Ball.,., P. TttNdJ W•..,11.: ClIrQoi< porki.IOIIb m ,. hom.",,,,", '0 ' """'''''' Qf ""pori, di,..4IIIl"l ScI<e« 219, 979-10. 1911). 1..>111,1. E.I...<>wIOA"' : """i • Fi ... 'o( ,..., pano. N Ell" J Mtod JJ9: 11)004.'1. l'11ll. K" ...... o M ():~ ..... _katp.ol.,·IO ).. ... Iote,. Ae .. Nmtol S
.yndtome.
Cu. H.GoidI,.i.'" S.R.,k,.It, """
10
"<'"'.'''''
IQ79.
16
.,.,,..Il>0l,,, Spoo,._
y,.,., 1(, 1JpJ... on """""",·s
Proe6ol. S77.J16. 19f19. aorklloff. f;lippi M.Millot OH ... n1" Compa" "", 01 MR Cfilttlt .. n", "",,""IO. IO!'""licl .... •• ,.;". '0 <1 .....11, ""nlli.. mukipl. ",,;""";'. a,... 1» 1059-69. 1991. T .. """ ..... R...i,. .... M ........\1 .... "I" boIOIt<1 Ge· myol ... ,'''' '1ool""""" c.,.,.,...;_ <>f dlfl...... MR ;""S'"' rnl
!mo.i.,
c.,ly 4
". s....
an I M .II<>II..,Ow, B.... ' H L • .,al" M'a'
.. lie 0"DQIIIf><0 i ""Sinc afI!I clinical .. loIi"",hipo ,. ",oUipl< ",,"""i,. M.Y<' Pr .. 6l: 17.·... .
•
cu.
1917
""""'[i. A I. 0.«", ... S. "..Ips C E." aI. ThO
2.11. Rderentea
00-
"
".,V.,.
aIIlI<) 01 mlsn<'"'
~
" '"
... "
.,
.........a 1/1 "",icnl' .,,,11 ''''f'OtJm>oj .. /'1 •• ,oIocrH. 16:32·1, I"1 ........ D L. u..... E 8 ' M ",~Ie ........-., ",,",in. ofthtbnin ..... IfII... A""I ...... M«lIOll 001-
«""
l4.I~.
",_i, _
...
,'''11«_" ..... "' ... ""............
L>w~I.M.I"...-DE,CNS;S~mI_
M•
'I\".......
i< ~ 1""'1'" of i.. ""my.II ... '"I k
Ftu>J .... MS.n.o.,.p_[ J,O', .. ~O . .,
Ill.: R""""""ookoI ..1t>dMo1 nuol .....y.;, ", "" d, . ~. 01 .-..IIlp1o Itlcroo,,_ ~ _ ....." ............. A«h /'Iou",I.:!', S6.l-10.
""yy G 101 , 11
or _ .... er"""... """"'"
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>.
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Gu,tloi... 6ar.t.,ncIrDm&. IAJouI II I. lU.-?O.
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" ". ~
~
" M,
..
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k'",,'" I c. OJ'''' M L! M1
" O. W
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....
,,1M. ""'_'......
2.11. RafBNmce5
oloTri_,/'I. ~E:I"" ........ I .~"
""""""'IOII&k>rnpllo<"",., .,._1""""<0"'" S."/'irtonI 9-I~7 ' . I'I1 • • RIItittIJotIIM S. D
... &hIl .. """"'''''''"~'''''''''''''' '~)''''' lI1.yo C ~" I"ro< 11. 601~ . 19~1 , 01......., V 1'1<.. ~piNI n..w ......"""',.-.Iock.ol """ ..."n>IaoJ"'kl&I
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..,.,ln ","",>I
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.
'
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, n. ~
" r..I"""",,,._. J""',
""" ... """"go....
s.rc._ "
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"
..... plool"",)'
,.". ". 1f
". M.
)(0: .... J J ~. I()<"OI '"III<)<"i" .s.r.ydj""'I Io. ''''''' oftht Inlo' 1........ "''' . .... ~pIo
r>f1111 ow MRI . f..,UI< tnnO, .... 10)'<11'," J C ..... p'" A..... T....... I' 6O'i.t.lonl . 0.-. .... " C, B_~ V V. Coole S 0: 1'01,,01< be.· .r""01 off... Dr"'. . . . . I v ......... 1honoP)' i. "'."
n
" "
".
_'II.
[E,
"'UIOIIIOCf;I"',
'9Sl.
~ .... F. C. Gol< ... S t.D_ ~ M.".I.
0."""
potor-""'" ""'.!<>polhy, C..ur.1 ""1.0"",,, . ...... iltd. A,," N. u,oI JJ . ~n~. 1m Moon: " M, It. Ntun>lol'" '''...pll",,!1on< of,"",.~'I .. An. 1'I<~ ... 1. jS~7.19n . Sol.""", C. COOl'" F. So,.d' L. .. ,,/, PoI1I"YlI' ".,h<~ -o:c!l_riW. N to.&1J M«l :J.07· l61·11. 'XK1z' SaI_iC,G"'-IS " .O'f. ILon W 101 , •• 0/ Tho "",\dtru 1!~ i< ill 01",._ M..... _ : A~n""l ........ i.,."Y
c..pp,"
..........
<>f.,..., ..
"'""'Y.
"""'y. "'"",.""'.
T.,""...I....,.,,"'A
' hlll""""'c:7 9], ~IJ..)O . 1"&, Ah. A A. Lie I tl G.It 0/" Iittw 0100, pt< . .... , -,i<.,.,~ If."""",.ff
't."""',,
NEUROwm·
•.".
lol'" II l.F....... 101. Co-rd>r " y""",~,.. . »o<_
a....cT,.. H_OO IbnpDM.,,~'.· ' '''
".
1<01 . .""", ~
...."'.. ......,.. A_ L....... rotod \11' 6U-1II. 1~11 . Sol........ C. 00iIrC1S E. O'F,IIoo W rot. II 1>/ ' fit...".,.., '" pOIJ~""p:o _ 1 0 0hn
".
r-.,.;" 11'-«11 ("~)""""d A... 110-
U.
" U
U W
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..... _ ,lIJ.. WI~I . 1 . c...;,,;F.WoarM C, OLIrieoi l.tltoI·. IitJ""""rl< ~ ..... ooodC·_~ .. _ " , .. "" .. _ _ af_ ..,,,"' ...........,I. roI,· ",",," rtorolUlico; A pr(IIpO
..,'o!t:a._
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_. ~" 22D-JI . 1'lIJ No .. _",.~ M«I ........ .It. 1S-6.
,,,,
~TlU,_'j.l .fecJ AS
1_"111"
" ' ' ' ' " _ ......... .,. ............ pocoi ... dI.opDI. ""_~
N6UROW OY
.. _ _ A.. JMooI 1. 97·
IM, 1981. 1100-6. 1m H_ A N. IIWC l .ZnI« DL.,oI. F,,,,,,,,,,,,· "'~~''Ill»l.
_,_".0", "'I~'''1' AJ R 1:16.9»-60. ,. ,
...,
l.L:rlc:oooo 1( . rc......,..,.""'I.. d,>pL.oI. ..,11<>1'.....
..... "'" 1Jnioo· ~ .......... 00 d! .. • ai, ..... 1I<.. ,it..~ra
M"oI,1'" II L. n_.... L"u) ;p.......... ,"" koo ... III C..... '" '_1'101 II.. oIiou .., 5" . 1<0 HI SH, Im .
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"
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O'I>oroAO ......... '- ~ i!::A"fJOI'''''''''' '''''' '''''''
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M T" ."" Cion,·
dl"""""""or._II· A"""'''' ..... 1.....1i.., ........ 1<16: 9~9 , tw,o
"
3.1.
Surface anatomy
3.1.1 .
Cortical surface anatomy
Figun 3·/ shows
som" importanl""rti~1I1 surface landmaTlta. This may be h~lpful in corre1atinr with MRI to detennine the location orl~()n!lL. The MFG ;" usually more .nnuou.s
CENTRAL SULCUS (Rolandic fissure)
than the IYO Or SFG, oonnec~ to p~...,entraJ gyrus
lind (t oA.en
the
viII a thin i!lthmua. The celltrsl ,uJcu~joins the Sylvian fiuure in only
2% orcases (i.e. in 98% of OilS"" there i , II -aubcentno!" i:'Y1'UJI) , The ;nte'l»'rieLaJ ~ulcus (ips )
I"parates the s uperior lind inferior parieUli
lobules. The IPL is composed primllrily of the AG lind S MG. The SylviaD fissure lennina te.'! in tfle SMG (8rodmann'. area 40), The .uperior temporal sylow; tenniollt.es in the AG .
Figura 3·1 C",ebral collical ",lIace lIrlaLomy' BI .~
Btodmann'l" ..... (~~ s... fllblll".,.rod f IllW3·ZlOIlbbr.
¥lotions,
Brodmann's "r"ali Figun 3· / also identines the tiiniClllly significant al'" ml/.p of th" cytoa reb; tectonic field~ of the human brain. FUnct,ionw aignificance ofthese areus
"all ofBrodOlann's (8r.)
ill /1$ (OUOWI;
Table 3-1 Cerebl1ll sulci pr«>tntral SlJeus poSI<&rlIIaI sulcvs ,a, if, SUperior,lr!IlIrior lron-
,..
,~
,,--
Sr. areas 3,1,2: prima ry tOmato8
"'"
"
3, Neuroa natomy and pbysiolocy
N1!UROSURGERY
Sr. area 17: primary vi .ual C<)rtel< We",;" ke'. ar .... (lan,guager": ,nlhe dominant hemisphere, most of Br. area 40 a nd II portion orBr. are~ 59 (may .. 190 include _ tJOSl.erior third or
Table 3·2 cerebral
STG)
Br. arell 8: the strilWd portion in FIf;. ey~ tield ) irtitiatc3 vol· untary eye movements I.(l tha oppOllite direction
"re3·1 (frontal
3.1.2.
Surface anatomy of the cranium
CRANIOMETRIC POINTS
Craniometrie poin!!! in Fillure 3·2. Pterion: ~gion wherf). the following bonOIS a~ ilP' prollimaU!!i: I'rontal, pari. etlli. l.eroporaland sphenoid (g>'1!IIter wing). Estim ated as 2 finger-breadtlulab(.v" the ~ygomatic arch. and a thumb'. breadth bmind tbe frontal p~ofthe ~y§l:l_ matic bon • . Asterio n :jllndion of ilImbdoid, occipitomastllid and parietomllstoid . "t"us. Overlie. thejllnoli(lll of trnns"erae and lrigmoid .inu,;es . Vene.., the \.Opmosl point Ills skull. LlIlIlbda : junction of tll .. 1ambdoid and sllgltttli sutu .... s. S t e pbanio n: junction oftbe coronal ,UIUreand the 9up('rior tlllnporal line. Fll1 ure 3-2 Cranlome.rIc pOOnl5' CfBlllailltll.vtes G h.bclho: thB """" for- AtJtiIt!VJi!IQm; OWS ,. UIOtitCI w'ilg '" ~~ IIOoJe. Q\ " ..... 01l1li r;uward prtlject.ing painlof tbe Iol!. is " lambdoid SlJIuIe, sU ",supetiot II!IIpOI8i line, ZVO. z:ygomali:. forehead lit the level of ch~ N;Jmed boneS awe~r irI.~ vppeI elise I!1t:e1~ supraorbital ridge in the midline. Opiathioll: th e p61terior ma rgin of tile foramen magn um II' the midUn e. 8 l"fl gm 1O: tbe junction of the eoronnillud ugittaJ BU1Ul'@a. are
~ho\lln
or
A.
l~.~ r~octioo ... "" .... ~ <elitbJy I.... U.. d 00 . "",tuol< """,,,,,, d~n.. l
NEUROSURGERY
3 Neuroanatomy and phY8loiogy
..
RELATION OF SKULL MARKINGS TO CERE8RAL ANATOMY
Taylor-Haughton IiDea Taylor-Haughton (T_H) line s ca.o bf! constructed on an angiogram, CT scout film, or uulllt-ray, and can IMn be reo coll8lructed on the patient in the O.R. bned on vi.ible external Isndmsrk$'. T-H li""l1 are shown 1108 dashed lines in Figure 3-3. 1. F't-ankfurt plan e, AKA baseline: line from inferior margin of orbit through the lIll.Iltt ma rgin of the exte rnal au dio tory meatus (EAM) (as distinguished from Reid's bn&e line: from infe_ rior orbital margin through thec~lIj~rofth e EAM)' lo llll
2.
the rustll1l<:e from the nasion to the inion is mea'-- ~"",., tine su red 8"rt>55 t .... top of the CIIIlvsria and is divided into qusrters (Clln be done aimply with a piece Figure 3--3 TayIor·Haught"" liMs of tape which is then and Other localizing methOds folded in half twice) 3. posterior ear line: perpendicular to the bsseline through the mastoid process 4. oondylar line: perpendiculsr to the b8.$eline through the mandibular OI)ndyle 5. 'I'·H lines can then be used to app,oximste the sylvisn fissure (su />flow) snd the motor OI)rtex (alao su ~low)
Sylyi an fissure AKA lale ral fi ss ure Approximated by a line connecting the latera) canthuB to the point 314 of the way pDl;tenor along the SrC running over convexity from nasion to inion (T·H lines).
Angula r gyrus Locatedjust above the pinna, important on the dominant hemisphere aa part of Wernicke's ares. Note: there is significant individual vanability in the location'.
Angular arte.-y Located 6 cm sbove the EAM . Motor cortex Numerous method. utilize externallandmarb tol"""te the mowr strip(p~n tral gyrus) or the ~ail.ull:l.m (Rolsndic fissure) which separates motor strip anteriorly from primary sensory cortex poIIterior ly. These arejust approximations ain<:e individual variability causes the motor ~trip to lie anywhere froro 4 to 5.4 cm behind the coronsl 8utUre', The centralaulcus cannot even be relisbly identified visually at 6urgery1. llIethod 1, the superior aspect of the motor cortex is slm08tstrsighL up from the EAM nea r the rnidlioe method 2' : the <:entralsulcus is approximated by connecting: A. the point2 cm posterior to the midpoaition of the ArC extending from nasion to inioo (illustrated in Figu.re 3-3), to B. the point 5 Clll straight up from the EAM method 3: uBing T-H lil1f:s, the central ulcus is approximated by connecting: A the point where tbe ' posterior eaT line" in tersects the ci",umference of the skull (Ut Fi6W""1! 3·3) (usually about I em behirnl the vertex, and 3-4 em behind thO! COTOnal ~uture), to B, the point where the 'condyla r line" intersects the line representing the aylvian fissure
"
3. Neuroanatomy and physiolOiJ
NEUROSURGERY
me.Ulod 4: a line drawn 4S' to Re id's ba(.e liJ:,e starling III tha f le.non points in the dJ.tKl~ Gf tbe. motor $tOp' .. ••... )
R ELATIONSHIP OF VENTRICLES TO SKULL f?iIf,un 3-4 ~bDW~ the. rei,,·
tionsbip of non -hydrocepbalic v!!ntricJl!!I to t b!! $kull in t be lullral view . Sollie dimensions of Internl are 8110wn in T{Jbl~ 3_3'°. In UH! nOIl-h~drocephaHc
adul t. t hE' latera] ventric~. lje 45 ~m below th outer ~ k ull !urf!ct!. The center an be bodyaftbe lateral ventricle "UI in tile mid pupillary line, and the fronta l hom i. intersected by lit line passing pe.rpendicu la.r to the colvaria 1\10118 this IIneu _ The anterior bomsextend 1-2 CIIl IItnteriorto the C/lTOlIlIl Bnllln,\'. Averagl'llength of t hird ventricle _ 2.8 CIO. The mid poin t af'J'wining'.line ( . in Fic''''' 3-4) should lie wiUlin the 4th ven t riet • .
FIgura J-4 Retalioosl'>lp oj venll.c:le5lo $lWll lanclfTWksAI:It>«Iv~liont : jF E ~ OOlall»'n. B E t)II
-'' ' '1'0' '
T"ble 3.3 Olmensions from FlgufII 3-4
"""""'" ,
(S!~
Description
lowerUmlt I~I
F'PgIJfI1.NJ DO
,.
OJ
3.1 .3.
Avrr. ,~,
&'tglll at IIOIltBI 110m atlieriDr LO FM
dis(&nCe!ram clivUs LO fOol at41h vl!lllriele I I ievei oIllStiqium' Iengm 01 411'1 ventricle at le-;eI oIlast¢Im'
'"10,0
dis18/1O&!rom lasb!lium' 10 opisthion
30.0
"".1 14.6
'"
Up$It1' lI mlt (mm)
40.0 19.0 " .0
Surface landmarks of cervical levels
E.timalel gr""rvicallev~J. for "nterior cervl~"l BpinelUfgery may be made using the lan
NEUROSURGERY
T" ble 3-4 ClWliClillevel. "
3_ Neuroanotomy and phyo;iology
"
3 .2.
Cranial foramina & their contents Tllble 3·5 Cranial lO'811'1 1n8 and their eontent s'
, III
I
I
*_.
"lItn..alioN. a *~. U • ~ ~e(..... v, W s .M'., n _ ........... M '" ~ . • .,. ...... _,I"," • Iorlrt'ol'n, doll . If;vl,,,,,,,
n~"'H.br .
_ Ill""",,, Ct.
Porus 8c u s ti c u s !~ Fillur<': 8·5) Th'l lilll lrulnts of th ... aCQuuj" pon;on orVIIl ""netTate th e tiny open-
inp arthe lamina crlhrosa of the t'tIChlea. "rea" .
T ... ns"t'r'IIecrest~ sra les auperior ves tibulHr
laool ca~1 (edt VI!) wni::alaesl
,,<>
area /lll\d fadal canal (above) from the inferiCl' vestib ular IIl"1'a lind cochl~ /Ir .. rea (below'" Verr.ical Cn!!'It: ""pllo
ra tes superior v", tibular a!l!1I from meatus to canal.
"
Ito ulncIe &. 5!!perior &. literal semiciroJlar dt.c\$) supelPDT ~ ilIe!I
1IaMW!/$& tresl (CIlSIa lalQ/orlms) lI1IeTIOf vestibu~ area. (10 $OICCule) ~~rnef\ sitIgtia.e (10 posterior semio::ireul~r IliXIJ
tlllCllJ$ 5jliraiis 1000miflOSU5 (oochlel r ~rea) (ae(N.Islic: pcttiJn 01Cr N Villi
r~ci~l
3. NeUroatliltomy a nd p hysiology
NEUROSURGERY
3.3.
Spinal cord anatomy
3.3.1.
Spinal cord tracts
~
..
MOTOA (descending Pllths)
bi..jlrectional ",
S = sacral T = thoracic C = cervical
SENSORY (ascending paths)
11
13
"-_.1. anlooOf spinal
,"'"
15
anteriOr motor nerve roo
A gure 3-6 Schemalic crClSS-sec1lOI1 0/ oervicaJ 'pinal !lOrd
Figun.3·6 depio~ a cl"OiSs-s« tion ora typi ..... l spinal cord Bl'gmenl. combinlng some elemenU! from dirre~nt levela (e.g. the Int.ennediol~t.erlll grey nucleull is only present from 'l' l "' _ L l or L2 where th"I"I! art! synlpllthetic lthoracolumbarou tOow ) nudei). It is schematically divided into p;;<;ending lind descending halves, however, in actuality, as· cending and desoending paths <x>e.ust on bolh aides. Fisure 3·6 abo depicts sOme of the laminae according to theschemeofRexed. Lamina II j, equivalent to the l ubstantill gelalinosa. L.e.n.;nac III and TV are the nudeus propriu l. Laming V1 is locat.ed in the baJf.' IIflhe pOf!terior hom. Table 3·6
NEUROSURGERY
tracts (
I
3. Neuroanatomy and physiology
Path
hllle 3-8
functlcln
Flgure3~
SeNSATION
PAIN & TEMPERATURE: BOOY ReeeptO\'1l: tree nerve end ingJ Cproblib!e). 161 order neuron: amaU . nnely mye'inll~ aff,,",nta; ..,ma in d"",,1 "",l","glion (no l)'1UJpae). Ent.croord aldor1!Olptllrallrllct (zone OrUOIU\ler). $)on_pH: lubltoanti' .... (ati nooa CRaxed 11). 2nd order neuron ll.I.on CI"OQ obliquely in the pnterior white WllImlalmllll,c:endlnll ~ 1-3 Ilegme ne8 while CtoMing 1.0 e nter LM Illma' spinothallmic lracL Syn>l~' VPL thalamus. 3Td order neurons pas. through Ie 10 poIItcentcaJ gyru& (BrodDl8nn"...-u.s 3, 1,2)_ FINE TOUCH, DEEP PRESSURE & PROPRIOCEPTION: 8(XIY F ine touch AKA di!lCl"iminative 101><'11. Rettptors: Me.i$ll.ol"\'"" &. parini8n a..-pu"..r ..., Merkel'. d',b. frl!e nerveendlngs. lit ord~ neuron : helvi'" myelinated 8tfe~nta; 1IOm8 in dQf"llll..ll'OOt pngJioa tno IIYnBpse). Short branchnlynapte in nud eu. propnus (Rued 11\ &.1V)orpGliu rior grq. long f,ben enter the ipsil at6-al poaterior CX)lumn. with(IUt lynapsit18 (below T6, fucic:-uIUlgratil ••; above 'l'6: rudeul u. uonutu.). Synapse· nudeu. f'"3ei1is!cuneatul \ ... pec:li~,.). j ...t above pr.amid,1 dKUlHlion. 2nd order neuron axon. i;)nu iolemalan:uate fibers,decu!ISBte in \ower medu lla a~ m e dia lle ....... l..,,, •. SynaJ)H: VPL thalamua. 3Td order neurona pHI throullh Ie primarily to P'II'tcentfal gynq.
L.1GHT (CRUDE) TOUCH: 800Y
RKeplOl$: ILl fuw tooch t- "bout). a110 peritridUa.i . rboriutiooa. lit order neuron: larp, bovll,y myeli,..~ .I'fueIlU (Type IU; 50lIl. in do,...1 TOOl pnelio" (no '1fUIpH). Sortlfl IlC:eod unc.--l in pIIIt.CDlumn. (with fine touth). lJIast qnapu ,n Re.ed VI .. VII . 2nd order n-e .... on lUCon. trOM in anterior whiteC(IIDltuMmt fa few don't crou): enloer anUorior .p;nOlh.lllDi~ 1r'~L Syn'PH' VPI.. thlll.,O .... 3rd order ne .... on. PllIIlhrough Ie prim.ril,y 1.0 poIt<:en1r. 1CYflII.
3.3.2.
Dermatomes and sensory nerves
Fi8un 3·7 . hOWl anlerior.nd pCllUonor view. uch ICheQUltically Hpar.lltltd into pi!riphera\ H n-..ry nerYll di.tnbuUon.
li!n~d«rm.tome. (M'fPDt!IIUoi) .nd
3. Neuroan .... tomy .nd ph,.iolOfDl
!-lEUROSURGERY
ANTERIOR
RMAT OMES
POSTERIOR
CUTANEOUS NERVES
Flgure:1-7 o.,rmalomal and sensory nerve dislrl:>U1ion (Red.awn lrom -Inlroduelion 10 BaoiC Neurology'". by Ha.ry O. PallOn. JoM W. Sun
3.3.3.
Spinal cord vasculature
Although a radiculAr artery from the aorta acwmpanies the nerve root at many lev_ els, most of these C()ntrihute little flow t() the "pinal C()ro itself. The major blood supply t() the IllIterior spinal C()ro is from 6-8 radicular arteries at the following levels (" Tlldicu· lomedullary arteries·, the level.lUited are fairly C()osistent, but the 5ide vann" " " ""): C3 - arises from vertebral artery C6 . U$UBUy ari&es from deep cervical artery} _ 10% ofpOpulalion lack In anlcriQr C8 . uyually from cost()cervical trunk m ic:ulu ilIluy in lower cervical . pine'· T4 orTS Adamkiewici (rUM/OW ) The paired posterior apinalarteries are leu well defined than the anterior apinal artery, a nd are fed by 1(1·23 radicular branchl'$. The mid thoracic I'
NEUROSURGERY
3. NeutOOnat()my and physiology
"gu,e 3-8 Sdlomillc ($11\Qf1l/T1 01 ~I COld a_1aI su~ty (Modi!ie
Oiognosllc Neu,o'.~IoIogY . 2f11l"'~ VOl""" II. PII. II B1 . Tav." ... J M. WOO
p.''''......
.Artery of Adamkiewicz AKA arteria radi cwaris antl'riQr magna the main .rtvnal tupply for the ,pi lUll cord from ~ T6 to the ronu. located on tb. I"R in 80% O<:c:ura ootwHn 1'9 &. 1.2 in 851l. (between '['9" TI2 in 75%); in remaining 15% ootwoon T5 &. T8 (in t.... eaa latter cases. lhe~ may be a ~uppl 'mental radicular
artery further down)
usually fairly large, givtl8Qffrepila.lk IUId CAudal branch (J atur i. u3u811y larger) giving II eheractcristie hair·pin appearance nil IIngiogruphy
3.4.
Cerebrovascular anatomy
3.4.1.
Cerebral vascular territories
FitJun 3-9 depicta apprnUmn!~ VlIOitulardi,tribut,oBR nf the major cerebral anories . 3. Neuroanatomy and phJlliology
NEUROSURGERY
ThenJ i . conaiderable "ariabili~y ohbe major o.wr il'll'. " willi ... the cen lrB l d i!Lr ibuti('>1I {tJte.lentiadostrintH. recUrntlt artery of Hlllbner (ftH) (AKA media l uri.1.e artery). d c. ha"e v,u:yin;: di$tributiaml'nod may have oligin!l ()/T' ofdill'l"'!cne Hgmentl oflhe middle or an...nor cerebrw Itt.ery)
4XIAI. ... ,EW
CORON.I. ... ,IEW
3.4.2.
Cerebral arterial anatomy
The aymbol "-" i. used to denaw a region supplied by the indiCllted .~. SeeAn . liography (NnbrafJ on pIIte 130 for anrlographic diagram .. of :.he folla...inC .....tomy.
CIRCLE OF W ilUS A baJIIJlOOd eonfigurlluon o{ th e Ci.rcle of Willi. ,~p..-nt If! only Is.. of the populi' !.loo. Hypoplasia of I nr both p-comms IIC:curl in 22-3K, llbam~ nf hypoplastic AI ,.g. mentll oa:\.ll'll in 26".
AnatomicalllegmeDta of iotrllcranllll cere.br . l arteriell carotid artery; the traditional numbering
(~un\.er t.~'~'~"~3~'~'~~~~~'~';Ih~'~'C~'~
to th ... dIrection offiow. nnd theda! nwnbering ly,;teUl" WlU &(,>IJI rostra l totocau ""bl!!nt "fth.. laher IIJ'Wri~). A number (,>f ayl\.e1llS have been deacribed to addresses thli in<:OlUj'tency and qlflO 10 IdeDtH'y I1l1n· lomic:a lly impDrtant IMIgmenl:l oflhe leA th.t wen! n .... oI'iginaUy delif'lf!al.l!d ('-1. "" Thblft 3·9'°). AlIo."" ~Iow for mOrl d.t~iI an terio r cerebral It; • AI: ACA f,,:n'U onain to ACoA • A2: ACA rrom ACoA to branch-poinl or wlOllOmargiDal • A.3: from brandl· poi IlL orcaUOIoOtnirain· al to , upenor aurface of ~rpul calloosun'
NEUROSURGERY
"
3 ~r;n posterior to the. genu A4: pl!ri~al1ol8l • A5: t..nninal branch middle cerebrnl'": • 11011: MeA from origin to blfurwtion (horizo ntal segment On AP angiogram) • 11012: MeA from bifun:.!Ition to emergence from Sylvian fill>lure • M3·4 : distal braoch .... • 11015: t..nn;nal branch post..rior cerebral (PC,I t ) (several nomenclature schem .... ""i8:"''' ~ • PI : PCA from thllQrigin l.O post..rior CQmmuni~ating artery (AKA melM!n · cephalic, precommlUlicating. circular, peduncular, ba,,;lar ... ). 1'be long lind .. hon circumflex and tbalamoperforating atteri .... arise. frem PI • P2; PCA from origin of p-comm to the origin of inferior toomporal arterieol (AKA ambient, potitoommWlieating, perime!lencephaUcJ, P2 traverses the nmbient ciBt..rn. Hippocampal, antoorior t..mporal, peduJlcular perforating and medial po.tenor choroidal arterie!'rise from P2 • P3; PCA frem the origin of the inferior temporal branches to the origin of the t..nninal brlU'che" (AKA quadrigeminal ~gmentl. P3 travenlu the quadrigeminal d stem • P4: ~egment BitU the origin oflhe parieto-oecipital and ca lcarine Qrteries. indude~ the cortical branch"" of the PCA
•
•
oplicn. (er N. II)
. I
FIgure '·10 C"tlf-ol WiUis viewed Irom In lronl of nne below 11>8 briM Key po in t: the anterioroe~ral anenes PI'~9 over the supe.rior ," urface of the optic chi asm.
"
3. NeuroanatQmy "lid
phy~jololO'
NEUROSURGERY
ANTERIOR CIRCULATION
INTERNAL CAR OTID (ICA)
Acutely occluding one carotid arr.ecry will eause a stroke in 15·'20'ltofpeople.
Segm e n ts of th e lCA and its branc hes "Caroti d sipho n": begiO!l at the posterior oond of the CaVemoUli ICA. and enda at the ICA bifurcation (thus incorporating the cavernous. ophthalmic and communicating segmentsl" . C I (ce rvic aJ): begins Dt carotid bifurcation. Travels in oarotid sheath with IN and vagus nerve. encircled with postganglionicaympathetic nerves (PGSN). Lies posterior & medial to the external carotid. Ends wheTe it enters carotid canal of ~trou8 bone. No branch~s C2 (petroua): still surrounded by PGSNa. Ends at the posterior edge of the fon· men laoerum (f.Lac) (inferomedial to lheedgeofthe Oso.serian ganglion io Meck. el's cave ). Three legments: A. verticalaegment: ICA ascends then bends as the .. B, posterio;>r loop: anterior tocochlea, bends antem.medially becoming th e ... C. horizontal segment: deep and medial \0 greater and lesser superficial ~tros.ol nerv'ea, anterior \0 tympanic membr/lne (TM ) ClI (Iace rum) : the ICA passes over (but not t hrough) the f·Lac fonning the lateralloop. Ascends in the canalicular portion of the f·Lac tojuxtaBeliar position, piercing the dW'8 as it pasae8 the petrolingualligament \0 become the eavemo\UI segment. Branc h.... (usually not visible angiogrnphicallyl: A carotiwtyropanic (inconsistentl - tympanic cavit y B. pterygoid (vidian) branch: passes through foramen laceTUm, pruent in 30%, may wotinue a~ artery of pte rygoid canal C4 (cavernou s); covaNd by vascular memb.-ane linin, sinus, s!illsurrounded by PGSNs . Passu anteriorly th~n supe~mediaJly, bell<.b J>OI!leriorly (mediall{){)p ofiCAJ. travelshori:tOlltally, and bends antenorly(part of an len o r I{){)p oflCA) to anterior clinoid process. Ends at the pro~mol dural ring (incomplete encircles ICA). Many branchea, main ones indude: A, meningohypophyseal trunk (1argeat & most proximal) 1. A. "ftentorium (AKA arte ry of Bernasconi & Cas6lnari) 2. dOnll!.l meningeal a. 3. inferior hypophyseal s. (- poIItenor lobe of pituitary): occlusion causes pit uitary infarcts in post-partum Sheehan's necrosis, however , OJ is rare because the stalk is spared . B. anterior meningeal a. C. a, to inferior portion of cavernous sinus (present in ~) D. cap$ular aa . of'McConnell (in 30%): supply the capsule of the pituitary" C6 (clinoid ): eods at the distal dural ring (wmpletel)' encirdes ICA) where the ICA becomes intradural C6 (ophtha lmic): begins at distal dural ring, endsjuat proximal to p.comm A. ophthalmic a.: the origin from the ICA is distal to me cavernous sinus in 89%(intracavemous in 8%, absent in 3%"). Pssses through the optic canal into the orbiL Has s characteristic bayonet-like ~nk· 00 lateral angi~ iTam. B. superior hypophyseal a. branches _ anlerior lobe of pi t uitary & stalk (1st branoh ofsupraoiiooid ICA) C. posterior commWlicating a. (p-comm ) I. few anterior thaJaOloperlorat(! .... (- opt ic tfllet, chiasm & po!Iwrior bypothalamus): see Plnttrior t ireulation below D. a.n te rlor c ho roldal a r tery": takeoff2-4 mm distal top-wmm - (variable) portion of optic t r&ct, medial globus pallidus, g~nu of internal capsule a C) (in 50%1, inferior half ofJ>Ol!lerior limb of IC, uncus, retrolenticular fibers (optic rad iation), lateral geniculate i»dy (seepage 778 for occlusion syndromes) I , plexal segment: enters supracomual reteU oflemporal horn, - only this portion of choroid plexus 2, cisternal segment: passe! through CTUtal ciltern C 7 (eommun;ea t ing): begi... just prox.iJnal to p-tomm or igin, tTavels bet"""",n Cr. N, II & Ill. wnninstesjust below anterior perforated l ubstance where it bi. furcates into the ACA & MCA
NEUROSURGERY
3. Neuroonatomy and physiology
"
ANTER IOR CEREBRAL (ACA) r"un between Cr. N II . nd ante rior perfol'llurd 5ubostMce. Su 1'16",..5·2. pap 132 for angiornm lind bran~bBlo. MIDDLE CEREBRAL (MC A)
$eU"lIurt 6 ·3, pag~ 132 fllr a"llogram and branch"'. PO STER IOR CIRCULATION
VeRTEBRAL ARTERY (VAl 'The VA i8 t he first and usually the l.rGut brllncil ortlle.ubel.'·;"n a rt.l!1")'. V.rianl: the len VA Bri., oJT the B()I'tje arch in . ... Oi.mft-olr. 3 101)'1. M"e blood flow _ ISO mllm,n. Thlliell VA i, dominant in 6
PtCA~I'
a'fI _..urior II'IeII1oIi:Ir "9111'" 1m _lateral - . . . ~ !aud.llloop) 1m"~$Ij1m1.'t11
M _ ~ (wpra:onsIIol H9TII!" (~.loop)
ts _ COIticiI
~
FllI"'rw 3-1' lI~aI
Four Mlmen ..., fil"lt legmen!! COUrHt a Upf:riorly and poatenorly and en ...... L'-w fora ruen tron ... venariulll , "lual1y oftheGth vert.l!bl'lLl body a«ond IIII(I'ITIen!! a_ndl vertically with in the tnl nlJVUW fortuninaoftheoervial vetteh,.. . accompanied by a nelwork ofaympat.heu.: fibel"l """m the lteUale gan .
.
NEUROSURGERY
glion) lind II " .. Mug pi"""". It til"", JawraUy will,," the
{rB'llI~ proc: "~
oftl,1!'
~" ~gm~nt: exi t.\. the fOnllnen oftha IU
a
6.
(contain. rot.niPJJ\!:D.lI. on anglo) 5. cortical e~ents
3 branche! I . o boroidul a. (BRANCH I) ~ri S\lli from crenialloop (cboroid!! l PQiruj, _ cboroid !,lexus of 4th ventricle 2. tfrminal branche" B. t onaUlobem.J.pberic (BRANCH 2 ) h. inferior vermian (BRANC H 3) inferior jnfle<:tion ~ ~ JI.!ilill, on Bngio Mteriorapinal
BASILAR ARTERV (B A) Formed by th aj unctiou of~lle 2 yert.ebral ~riea. Bran~h l!ll: I . AlCA: from Lower part ufBA. runS post.erol~Lerally anteriurU) VI, VII & VI II . 0(. ten give! oITo loop !hal runB loto tile lAC and give$ ofT the labyrinthine 1I.l:tery lIod then emerge!! to a,,!,ply tll~ anietoLatera.l i"ferior co.:teb~Hum and then ""811tamoses with [lICA 2. intemal auditory {labyrinthinel 3. IJOOtine branch", 4. .uperior Cf!rebellar a. (SCA) A. J Up. v~rmian 1'> .
pru&t.eriDr " ....... " .... 1: joined by
J>-<'O'T'm~
major origin of the PCA in 15% end ;.
_ 1 ern f...... m o rigin
\.I!rrD~
\ th~
tH-""nTT>m i£
lh~
· feLa]" ~it<:ulatiou, hilateral ill 2%1,
3 ~e,,'-' (named (or sUlTOuoding ciatem.l lind tbeir brnn~be!' A. pedulltultlJ" .egment,(P l) I. meseneepbalic )It' rforatmg Ga . (- teetum, ~erebral pedWlcles. and thl'S~ n"deli: Edinge ... Wes;tphal , oculomotor and tl"l)Chl~a rl 2. int.erpeduncular t bnlamoperforatol'fl (l al of 2 groups e( poIIterior Ihalamoperfor&ting aa.) 3. roedia! post. choroidal ( tn
NEUROSURGERY
3. Neurosnatomy and pby!iology
"
6. .,.learine C, q\,ladrigeminal Sl!(l'ttM!nt (P3) 1, q ... lldrigeminal & genicula~ bnnchu _quadri,cminal plate II post. pariulJoaal('plenia1) (1UlIl8tIlU:lOIu with periclIllOilftl of ACA) E XTERNAL CAROTIO
1. aupermr thyroid' III anterior braneh 2. Alloeooine: pharynruJ liogual ... r..,;a1: bran.ch.. ana,"'~ with ophthalm ;~ Ikwy in rol1ataralllnw l 5. oocipiUll 6. posterior lIuric:lliar 7 . aupcrficia! telJlporal A. rmnlal branch 8. pAJielal hranch 8. maxillary - initially within parotid ,I.nd A. "';ddle meni'IjIar.l 8. IIHIninp.1 C. inferiwal .. toI)lar D. inf'r...".bital E. Dthen: dial.al branch" ofwhich me)' an.. lO_ with brarKh... orDph. thalmlc artery in tlla orbit
a.
_.0.,.
3.4.3.
Cerebral venous anatomy
S UPRATENTORIAL VENOUS SYSTE M
SM FiB"" 5·4, page 133 r",. lltIp:.gr.m .nd braneh ea. The len. end richt intema l JuguJar ... ina (IN.) are the l7Ia,ior -.un:e or outllow 01 blDod from tht: inlr&Crllo;al companment. 111, ri&hl. IJV i. u.uaUyd~minlUlt. Other iOIJrce! or our.f]nw include orbi1.al veill.! lind the vertebral W!nDUS plul!!.. Diploic and &Calp veina Illay act .. collate... 1 pathwayll, 01.1 . with ,uperior ~tu l t iDUM ohatructioo". Th" followlne oUllloe uaces t.I'. e v"noW! drainage back from the UV • . A.. inferior petroaa! Jin ... B. aivrnoid . inuB I. I " perior petrosallin u! 2. traru;ve~ sinw; (R,. L in 65'1» A.. v. ofl,.a bbo. {inferior an astDmDtie v.1 B. ,xmfiuefl5 ef8illllSe~ (IOrcuiar benlphili) 1. 0«!pltaI61 ........ 2. , u perior .agitUll 6 inus g. w. of'Trnlard tsuperior 8na.llwnlIItk ... ): the p ... m'j nen~,uparl'icial vein lID the Illm·dominant!hie l .... bbtll it...",.. prominent on the d(>minlll)t aide) 3. alrllight sinus a. inrerl~r ugittallinu B b. grWot cerebral v. (orOalen) i. p re·cen t. a! cv.,.bel1l1J' ... Ii . bsaal .. ein of Rc8t!nthal iii. intemal cerebral ...: jolnl!
CAVERNOUS SINUS
Althoulb ch'IMicaJ tel:lcb inl depitU: thl! caveruou , ~in"""" allr,e venoua,pfou witb multip ~ trabo:!culatioll8, ll\il!Coou It"dlu" and ,urgical experi~ I IIPPOrl.ll thl ...{ t he caVlmoU' Binu, pll :\uMofvelll8. I. contrib ... tinl! "elnl :
concap~
"
u.
3. Nll'Umana1.om), ind phyRI(>logy
NEUROSURGERY
A. , upol'rior & torerior ophthD.lmic "em.i B. l upemcia.l middle cerebral vBinS C. sphenoparietal ' i-
"0'
D. superior &: inf"'; OT petroSD.1 ainul 2. contents:» OculoUiotu n. (lll)
Trochlen r n. (IV) Ophthllimic divi_ s ion o(trigeminal (VI)
Muil illrydiviaion of trigeminal (V2): the only ne ..... e of tbe CS"erDOUa ai-
'I
nu.th/l.t~.n'l
exilthe skull through the iUpe_ rior orbitallissure (it Hits through Figura 3-12. Righi ca.emous sinus (ooronal sectloo) (Modilie
,_rv-r,
3 .5.
Internal capsule
Vasclilar ,"pply or the internal cap, "le (I e) I. anterior choroidal: - all of ~trolenl.iCtllar part{indudes opti<: radial;on ) and ventral part of poat~rior limb oflC 2. latera l stri ate branebes (AKA capsular branch",) of middle cerebral artery: mMt of anterior AND posterior limbo of lC 3. genu uaually r«eivl!!! some direct branc b"" of the intemel. carotid artery Most Ie lesions are eIIused by vascular e(~identa (thrombol!is or bemorrhage). Table 3-10 Four Tl'Ialamic: "Iubradlallona" (AKA Ihalaml c: peclunc:les) (!abeleo A.0 In F">gUIB3 /3)
-- ...,"'"
..."" ...... ......
RadllUon I~
oentrallh/Jlanie IIIJ- gere,al ~ Ir!lelS I'om boItt
,olandic:a'eEl$
'"
DCcipMl & posIerlor ))lin.
1'1
.~
posIerior
i1leoo,
101
"'"""'"'
ConMCIIon medial & ~le
IllInlMlrstl temponli 1If' rusol Hesctll
NEUROSURGERY
3.
-
'"
& head 10 tefn*lale In posICen-
MOB
(smaU) Ir1c\uOeIl ;JU(jitory radWil -
!IaI~s(OIJeu3,I .2l
CII\II8I )I'Ialamus
N~url)an8tomy
""
and physiology
"'
3.6.
Miscellaneous
OBERSTEINER·REOI.ICH ZONE ( O R Z)
AKA root entry toue. Tr&-'' liition f!'(lm eNS myelin to po!riphera! lI\)'clin of cranial '" area whe~ root ~ntry zone p",ssure fmm inllll~rnniB] sW"ucrurea CIIn caUSftCrtl·
I\I!rv~
nial nerve 3ymptOmt (trigeminal
ne"ralgi~.
hemifl.dol apa3m, disabling pOllitional ver-
tigu, e!.l: ,)p. Also, zone where neopla.m5 U:'nd to occur, l!!lpecililly aal!l.tic neuroma . On Cr. N. VIlI , the ORZ is 8-12 tum dilla! t.o exit point from brai1ll tem, Bad is dose to porot
8custicus (e&peci ally common on ve.tibular division Y" ....I. DENTATE L~MENT The spinal IICtellS(lry nerve is dOrMl to theden t.ate tigument. 1be denLllt.c! ligamenl fi.ep8ratf's do""aJ from ,·.. ntral nerve fQOl.S in !.he spinal ""'rvel.
3.7.
Neurophysiology
3.7.1.
Blood-brain barrier
'!"he paaaage ofwater-lQlubJe substanCl'll from the blood to Ihe CNS is limlted by tight jW1 t1.ionll (um ul" .. ocdud"D~ ) which lire found between cerebral ca pillary eodo1.belia! ""IL., limiti"!! penetration "r the "",,,bra! paraucbyma (b!<>Od·brain barrier, (BBB»), lit well as between choroid pluuB epithelial celL. (blood·CSF' barrier)". A "urn· berofspecialired mediated Lraru;port sysloelOSal]ow trIInlmisshm of, amc ngothcr tbinlJ$, glucose and certain amino acide. (especially preeursOl"li to neurotranamitteTII ). The "ffi""cy of the BBB i. eompr"",ised in ""rta'n p~tbological ~tates (e.g. tumor. infection, lrauma. stroke, hep;atic elll.'I!phaiopathy. .. I, and can al!lO be manipulated pharmacologically (e.g. hypertonic monnllUl increases t he ~rmeahmty ...... her~s sloeroidl! 1'<1duce the penetration oramall hydrophilie molecules). The BBB i~ ab&ent in the roUowinll areas: dlOroid plexus, hyp(lpl\)"IIi" tuber ci. ne reum, srem paatrems, pinn l and preopl ie n!'CeSB.
3.
N~uroonatomy ~nd
phl'llioiOO
NEUROSURGERY
CEREBRAL EDEMA
Three balic types (dif!"u.ion·w.. ight.ed MRJ may be able to dif!"ert>nti.te, Ht JXl6t 136); I. "ytotOld ,,: BBB is closed. the ... f,,", no protein utrav ..... tilln. then!fure nG en·
cr
hanoement on or MRI . Cell ••well then .brink. Seer. e .•. in h u d i.,jury 2. v" lIOg .. ni,,: BBB disrupted . Prol.ein (serum) INk. out of v..cular IYttern. end the ... fon may .. nhllJ\Ceoo imagin,. Extra"",llular'paee fECS) upand •• Ce ll •• re stable. Responds to cortioosl.e...,id. (..... dex.met ........ nel. Seen ' " .lUrTOund inll metastatic brain tUmGr 3. i,..,bemic : . combination of the.~ BBB c\oted initia lly, but th.n may open. ECS shrinkll then upandl. Fluid .x tra vallal.ellate. May aUN d.. l.yed del.e'ioration following inlra"", ... bral hernorrh"'. (_ ~ 8.S$)
3.7.2.
Regional brain syndromes
This section IoervU to brieRy describe typiaol.ynd .... me .nociated with Ieoion. in variou8 areu of the braIn. Unlu. otherwiMl not.ed, Inionl conlidered Ire dtJ!oli:m:... I, r.on t allobo!! A, unilaterlll il\iury: l , may produ"", few clinic.1 findi .... ,x"'pt with very I.rge Inioou 2. bilaur.1 or I..ge unilal.e.,1 lesionl: apathy, abulia 3. the frontal.ye field (fGr contral8l.er.1 gazel dlocated in the poIIl.erio. frontal lobe (8 • • a. ea 8, , hown I I th u triped area in Fitu", 3·J. page 68). Deetructive le,ions impair gaze to thecont •• lal.eral aide (patient lookl .llU!Llllh the .lde of the lu iGn), whereas irritative lesions (i.e. lei · lurn) caule the center to activate, producing contralateral gau (pa. tient looltll ~ )'!'lll. from the side Gfthe luion) . Also IIU pagt 584 B. bi lateral injury: may produce apat hy, abul ia C. Glfactory fioova rtgiGn : may produce Foster· Kennedy syndrome (see bf,low) 0 , ~fronta lobet control "exec utive runction": planning, prioriti:;ng. orga· niling tholl4lhta, suppressing impul8el, understanding the OOflsequencet of dedsionl 2, parietal lobe: major featuretl Cst. pog# 87 for deta il9} A , either ,Ide : conical aenlo,y ' yndrome, sensory extinction, contralate.ral homonymous hemianopia . contralateral neglect B. donei,,"n! parietal lobe luion (len in rn<>St): language disorders (aphasilll), eerttmann', sy ndrome (_ M t 87). bilateral astereognosis C. """.domin,"" parietal lobe lei''''''' topographie raemory Iosa, anosogTlOliia and dressing apra~i. 3. occipiullobc: homonymGul hemianopsia 4, ""'rebellum A. leaiGni of the cerebellar Mmi.pM", cause atuie In the jpsilateuJ l'robs B. letion. o{ the cerebellar vennis cau"" truncal aluia 5. brainstem: usually produces a mixture o{cranial nerve deficilll and long tract findings (1ft Mu.w fo.- some specific brainsl.em syndromes) 8. pineal region A. p.rinaud'. ,yndl"OV'le; 8ft pap 86 FOSTER-KENNEOY SYtJQROME Ul ually from olfactory groove CIt media l third aphenGid wif\i: tumo , (ulua)ly menin_ gioma). Now nlre due to earlier detection by CT KaD. ClaAic triad : 1. ipsil.teral anosmia 2. ~ centra l scotoma {wi th optic I.1aUili:t: due pressure on optic nerve} 3. contralatergl papilledema (from elevated lCP)
O«lIIiooally ipsilateru l proptosis will also OCCur due too.bi",l invuion of tumor.
3.7.2.1 .
Brain stem and related syndromes
W EBER'S SYNDROME Cr. N. III pally with contra later.l Mmipa reti. (a lso 1M LDcu ..o. "fOott" PIIp 716). Third nerve palsin frGm parenchymal leaionl may be ..l.tively pupil .pa rin • .
NEUROSURGERY
"
BENEDIKT'S SYNDROME
Similar to WebB'a, plus red nucleullellioll. Cr. N. 111 pals)'wilb rontrnl8~TlII hemi· Uceplllml which hn hYP" rk.ine8ia , atu.io, and a CO'ndbrlin Io!ymentum i"Y01 ~; nl! ~ !lucie"., brllthium col'\lu llttl.V1,m , /llIIf fllaeitlel oflU. panstS
M II.I..ARD-GU8LER SYNDROME Facial (VI I) " abducenl ]VJ) pal.y . contralat.er~ IM!miplegia ((:(Jr tlcoapinsl lTatt) from Leaio<1 in hue. of po ... (1.IIIIall), iKhmic in faf1:t, oo:euionally tum or). PAR/HAVD's SYNDROME Con~rrence • • a:ommod.tilKl fInd IUpr'lllicla. r upwnd gaze pIIllIy \I .• . U ppl~ pill · a)' wilb nO"",11 .tllponsa to yO!l1.lc.l dol1'l e)'u' wittl lid retmetion (" Pia .. pilla), + lid reo trlKlion .. ".euill' a UD . i.,.o). M-.y ha~e fuctd pupils, dinoo:iated liJht.-ne8r responae, convtrgeme apelm and n),IIUIJIIlUI re lrac.toriu • . Skaw ~.tion mil' be a unil/1teraJ ~arianl. When combined wi !.h downga .. pall)', I'arinllud'aI)'JIdfOUll (P S ) i.lLnllWn 11.' !.he I),od ""..." ollila Syl";lIn Iqued u c:l .
E ti o logi el l. 1IUi.u1 pAHingdirec:tly on quad ripm;nl l plate (1I.g. pineal region tumOrl) 2. eJ~.ted ICP: MICOIIdary to c:ompl'ftSion of~phalic teclum by di\ated~u· prapinul noI'' ', "" . In hyd.rol:tphllllli
Diffe rential diaguo. i. Condition • • rrectine oo:utar mati lit)' thai could fOlmic the u pglal! prills)'of f'S: l. Cuilla.in· Bam 2, m)'ll5th.nia pv15 3. bot'uLism 4. hypothyroidism 5. !.hen ma), t.. gradual benign 1_ OruPi'lI18 with sene_ nee
.,nd.o:ne
3.7.3.
Jugular foramen syndromes
Ccmuntf orjugular ro. al:'lea (JY):
Cr. N. IX. x, Xl , pelTO$ll K\nus, $IgmoId
.l nlll , lOma m~i ngeal branch .. tram the __ ndin l ph8ryngeal and oo:cipiLal aneri~.
Nearby: Cr. N. Xl i p.iI$~ Ihroulh hypoKi08la\ t'III'Ial in the OttIpital ""nd),lll. Theca. oUd IIrt@l}'wi!.hthl!aym· pathelic.pluu. enlerl the carotid canal. See Tobk 3· // for II I UDUna1)' and Fig",. 3 ·14 for a ochemalic diai"UD of denc;\a in various JF synd romes. ~ha
Vernet'a "yn
VlIlaret', .,.ndrome: Postarior 1'1111'11' phl!ryng~a l
"yn d rome.
Agu.. 3·'. ~kllIne
Sc.he1Tllllil; a.~'.mol ~ lor..
mM .... ndromta
tnrough • nerve indIcIIlet a dIfId\) IdW>ed line IncIical.,. ~
.
"WEUROSUROERY
Table 3-11
3.7.4. P{lRIETAL L08EANAT04fY
The parietal lobt: II loelli.ed behlnd tha amU'a l .ulcu., abo¥e the Sylvian fiuure, mel"flnll' posteriorly into tha ~pit.ol lobe (the border on the rDl!dial.urfB("e of brain i. dllfi ned by II line connect ing \lMIl'ariet.o-o«ipitallulcu. ta lhe p...orcipit.ol nOld>l, PARIETAL LOSE' Nf!UF/()PHYS/OI..OOY elth~ r , id.: . ntenor parlet.ol CONK organi,," tactila pre.:ept.s (probably conUlllatera l) and inwt;ra~1!I with vUIII.I.nd IIliditar)' -.nsaOon to bu ild awareness IIf body ond its IlPltial relaLioli' domiNlnt $id. {on l"ttln 97-' of ad u lu }: unds",unding 18ngu11lf, iIIcludl!fl "cros.s. mod.l mntchlnl'" (a uditory· vieu",], vlsual.u.rtik-, ete,). DysphMi.a pres<mt wit h domina.nt lobe lulan. olU:n impedellWlln$men t non.do minant .id~. (r i,hl in m()lll ): inleiTl'~ vi,ual and proprioceptive $\Inliation to allow manipuillion of body and objl!"Ui, and ror e~r\.lli n toOSlroc:UonalllCtiVi-
tiwi CLINICAL SYNOROMES OF PARIETAL LOBE DISEASE
l.
unilotel'lll pruil'tallobt di!M.'aat (domman l OT non -docoill8llt): A. eorllcal HI'ISO r)' lyndrome /~ w lmu) aod HMOry eJ
'p_
NEUROSURGeRY
87
CORTIC AL SENSORV SYNDROME
Letlion ",fpostamtrnl gyT11S, £specially 81"118 that mUJlII 10 hand. llensory defitit ~ A. loss of position ,""nN and of pa!l8lVe mOVemCnl sanae B. inability 10 Io<:ul.i ~ l1Iotile, therm&!, Ilnd noxious stimuli C. ps~reogn06is (I nability to judge object Sil~, shape. and iden t ity by feel) D. agnphesthesi. (carutot interpRt nu mbers ",riLUn on band) E. 10llll o(1:WQ poio.l dilOi!rimination preserYed un9alionl: pain. lOudl, pre!l!lure, vibrst ion, temperatu re other feature!! A. easy fatigsbility of.aen801"y pe=ptiOl:\I B. difficulty distincuiahiog ,imull1lnOl(lu8 atimulanOlls C. prolongstion ofauperfieial pain WiLh hyperpathia D. toueh h 9Uueinaliool
A SOMATAGNOSIAS A NTON· BABINSKI SYNDRQt.fE
Un ilaUiral a!lOlDIIltIgIlosia. May &I!~m more common with non-uomina nt (r ight) pa. rietal luion, ~lIUU it maybe obscured by Ihe 8ph!llli~ that Ottu". ....ith dominant(len) sided l"ioM. 1. 1ltI000000oaia (indiffereoce Or UnaWllTeneSli ofdefioit.s, patient may deny thal paraJyud exlremity il r.l1eiral 2, a p'llhy (indiffe rent" 10 failure ) 3. olloc:heiria (one ... ided 'limuli percl!ived contrelaterally) 4. dre5Oiinl: apl"!ll
APHASIAS
All related to parie13l lobe lesioll!!: 1. Wernicke's . ph .... ;,,; lea;on of \ludita-ry 8I1!111ciaLion 8""111 nr tbeir ,epa rati on from anguJar gyrus am! primary auditory rorte". A.D.llllll aphasia (normal ten· ten~1! length &. Intonatioo, devoid ofmean ing1- May indud .. paraph.a.silll. Lealon in region of Wernicke·, area (Brodmann are~1 40 &. 39. l ee FiC"'" 3· 1, Pl'1:1! 68) 2. Broca'a (motor) aph_i.: in reality, "8praxla" of motor "",uenc;ng for speeoh (Ipeech and phonation muKieR aren"l parll]y~ed, and fundlon for other nd-lVi· t inl. prod ucing faltering, dyeaJ"\h.rit Jpeech . Lesion;n region ofBroea'a ar~ (Brodmann area 44 , ' '''' Fig"N! 3· 1, page 68) 3. globll illpha ,,;n; us\U. lly due to lulon that dellrOYI large port,on oflangual:a tenUlr; aU IiBpects of speech and lanb'Uage affected A. unab!" to apenk "xeept for lIO!tIe tlicho!s. habiu.al phrases. or expletives B. anomia Unnbilit)' to name obje<,:ts Or POI'\.S or otl,iecta) C. verbal and motor peTSeVenl t;on D. unAble to und~'.tlltld an Ut .. pt fo r a r..... wo.d& E. inability to read Or write 4. coaduction \lphasia: tua to disniptiOll o(connections l>etwee.:l fronl1ll and temporal apl!etb areas, usually involving supramarginal gyrus. Si milar to W~mjcke·a U1uen~ spontane0U8 spe~ sod psrsphui,..l. b"'l patielltl! undel"lLllnd spoken or wtitt.m words, and are aware.of the;r deficit. Repetition i9 severely s lTected S. pu.re word bUndnf!S5: AKA Ilh~a ill without ag"I'lIph;1I (rare) due to lesion in pa. rieto-oc:<:ipitallobe thu intf!rrupl.ll conn&etionl bIItween left angula r SY"U and bot h oc:<:ipitallobes. Pntienta Mn write, bUlllJ"ll unable to read what th.ey've written, and freq ueotly uem Wlconco::moo nboUI thii. Often ncwmpanilld by low of ability to name .:(lIon. Reading and naming number. usuall.Y preserYed
3.7.5.
.
AltllQugh
Babinski sign ~b'lltdlld
as the mO!lt f8mou ~ slgn in neurology, there it still di ugTHmI!nl
3. Neuroanatomy and phy!liology
NEURQSURGER1·
over what constlu.lles a notmal rupon"- and ... hcn .bnormlll r1!.!p
'The following
rep~"nu
OC(U~'.
onc inl
pl .. ntar ren ex (PR) (AKA BabiDski aign) i.!I" primitive rene)!., present in inr"ne)" conailting of extenslun of the GTeat lOt! m IUpOll!!<'! to" nCIlI:ioWi ! bl1lulUfll,lpplied to th e foot , The~malll.oell may fan, but W . r. nott'l)ntii~knt no r cHlllully rmpO~nL The PR diaappeanl u8ual1y aL - 10 lDont .... age (rang~ : 6 !liD!! to 12 Y1'S), pres umably UIlder inhibitory control u myelination oftha eNS OOC\1r$, and the IIDI'1'DII] .espanae then eOn verU to plantarl\""ion of the greet toe. An upper motor neuron ruMN) Jellion anywbenl along the pyramidal (eorl ioospinal) Irlltl ITom the mowr strip Wlwn to _lA will ruult in a 108& orinhibition, arK! the PR will be "unmasked" producing u le/ltWJn ofLh .. great toe. With auch IUl U/To(l'Ilealoo, there may also be eX3Jgeration arn.xor ,,),nerK)' n!l!ulting in rlon;ifle..Uon ofth a ankle. and flexlon ofth~ iule8 and hip (AKA tripl e nOlIor ""lIpo ll fe). Th~
Ne uroa n a tom y Th~ affennt limb ofth~ reOex originale! in cu· lAneous rl!Cepto~ restritted to th e first !;acraL dermalome (S1) and travel~ prQx.imal1 y via th~ tibial nl!I'Ve. '!'he ~pin81 cord Hgruenta Invol..oo in the renex-an: lie within Li:li2. 'The efTe:rent limbto the t£Ie exten50111 tnovela via tho !l!!roneal DCal!.
Eti o lo gi ea
Table 3-12 DI!ferentlal d iagno,ls of the PR spinalooro~ies'
cervicll SPinal !l'l)'elopall"t'
6on'l in
Les;OIlS producing '- PR Deed not be ,\ruc!u... l. but may be runctionalllnd rllWl!reible. etiologi~s an! Ij,lA.ld fn Tobi~ 3-12.
wtQ,'aIO! epodulll! t-1«M Ilydllmeraphaly loldo;.melabok COII'IiI
Eli ci ting the PR, and vari a ti o n s
$8Ilures lrau!!llo
Theoptimal stimu h,s consists ohHmulnlion flf the lalera] plalllAr I Urf""", and l,anaverK areh in II henIJp. mIg,;une I llIgle movement IMtlng 5·6 1!IConM". Other means moIOI neurm disuse (ALS) ror applying noxio", atimuli may also elicit the plan... spinIIl CtlId Jr.tu--. 1M FR .... y tar refln (even oUl.8ide L'>e 51 diormatoMe, although ~ I~· t>e _OIl d-.gl/le PlflClCloI these do not produce toe nexion in lIorm.I~I. DI!. $pfI\01"1I\Odo.°I_page ~ scribtld m.neU'·era inclu:le; C ha ddock (&er atch Ih\'laleral fOOl; pGIIitiveln 3'\to wb ..", plantar stimulat ion was negative), Schaeffer (pinch the Aehllles tandon ). Oppenheim (Ilide knuckles down shinJ, COrdOll (momentarily 8Queeu.lower g~stroene.millil). mng (light pinpriciu on darsolateral ~I ), GondaorStr QlUlky (pull thl! 4th or 5th toe down /lnd out a nd allow it to snap back!.
"'"
Hoffmllnn', .iltD MIlY signify a almilar UMN InlnTuplion to the upper extremitl<>&. Eli(;lted by Jna", pillJl thedis!al phalanx cf the Iniddle finge-r; a p8thologicresPOIISllroll5is ~orthumb nexion (may be weskly presenl in nonna ls). Can $Omelimes be see" AS normal in YOWiG individual wilh dilfu'l
ca.
3.7.6.
Bladder neurophysiology
CENTRAL PATHWAYS
The primary
coordjn~ting
eenl.O't ror bladder fundion re-
sides within the nudeu8locu~ coerule u8 of the pons. Thi BOOnIA.lr 8ynchroniu!'I bladde r eontraction with relaxation orthe
urethral sph,lIcle, during voidin, .. VOIW\t8ry cortieal rontrol primarily "wolvei inhibition of the ponline ren~, "lid Otigin81.O'8 in the anternmedial por. FlgrJr. 3-15 LocaliOfl 01 lion "rthe frontal lobes .lId in the genu of the rorpu~ callospinal cord bladoer eN.. • 8Um. In an uninhibitt!d bladder (e.g. infanQyl the pontine voidingcelll.O'T rUllclions wilhuut ""rtiCIII il,lhibi tion !lIld lite detrosor IIl"aderontr.ctswhen lite bladder ru.ch ua critia,,] capaci ty. Voluntary .uppre"i~n from theeol'tel< via the pyl1llOidal tract may r:ontratL tha e~ternal aptlinclerllnd t:lay alIIo inhibit detruwr rontraction . (."orticalle.e.iofUI in this 10eaU"n - urgency ineontinence willt inability to luppr,," th~ micturition renex"".'''IJ. Elfen!llt.i; to Lhe bladd .... trllyel in IhedoNl~1 portian ofth .. !al
3. !'leuroanlllolny gnd phYlliology
"
nal cord (ahaded areas in Figan 3·/5).
MOTOF! There are two aphinctera that pre,·ent the flow of urine from the bladder: internal (a utonomic, involuntllry control), end external (striatOO mu""le, v"luntllry control). Paruymp athe Hca (PS N): the detrusor mo""leofthe b!addercontracts and the in· ternal sphincter relaxes under PSN stimolation. PSN preganglionic cell bodies reside in the intennediolater al grey orspinal cord segmenta ~_ Fibe,... exit e8 ventral nel"l/e root.s and travel via pelvic splanchnic nerves (n ervi e ri&"
SENSORY t...,.S!; well UJlderstood than motor innervation. Bladder wall stretch receptortl sense bladder lilliI'll' lind send afferent signa19 via pelvic, pudendal and hypogastric nerves to spi nal cord llegmentsT10·L2 '" S2·4. Fibers 85C8nd primarily in the spinothalamic traot.
UAJNAAY 8LADOEA DY SFUNCTION
The tenn ne uroge nic bladder describes blAdde r d}'$Function due to leBion~ within thecentrai or peripheral nervous systems. Some use the tenn synonymously with detru· sor arene1<.ia. D0r881 (sensory) root.lll~ions interrupt the afferent limb, producing a n atonic blad· der that fills unti\ dribbling and overflow incontinenC
"
3. Neuroanatomyand physiology
NEUROSURGERY
iI.bmr.e. the 52 ,pinal ~ level. which is ~ T I 2/l..1 ver' tehral body level in an adult): the sacral voiding center is located in the co· nus medullari$. Etiologies: spinal cord injuries (after spinal shock bas 5ub~idedAJ . tumon;. transverse myelitis _Usually develop de!,Cu80chyperre. Jll:xi.lI- involuntary bladder contractions without sensation (autom at ic bla dder). smooth sphincter synergy. but stri ated dyssynergy (involuntary contraction of the extema l sphincter during voiding which produce. a fi.IJ1c· tional outlet ohstruction with poor emptying and h.igh vesical pressures). Bladder fills and emptiea .ponlaneously (or in respoOBe to lower extremity cutaneous s timulation). Bladder compliance is ofu!n reduced. Managed by intenl)ittent catheteri1ations + antichohnergia B. intT8IIIIcraliH io n s (lesion below the S2 spinal cord level): includes injury to conus tneduLlaris, cauda equina or periphe ral nerves (fo rmerly referred to as lower motor neu ron lesions). Etiologies: large HLD. tnuma with com · promiseofapinal caDal. Usually develop detrusor sreflexia. and do not have: involuntary bladdercontractioo8_ Red uced urinal'l flow rateorretentioll re-8ulta. and voluntary voiding may be lost. Over flow incontiDeDce develop6. There may he reduced comphaoce during Iilling. a nd pa ralysis of the smooth sphincte r. Usually associated with lou ofbulbocavemosusand anal wink rene:.: (preserved in 8uprasacral lesions) and perineal sensory l088 inte lTUpti on o£the peripheral .... ne:o arc : may produce disturb.ilnces similar to low spinal coni injury wit h detrusor arefle>
3. 4.
ft.
6.
1. 8.
9.
URINARY RETENTION
Etiologies of urinary reten t ion: 1. bladder outlet obstruction (a briefdiffereDtiaJ diagnosis list is presented here) A. urethral stridu re: reteotion tends to be progressive over time B. prostatic enhu-gement in males: 1. benign prostatic hypertrophy (BPH) & prostate CIInCer: retention tends to be progreSllive ove r time 2. acute prostatitis : onset of retention may be IIlId!I.m 3. rare: extruded prol!ta t ic stone C. women may develop a cystocele whkh cao produce a urethral kink D. rare: uret hral callter 2. detrusor arenexia (u, poe, 90) or hypotonia A. s pinal cord injury B, cauda eqUlJla syndrome (s« page 305) C. chronic infection D. long.tenn bladder c8 theteri~ation E . certain dn.lgs (narcotics. pherlOthia~ioeal F. injury of tbe cauda equina or COIIUS meduLlaris, o. of the spinal cord at or below the sacrom I. tmuma 2. tum<>r 3. myelomeningocele A. durin&: , pin.1 'hook
<- pop 698).
. phin ... , IO
NEUROSURGERY
~ te nli o n
""d ... Ihmc
[~truso, . ~n.,.;. ~
i. Lhe ",10 (urin.ry in
3, Neuroanatomy and phyaiology
"
G. di"betH mellitus (eutoll
URODvN"M/CS Urou.Uy tOmbined wIth :..."" (o;y6to .... lrnIfr.m (CfttG)1 or nUll"" (v id*"u.odynam. ICil. MeMllrel inl ...... aieuJar pruaure. durin, ,..trognd~ blBdd~r mUn, through" urethral catheter. usullLy comhlMd with Iphlnct.l!r electrolD)'OllJ""phy. ~Hnce or ablente td~lru""," .~n.l<.i. ,.u k/.,...)oI"detru"". ,..flu il detected. Ifp.uent. plVtedur. is repeeled. askin, plltient to .u~reSl thl! UTi" to void. Inability to I UPPI"dI i, CIJled "n un · mhibittd detrulO. re.nu IAKA detrulor hyperrenexil • .Ift oboo. SPHINCtER ELECtROMI'OGIIAPH'f (EMG)
Eillw.".. needle electr0Ge6, Or .... ith externaJly lD(>u n~ w rflrotl e:lectrodes. VoIu,,uny .phincter CUnlra~I>Ofl ...... IlIl.aM:tnea ol l uprlUlpinal innervllti(ln. When CClmhin!l
VOIDING CYSTOURETHROGI«'" "NO INTRAVENOUS PYELOGRAPHY (IVP) Void"" c)'ltou.e~luvcrn m (VCVG ) del.e1:t\I u",wlIl paili(ltogy (di vertkula, stric· tUtes ... )' abnonm.li til!l oIbla4der (diverticula, detroSOl' ~tabecul.lio.'1li" Usocillted with ionpu..ding contracl iom apin.t high rN ilt.llnce ... ), Ind vesical-ureteral re!lulI: ,
T REATMENT
Goa.Ia II'f to prt&erve renal funttion (wh'~h lUI ually iovolvilS p,..,veotion ofUTb,"'" filii clIlculi, and ureu.ral renu>; due III high int,..,yesiculu preslUres) and opti millition or utinllry rontinem:e. Palient$ "',minad~uate emptying or incre.sed bladder preuure I,.., often II\3nlged by intermittent c.th.~riutiGIII I nd I1Iticholinergica. Anlicholinerti""'lInd beiulvio ... 1 the..py an used fur patients with mIIintained voluntary blldder emp~y'ng w,th Utlnl!"), frequef1cy or urgency incontinen~.
3.B.
, ,
References
_1<~ TI';",a ''''''' afn. .." ... -.-,..
_..........., ])tSowI~~.~,
0 , . - G. 0,-- J.ldlKlo E. n ... ~..;n' ' - ' '..... Il1<01 .............. _ ............. "'ft","""ltlot _ .. _"'"""'""-....:.~ , . In 1'"'''''''' ) ''''~.I," Sorut.I"'."',..IN 1..."""',.,... """', ..... _ ........
j,....,,""*""' "
...."" ........ ..,-"",-...,._,.,.. " ....... In ... _, . .....
•
,
•.
.. •
("_~ J_-.
76: 101-"10 . 199:WI" .. WD.Gt_.RG ,...""""_ ........ ~ I.MeoIic>oIMMI"CIIIioIoslr ("V_.lI
1....~.),d .d
. ' 91j
pp'91,'.
W.. U;~ R. w ~Iw... ~ L,I" . ~
Grv-o_,.
}S"'..t. WII . S..........,IoIIoIjoIoI. '97)
Ki40D,LlMo1 M. LI.",_" .,• . C~ .... """..... lorolb.oo_oltht _ _ l)"IrO • ••• '"""",,1.J) 11'-T,IUO
M.MI,....Go.n...S.\I,_F .....~ I_""" .
""'''''' .... """", r.OM)oo.oIIo<"'''_ C....
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w._ ........ . ..l1>li_.'''I. ~ 1I'
_
1»M. I"'-1.
I [ C,-·..u.. af~ "'<11, T.
101"...... 11 " •• _"""'S S.(.... ,): ....... -..., ~·IIiI.Nt .. Y.. ~ , t~ .
..--.,w.
lMMLI. K... rL"_"' ... _ _ JooI ............... Mc
_101,..,.... __............. I. . . . " ...v .."" f'Icw 't'-" ''ill' ' -6 roo.. . _ _ l>t'1Ioo
" " "
~
lIP
~"L, .. . ,...~-.Io-
poo.o.< ...
....
_~
~
1'I.. -.,..,4 10s..JIIII~S'.I).' 1'I ,
n_JM . Woocle" , ~
___
ItJ 2flCl0II w _ _ W.l... aoll_. 't'7t. T _ I ~t Stria .... H..... O' ' _MlII"Yoi
NEUROSURGERY
,..."um"...
"
'''"«f''O
g(,,,,,
'1, ,:r...o.
doI._
•
E. "'lI'ab....!chunS'" "c F;I<"" ~lm ~"""Id. Z<.lnlbl "".I'fI<""'l'JOI).
»
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" C
Die
" " " U
"n . ,"C
tII~
l1. 1911. Sot.,l\illiof II ..... I,.c""... H M. Kd" J1' kII· ...... 01 ,II< in.."", , orOl..! ~fJ" A ...... Uh1 H. y..,..,,, MG, R"",,~"' _,,~,",j. tU~M"W/O'" I. 7......, ...... onKler.' "'pblt!lo. ~I;nit. ."d p",". Hobo, r .led.) TIl, .... Y.,IIS.S,""'"'" 1m : ".,*:016
""""" "".,,,,,,,,:,,,l1: E<~ ..
...'...,,_d Clout,
A. il.. _no<,iIorI " I\; '"A.~phlc
Io<.oU .. t~ofiat ..........w ......... ,ClI .."' C. n · """ •• s""",r.. kJ.III ,,,,,, •• ' 9" "".)l , K"I~.t.Ii,., H ,. . y ... ",,11ot G. In C...... "'I •• • "",""~~r O"'" .... """..LDnd<>o.
It<M W H. R"'"" II l.: M.crooIIr£lrnl_r"f ,...... It..-"',;,., J /'<'C\I ....... ~l· lU·". 191) R""""'"L.II. Tb<_ .... Nt-u ..... ••• ,... , " (' S-rplI: SlJ.l20. '.!CIOl. ll
".
" ". N.
'_flf'l II
NEUROSURGERY
_""'I.
J N........"" 7~: 1IJ7.
y .... "') 1t.(N 1 "' ......... &1<..........,.,. ltt
_ ,n.._
Ncu"",~ EII.O"",. P .... MtCormo
.,.,.,.,""i_-".• lb~""",.uMl _ _ "'IkU•• " U.
.. « _,"not IloMl.,a !>n". N"reAI'II<'" 11 .,9·11. "IllS. $ ..... H). lIoI;" H I.. ~ : .... lot H' I.!.,. "" ...... .)'00""". ....,
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vol.,., .... (><»..- ..fu.,,«I'Ob:II .. ....,.,.c.....
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.... 1119' .
~ , .
Gil>o H ,t..."~yC.~"L.. Mo<_~".'",",· "'"r or,1I< .opn< IONHd ~"on of",. ,...mol ...... ........ , ' J N"_"' J"· ~l'.l98l.
....".,
Soklla> I.N· ot>m>"~_"', ,0' ~0>1.3 '''' __ ..... , 1.. ",. 10 To"..,,,. of .h •• r. aial_, I>O •• ,.s........ I./'I ..., S<~_ Y L.I..:I>.I. fIio"," P\d)~""", . M""", Jtn ",~, 1911. ppst~I~ . u..-.~ , F. """"," H' Tht "'''''oj . . 011 or,,,",,,,.· • .,.,." ....... "'iU 'l>"
.U'''' ' r _ N....o!ou
:""..,. Mo;(in.".n,tl. "'~ yert\.. l'ltl MaItu. I C Flo.", ~t.o .......""""'" I. ct..~1r<" .. io~ """". _ """"'" Ic:< ...... olrIi ... l""""""of' t>J" "'."".,'" bia'.l
.(>10'"
• "'OI:Di_ '"
~.
(J,
191.!109.llio6l. Mom' It D. Y'n. M: Pri"'lpI .. oe ........ ,"I1.
"'_0.,11(.,·
w, "' ... J N'"'_<>
."""'"".<, ,.,.,.,,,
~_'. ln
"'ii < "'_,
C_pboO', . _
"11'. w.l>~ P C.~,riIr II B. "'_""" t o . .,oII.• fOll'.I. WO ~.I'b;"",,,lpioio. 1O/1«1 .• IWIi. Y'" !. Cbopl<'" I'l' 9ll·loot;,
3. NIllJroanDt
.''''
)
.....
•
4.1 .
Arachnoid cysts
4.1.1 .
Arachnoid cysts, intracranial
AKA le pto me ninge al cy~te . di stinct frompMllro",.."IU: J~pt.Gmeningeal cyst:! (AKA growing sku ll frfIctW'ft, _pa£~ 668), and "nnlatt:d to i"f
2.
cysts with tnore:complex lining which may slaoOllntain neuroglia, ependyma, and O lh~r
l i,o;su.,
type~
PRESENTATION
MoslAe" thaLbecome symptomatic do so in enrly childhood'. The presentation varies with location of th e ~Ylt. and of'l.entiruu appear mild con sideri ng the large 'Ii ze; of some. Typical prllsent.a-
tions are sMwn in Tllblf "_1" and i ndud ..,
Tabla 4-1 Typical presenlaUona 0 1 araehnold cysta
- --Mldd ll los·
.. ,yt l.
SuprastUar cy5l1 wllh
hycl,ocep!lalul
-, ~ant.l1
headache
h)1le1lftnSior1
hemiparesis developmental CIeIir~ 'iWa11oss
D,l1uaa I Up"'" or Inlra!flllOf1. I I cyJts with hyc!racephalus inUlICIa"lia! hype<1ensroo ganiotr~
~mernaI 08Ia~
I.
~~"'!1<111'~ symptom$ o(;n· t rBerani,,1 hypertension (ehwaLed l e p): H/A, NN,lethargy
2.
eei~urea
3, ludden ddt.(!rioration: A. due to heloorrhage.(into cyst or au bdllul cum Pllrtment): middle fO&" cyst>; are notoriou . for hemorrbagedu!! III t.ear1ng of bridging vejlUl . Solue sparta organj~tion~ do notaUow participation in OIIntact$port5 for thue pat ienUr
B, dlletoruptureoflhecyat at a focal protr"u~;o n aflh., s kull wilh focal BignliaymptOn-.. ofa space occupying le~ion incidental finding di scovered during evaluation for unrelated condition 7, su prasellar cysts mlly additionally prellent with': A. Jl,)'drocephaJIUI (probably dne to compfl'alIion oflhl' third ventricle) B. endocrine sympwma; QCCUN in up 1Il6O%.lncludea precociou./l puberty C. head bobbing (th e t a.ca lled "bobble-bud doH ayndrolue"): con. idered lug. gt!'St.iVl'-o{ , uprll'l4!!i IlT cYlib!. bu t O«UTS ,n aa few at 10'J>
4. o. 6.
4. DevelopmenltlJ anom~Jres
NEUnOSUnGERY
D.
vi.ual lmp&ir!llen~
OISTRIElUTION Almon all OC:CU r in ,ela lion to a n arachnoid cisU!rn {ncept;\ln: iotrasellu, the cmly one that;, (!>i tradural, • • TobI~ 4.2). RetrOCt
Table 4-2 O!stribullon 01 arachnoid cvst.·
<1ft!
EVALUATION Routine evaluation WIth CT Or MRI Is u5ually l at15fao:tory_ Further eval uation with CS F c"ntra~t Olr now 6tudie,
Smo<>th bord ered non-caltilied ext.aparenchymal cyatic Clan wilh densi ty .imilar t.o CSF and no enh.ancemeDt with IV cont,ost Expansion otollllrby bon~ by remodelling is usually ""eD, con finoiog their chron ic ORtUN!_ OlWn lISlIOCiated with V\!otriculoClegaly (in 64% of 6U pl1lU!D t.o ri a llllld 110% ofinfrat.en!.Oria! cyata). Convellity or middle fossa cysl.9 exert mns5 effect on Blljaoonl brain and may com;J Te sslps HaU!rallate ral v"ntride and [)ll u9O! midline shill. Supnuellor. Q:uadrigeminal pl a te. IlIld midline posterior-fossil eyst.s may compra.a thathird li nd founh ventride aod c8 us~ hydrocepha lus by "bstnJcting the foramiDB or MonrOl or lhe Sylvill.n aque-
Typtt l: ~ bioorrve~ kx:;ol
ed in anIIlrior letnpo!ll\ ~. No mass etfeet. Communic::aIIlS "'~h subilrac:ll1Oid Sf*:e on water~DII! conlra>! CT cis· ten\Og'iIII1 (WS
TI'JlII !t i'1'foflof!$pr~at'Id
jrtemmte segrnenls 01 SyMa~ flSSlJ rct. Comptelely opeI'l Jnrdl giVes r~r
5hape.. P,ni,1l COOYIlIJRicaIIOO on WS-CTC
dueL MR/ Br.tler than CT '" different iatinll the CS F contained In 8,..dlnoid ey.;11 from !.h.. tI"'d or ne
wal ls.
CISTERNOGRAMS ANDl'JR IIENTR/CULOGRAMS
Us ing "'i ther iDdinated cont.rast or radion uclide ttacer1 Variable rate of Opaeilic8\.ion W .... ~ulted in diflkulty correltltin g r p3u!ta with operntive findings_ Sollie cy~t3 are I>(:tuo]ly d ive rti.:. u!p. aoo may fiUw lth rad.ia tracer
Type IP~ rr.ot.l!S entire S)1v~
an IissuID. Mallled mdile
shin. Borty e~panslot1 01 mod· die lossa (elevalion o! lesser wing o! spheooId. O'J1WartJ ell' pansion of ~s terlIPGral bone). Minimal communblion on WS{:T C. $Ufglcl.llreatmem usuall'f does not resu~ in IoIaI rtol'~
pension ot brain (may 3:1prOilch type Il te$iOll)
Qrcont,.,.s ~
NEUROSURGERY
"
TREATMENT Mlllty t but n04 a ll ) a .. thon rf\COmmend not trealitll/ aracltnoid Cfst.& thai dOl not tIOUN mlln elTect or symptom" ""lIrdll!M ofthei. ,iu and 10000tion. Sur';..,.) tMalmeRl optiona are iummariU
CYST SHUNTING
Probe.bly Ihe ben O~1'1I1I treatment. For . hunting into peritoneum, """' a ~ I(u......, II ronm....,..t ~.nlrkulomeIt8Ly. on. may 8imult&n~u!ly place" ventocu.l •• Ihuntle.lI'_ I.htoUJb a -r conneccorl. Ultratound. ventricul
nIlS ... I.....
5uPAASElLAR CYSTS TI'1I!8tmenlll propDlled intlude; 1 In!inS(:llIiI)$lJI tyt;~Wrmy "
I"'n:utaneous vlrolricukM;:yuostomy' procedure of eh oiA orPielT8- Kahn et "I.', Puforroed via. paramedian roroaal bUIT hole through U\e IIIUlr.1 ventricle lind fo.amf!n of M ooro (may be r.dlluted by u&ing a venlricul~~) 3. lubf'rontal.pprQoaCh ffor fenennl;on OT R!mo¥/ll~ dongerO<>Ii and indf~I !¥'" 4, • ¥entnculllr d .... in .. I~: inefT«tjv" (aclu;)lI), promoleS C)'lI I en.Iat'Jemen t)
2.
OUTCOME E'I1!n follow.ng IU«esslu! tl'\'/Ilment . portion ofthec)'S( ma)' remain due to the rft. modelinr of the bone Ind chronK ..hilt 01 brain tQIltenu . Hydl"OCl!ph al u8 rna" develop fo4· Iowiug t ...... I~I. Enclocri.nopalhia tomd to pt!BlAllI>'en al\er IlIC"a!Q flll ,."lItm.. II' of '"pn.sellBt C)'116.
4.1.2.
Arachnoid cysts. Intraspinal
4.2.
Intracranial lipomas
I nlTl~nl. lllnd .Mrupol!.allipom" /lR rel~ 1.0 be fir maldevelo;Jfl"lenltil orisin" ,. .... ud mil" anN! from failunt otin¥otlluan oflha pnmilJve mMin".. 'l. Epidemiology ot i.ntra~l"'llJ)!a l lipo m/ls Inddo!nc.t: 81n 10,000 outopei"". U'''11I" round In or near lh.e llliduCl t tllJ pl.~, pIIrticuiorl)' over the corpu'Qlllo.um (I'pomas UI ,hi, ",~n a re frequently • _ _ ted
"
4. De .. ~opmen\llI.llOmll;es
NEUROSURGERY
with »genesis ofthe eorpu.. (lIU""um,~, /Xl&" 114). 1'h('. tuber ti nereullllln.d quad rigem· inal phl teare frequl'ntly affected". Rarely , u.eCP angt,",orc~rehellar ...emlie may bf: involved. May oc:cur in isolation , bu~ also has been dfSCribed in anociatiOIl with anum· bu ofoongenital anomalillS. Includin,: tri$omy 21. Pai's syndrome. frontal encephala· cele. fecial anomalies. ,.. Other midlineabnormolitielS may . lso be found: ogene8is of!.he. tOrpu~ CIIllolJllm, myelomeningocele, and spina bifida" .
I",
E valu a tion May be diagnosed by CT, MRI (study ofchoieel. and by ultrawUJ1d in Infanl8. C'J': Low dene' Iy, rtl6Y hay,", peripheral caJcifie>llion (difficult Lo apprec'iate on M RI J'~_ Oi rrerentisl diagrOllia on CT' primarily between dermo~ cyst, teratoma" and ge rminoma " . MR I: characteristic finding l& a midline lesion with signal ch... raCterlslU:~ orfot (higb int.enaily on Tl.Wl. low inten5ity OIl '1'2\\'1/.
P r p.s e n ta tio n Often discovered incidentally , u lr'ge Lipomas may be assoc:ial.ed with sei~un!8, hy. pothalamic dyarunction, or hydrocephDlu8 (pos6lbly from oomp r1'Ssion of the eq ue
1'reatm ent Direct eorgical approach is Hldom nE'Cll&Sll1')' for il)tlllcr~niallipom.81'·. Shuuting OlDy be required ror caSO!'! where hydrocephalu8 n!sulta fron, obstruction ofCSP circu lation"
4.3.
Hypothalamic hamartomas
Hypothalamic hamartomas 8m rnrll n(Hl-neop!a.tic oong.mitAl DUllfonnDliona <'lll1' .ilit ing ofnuu;ses Ofec:lopic neuronal tissue thot arise rrOOl t he ioferiorhypoUllllamul or luber cinen!uttL Cliniea l rnanl festationfi include: I. spedfic types of&fltures: A. gelll.Stic (laughing) ""zurea: the mOt'll characteril;tic type , Res;$lant \0 med· luI maml,ament and can lead to COf'titive and b~l!.avio.. 1defidl.ll B. 19l.er development of complex pHrtial &f!izures, d rop aIUlckB-, tonic seizures. tonic",lonlc I"'lures. and Sf:<:Ondarily generall.ed liei,un!a 2. behayioral disturbance3 : aggreniYe bfhavior, Toge allacu .. . 3. prec:ociou. puberty 4. menuol teurdation 5. visua! il1'lpairmfO: 'I'wa subtYJIU ofhypol ha18mic hamartom88". 1. pedunculp\.el:l 01' pIlrahypothaJamie: MrT'Ower base al taet.e-d to the floor of the hy· pothalem\l.ll (not Bri8ing wilrun hypothalamu s), Tend to produce precociOO8 po ·
berty more t han they produCIi'-gelaslk sehure.
2. s-esaileor intrathalaml<:: broad atUldllnent to hypOthalar.:ou li. More often associ· alA!d with gelsstie seilUre9
Treatment Treatment .ltern&t'v8ll: 1. medic.] tTeat.men ~ for precocious puberty 2. .Iereotact.ic ..di .... uf'£')1')' 3. ~u tgieol resec:tion Indieolion" fllr surgery: I. precodOUI puberty tIl"t faiia \0 respond 10 meo:!icallhera?y 2. );I!lllfllc .eizo res: no effectiVil med~ll therapy'· 3. neuroll1(ic deflcit from maSlleffect or the tumor SUrgicaloption8: I. ptt>nonal approach 2. tran_lIosal anten or interfomiceal" 3. neuTOt!ndO>lCOpicapproach: difficull because vel1tritl" aM ra rely dilated
NEUROSURGER Y
4. [)"v.. lllpml!flUlI
anlllllDHe~
4.4.
Neurenteric cysts
No uniformly ao;cepl.ed nomendature. Working detinitOo n: . CNSc)'It lined byendo. thelium primarily rtllembl..ina thatoftheGi tract, or le$&ol\en, the rapil1ltory tl'toct. Not true neop{umll. The mostcolDnlon .ltenatt term ill e n ter oge no ul c y a t , with 1,.. oom·
mon
U,>rm:i
including: tel1ll.olllatol.1f; cr-t. intntinoma, ar(:hentenc C)'It". enttl"Ofene
cyst, and endodenllal c)'It. Uwally affect UM upper thoracic and cervical ,pine, a nd other associated developmental vertebral.nomalin .r. commoo", &'-0 oc<:u r Intrac... n;. ally, including 6 reported en" in thi! «rebellopontine angle (CPA)" . S pinal neurenteric cysts (NEe) may h.~ a fistulous or fibrou. connection 1.0 the GI l!'IId and lOOle c.1I t hese e ndod erm a l .inu . eyat t. ' " - are thol1lhl tooccur al. rnu lt ofincompJett developmental separation of the noe.od!ord from the primitive guL 'MI lt is di, tinct from ;"'" !.led intracrani.1 entero&eoou1 q at&..
fool.,.
C lin ical MOII( commonly prueol d",rin, the r\l"5tdecade of Ii,. ... Pain or lQ~lopathy from the intraspinalma.. are the mo&t common presentation. in older children and adulta. Ne'mal.cl and)'Ol.l"l children may preMnt with cudiorupi ratory oompromi.e from an iMrathoracic mas. or cervical Iplna! cord compreMion'". Meningitit may Ottur from the fistulous tTact. upec:iaUy io newbornl and infanta. Hi stology MOit are sim ple c)'t'" lined by cuboidal_ lumnar epithelium and mucin secreting goblet celli. Leu common typet of epithelium described include: stratified sqUll11l0UA and psludostratified columnar, and ciliated epi thelial cells. Mesodennal componenu may be presen~ including I mooth mu.-:le And Adipose tiuue, and some have called these Ulr· stomatou. cy."'''·'' which it not to be confUHd .... ith teratomaa which are tn>e germinal cell neoplum • . Trea tme n t Complete removal of.pina. NECI usually reverses the symptolll8. An aoiherenteap, ule msy prevent completo ~jon of intracnmial lesion&. which p..,wSp0se8 todelayed reaJlT~mce and mandal.cllong.!.e nn follo...··up.
4.5.
Craniofacial development
4.5.1.
Normal development
F O NTANEL LES 6.u.tJtri~_ntJ!Mlli::
the largest fontanelle . Diamond shaped , ~ em CAP) _ 'Hi em (transve ... e) at birth. Nor mally d ose. by age 2.5".., ~~Q[
footwel!e; triangular. Nonnally doses by age 2·3 mos. and mutoid fontandl«: arnall, ilTegular. Normally, forrnercl_. by a~ 2-3 mos. latUlr by age I yr. ~
CRANIAL VAULT
Growth: largely determined by growth orbrain: 9~ oradult head size J. achieved by age 1 yr; 95'lo by age 6 yn. Growth esse ntially ceaaes at age 7 y .... By end of 2nd yr. bonn have interlocked at l utu"" and Further (I"Owth Ottu ... by accretion and absorption . Skull i. unU.minar at birth. DiplOe appear by 4th yr and reach a ,oa.xJmum by age 35 yn (when diploic veilll fonn). Mastoid pl'1Xftl: fonnationcommencu by age 2"... air cell formation ox;cUIli during 6th yr.
.
4. Developmental anomaliu
NEUROSURGERY
4.5.2.
Craniosynostosis
Ori(ina\ly caUed cr.n;Oiteno.i~. InCldf'llce: ~ 0.611000 liO(f: birth •. Pn"lIl1ily' pT. natal derarlOit)', po. Lnatal cranio.ynOlltoaia (CSO) occu"' III><'OJl}monly (postnatal "ause. cone;~t pri n •• rUy orpoilitioD~I . lt. •• lIon. which roay not repre" "l trull l ynClltotitj. CSO ie ,...,,,Iy nlOci,t.d wilh bydl'l'lClphalui {II CP)". n.e I&sftrtion that eso may foUow CSF Ihuntinl for HCP II unpNtVen Iior(' ~ J99). Other calll~ (or f, ilure ornonnalaku U gTowth inc1ud a llickofbr.in growlh dl.le lo AAY orlhe "~III""
orarrelted d8Yelopmf/ltol'the eerebnll
ria, ~e (.II~ea ofhyd'LlItnrtphaly... ).
hltllli , phe~1
Uiu enoa pha ly. micropalm-
'I"r'fatment;. WlUQUy furgiCIIJ. In roost LllllIIncE'll, the mdiar.tion ror 'U rJl!ry .. for (0;1111"11 and ttl p",venl the H~re l"'y1: holoric;alefflct.! orh.""". didipnna d,l'orrni· I)'. HOWl!vtlr, with mult.lple eso, brain Vrowth IN)' ~ im~~ hy th e unyielding .kuU. Abo. fCP may be pIItholoBically eleYlited, lind a lthoulh th1& 1& morealmmonln multiple CSO". el
tit."
r elluj"" theoomblned effort. of. neU(OiIurgtoOn and eraD.iof.cillllurgeon. and /NO)' nead In till ~t8Hed in lOrD" ca_. Ri.u of I Uritry ,"elud.: blood 10M, te\1,,-,,",. I tnlU.
DIAG NOSIS Many caJeiI of -'yoOllw.ia" .... ",Ilty dua to p(NI;tionlll noUe nil\f (e. r . "I.Ey l.tmb· doid". au IH:lowJ. U thit i. '1Upeete\t. inslJ'uet parenU to kttlp need olfo(n"IWied are. and tllcbl!Ck palie al in 6·8 wttlu: ifilwu poiIit.ional, it lhould tA impl1M!d. if II was CSO then il ul uully dk l" ..... ILllelr. The di .......it a f CSO may be . i~ed by . 1. PIIlJaoti<>n of .. bony pl'OminellC1e DYer the IUspected 5ynoatotie autura\UOIption. lambdoidall)'1l(lltDllil,.t b./ow) 2. I':~ntlfl finn pr~u~ ... IUl tha thumb. Wl.t to I'\!1JU;Y8 moye~nt of the bones on either lide of the luture 3 plaia sku\! x·ray.: A. laek ofnBI'"/I'UIJ Juoen l;)' In centet"ohut"l"e. Someee_ with oonna l x· ....y"". pearance of the ""tu", (even 00 CTt "'B)' be due \(J fOUl ! bon)' .picule ronn .. tion"" B. boos ten coppt!r calvaria 1_ ~~ 101), sutural d'liSllIlht snd .."",iOll ofth", Hils maybe seen in cases ofinc",8IIed ICE-t CT_n: 1<" hel .. demanal.raLe c:t'onlal rootour B. ros)' .haw ~hickening andlor rid~ng st the aiLe of,.,..,_i. C. ....11 demo .. alret.e hydrooephJ ... if preseIU D. nuoy . h_ expuaion of lh. rl'Onl.ai l ubaraclmoid sp __ E. ~hree-dim~Ii~ CT may help be~ter vi .... liu aboormalities 6. ;n queationsbJe ......... a technetium bon .. Ile8n tIlIl be p:-rformed"': the", it liWe ilolOpe uptllke byan)' ofthec:t'l ni el autu,," in the til'llt we;!Q ot"Jife • in prematurely clo-.nrsutures, intruHd aetiYJt1 almpam to the oth~ (normal) . uturel ...ilI be demonll:tnt.ed in compl ..tely cI~ IU~U""" , no uptaka will be de",orun.T~ted 6. MRI: ul ua Uy ...........ed ftI, ~ "'Ilh auoria ted inU'kl'\lni.J e bnllnnllolit;eli.. <men
ell,,'"
7.
not " I helpful .. CT measlltementa, ! ucb aaocc:,pilO-fnlnUo I-ci",umCe",1lOI! mII)'.wlJ, be sbnol'mlleve n In the fa«. of .. deformed I kuU . hape
locrep,aed l e p Evidenot ofi~aaed Ie? in the n....·born with cranio.ynOlUllis includ
-t o 1I..... lopment.al delay
NEUROSURGeRY
-to Oevelopment.al s nomahe.
.
T YPES OF C RANIOSYNOSTOSIS
SAGITT,u, SYNOSTOSIS
The mo.t common CSO affeeting a " nl/le luture; 80% male. Heaulu in dollchoce pb a ly or lICa phoce ph a ly (boat l haped I kull) with fron .... 1 bnuinll' prominent 0cciput, p'1p'bI. keel-lib IJIlgi Ual ridlla . OPC remains close 1.0 norma l, but the bip'rie\.al diaroeter Ui markedly ..dUM. Surgicall r ea tm e nt Skin inei.ion rolY b. longi tudinal or tralllverM. A linu r "ltri p" craniectomy i. per· formed. excisi ng the .agittal J uLure from the coronal to the l&IUbdoid l uture. preferably within the fiMlt 3·6 montlu of li fe. 'l'he wid th of th e "rip should bea t l.attS em, no proOf exi'l$ that interpo.i ng artificial aublltaneu (e.lI. silauic sheeting (IV' r the exposed edg.. of the p'rietal bone) retarda the recurrenceor .yno.~i •. Great care i. \.aken toavoid durallaceration with potenti al itijury to the underlying , upenor ••Kitt..l . in ..... The chi ld i. follo,,·ed and reoperated if fusion ree "MI before 6 montlu lIe. After . I YT 'ge, more extensive crenial remode llinll i. ulually required . CORONAL SYNOSTOSIS Accounts for 189lt ofCSO, roore common in feroal ... In Crou U)n'. I yndrom e th;. is accompanied by abnormalitie-$ of , phel\(lid, orbi\.aland facit l bona (llypopJu;' tlimid. face ), and in Apert'•• yndrome i. accompanied by .yrw:iactyty-. Unil ateral coronal
eso
- pla gioceph81y with forehead on affeeted .ide nattened Or concave above ~e(narmal side fa lsely appesMl to bulge abnormally), luprao(l.bital margin higher than norma l .ide ((III skull x-ray - h8rl equin eye l ilt'l). The orbit rotates out On the abnormal l ide, ond can produce omblyopia. Wi thou t treatmeot, flattened cheeks develop and \.he no. devi_ ates to the normal side ( mot of nOM tend . to rotate t.oward s deformity). Bilateral coroaal eso (usually io craniofacial d)'lmorphi l m with mul t iple lut ure CSO, e.g. Apert's) - brachycephal y with broad , nattened forehe"d (acrocepbaly). When combiooo wilh p~malUre d08L1A offrootosphenoidlland front.oethmoidal l utLlrel, resu lts in foreshortened anterior f0618 with maxillary hypoplatill , . hallow orbits, progN$Si,-e proptosis. Surgiealtreatment Simple strip craniect.oml oflhe involved sutUA has been used, often with excellent cosmetic """It. HoweYer, some argument that this may nol be adequlte hili been presented. Therefore, a moreculTf!nt recommendation is todofrontal
METOPIC SYNOSTOSIS At birth , the frontal bone consistaoftwo halvH sepa rated by the froo\.al or metopic lu t" re. Abnormal dosure result.a in II pointed forehead .... ith a midline ridge (trigonocephaly). Many of these have" 19p chromosome abnonn.ality and are retarded. LAMBOOIO SYNOSTOSIS
Epid e miol ogy Lons con.ider«la clinical rarity wilh " reported incidence of 1·9'/lo ofCSO'". re<:ent reportll IUllgest. hisher ineiderw::a of IO.~ wbich may be due to an actual increased incidence, or . imply \.0 increased aWareness orchangi"ll diagnostic t riteria. More common in males (male,fema le. 4 ;1), and the riaht'ide" involved in 7~ofcasn. Usually present.s between S· 18 monlhl of age, but .... y b. HIIIal early .. 1-2 mooths of .ge. Controve,..y emu realtdiRJ the criteria for Ih;' Qlndit ion, and !tOme autMMI differeu u . te bet ween those C88n whid> appe.r to have a primary abnormality of the lambdoid lUtu re from !hOM which rna, be due to .,..itional flattenin«, the ....called "lazy 18mbdoid". Othe.. do lIot make thi. distinction. and tometime. refer to the condition as occipital pl"gioctcphaly to avoid the need to implicete abnonnalilies of lhllambdoid l uture. Po.i tional na ttening (0. molding) may be produoed by; I . deereated mobility; patients wMconltanUy Ii. lupine with the head 1.0 Ihe lame lide, cemral pally, men\.al retarda tion. prematurity, chronic illn.... 2. abnormal postures: ~~I, Qln,enital dillOrde .. Of l he c:ervi~a l , pine 3. int.cntional poIitioni,.: trend , ince 1992 to place newborn, in a lupine sleeping position to ~u<:e the rill. orsudden infant death Iyndrome (SIOS )", IOmelimes
e.,.
".
4. Developmental anomalies
NEUROSURGERY
4.
with II fwom wedge toG tilt Lhe child to ,,"" .id.. to red",,", Ihe risk offUlpirati"n mtraul .. rine etillbglu": intrllutennllcrowding (e.g. {rom multiparous birth. or
ll1rge futalsizel. uterine !IJlomaJiell Clinical fiudiDglI Flattening of the oo:ipu~ May be unilateral or bil!lter~J . If llnilllteral, It Is !lOmetilll'" termed laro bdold ph.gi ocephflly which when severillailo produces bulgingoft.h ij>l:lillll.eraJ fo~he"d rtIIIuh.ing;n II "rhomboid" .kll il with !.he ipSlli.to'rai .,u located anterior and inferior toG the contralateral ear. TheoontTalateral orbi~ and forehud may also be flattened. Th;, may be confll$M with hemifatial mitrosomia or with plagiocephaly ""en in unilatolul oorona1 craniosYt\06toois. Bilate ra,llambdoid synostosi. produces brllchycepha ly with bilth eur.disploced antt'riorly 8IId inferiorly"'. Unlike \he palpable rid,gt of ugittal Or oonm91 Kyn""wsis. on W4~ may be palpated along Ole synos' totic lll.mbdoid l ulure (although II peri,u tural ridge may ~ round in alllIle). DLagnos tic evalu a ti o D The phy8ieal exllm illile mOBl impDrtlln~aB~Lofdiagn09i~ . SkIlUx'·rIlY mAy help diff~rlntiate (8<>. below). Ifth e IkuU x.ray ill equivocal. preven t the infant from layin;g on the .ffetted $ide rOT nvua] wl'ekll. ~ bons &tan Bhould be oblained. ifno improvement OCCUT'll (8« below). In definil.t ca5e$ ofsyn(lftt<>sis, and for SOme taSlU of refradory posi· tioll.ai flattening \ ... hi~h usually (OlTt'cr. with time. bu t may l.ake up to 2 yea..,,! 5llrgi~ol lr'~Htment /WIy ~ indicated .
Skull ,.·ray : ShOWIl 8 ""lerotic luarg;n along one edge orth .. la..rubdoid 8",t"'"' in 71)~ of ealle&, Local "beaten copper cranium" (BCC) occuion8Uy may be 6e'en due \0 indenta· u.;.nll in llIe bone from underlying gyri .... hich may be due to locally increased ICP. Bce producell a characteristic mottled appearance oftbe bone with lucendea orurying depth having ro",nd a nd poorly mllrginated ed8"l!! . BCC correlole:lwitb generalized, ICP only when it ia seen wllh sellar erosioll and sutural diastasis.. , c r .can, Bone windo ws may show eroded or thllllled Inner table In the occipital region in li)·2~ ofcollell"', > 95~ a", DO the side of the Involvement. The lIuture moy appear closed . Brain w;lIdow" 5how parenchymal bnti.n abnormalilies in" 2%: hel.erotopiaa, hy· droci!phalus. ag~nuiB oflhe corpus c.alIosum ; but _ 70~ will ha~.. significant ex pfUl~ion oflh., frontal " ubarochnoid l pace (mllY beaeen in syno.slOllis of other sutllres.~u above).
Bon.. scan : loot.ope uptake in the IllJ'IIbdoid 8uture itu!relUlell d uring the Ii",t year, wilh a peak IIt3 month" of ~ (follow;"1: the u.~u31 ioact;vi~y of the. fin;l w",kli uf life). TIuI finding. with "YTI06tosil.Rre those typ;ta] for CSO (_ fJ'J#1~ 99). 1TeRtmeot Ea rly sur gical t,.atment iI indicated 10 cases with 5evere.,:ranio!""cial d;sfill" rementor those WIth ~videllce ofi.ncreased ICP. Otherwise, cltildre,n may be matUlged nOn· ~u rgl~llUy for 3·6 month •. Tbe mll,jority of CalleS wiU .... maln stotie or will improve with time aDd limpl e nonsurgical ;nte .... ~Dlio". App roximately 15')& will oontinue wdevelop 8 significo.nt C
NDnaurgieaL mana.""mentM: Although ;n.ptovemenl ean usua)!y be attained, some degree of permanent didig. urement ii frequent. Rl!pllllilioning ""ill be effective to - 85% orciI.5I!i. Patienu; are plaeed OR theunaff~t ed sideD. on the abdom~n. Infanta wi t h occipita l fi;
NEUROSURGERY
4.
Developm~Dtal
anomaliee
'"
>II
often ""fIuirnd.
M UL T/PLE SYNOSTOSES Fu~ion of man)' Dr all cr8 ni81.IltuR~ - oEyu pb • •)' {to w..- stui.! wilh ainusI!II8Dd lh.lJ.".. (lrbibl).1'hese patienUi haye ~1e'<8ted ICP.
u"d~e'oped
CRANIOFACW. DYSMORPHIC SYNDROMES Over 00 ayndromell have been de!oCl"ibed, 1'obk 4-4, snows a few ~Iec!.ood Dlle!!. A number of ~r8niD6ynmt03il syud roIllBS are due to mutation. in the FGFR (fibro· bJ~8t growth faclor receptor) B"l!lIeII. FGFR gane .related cranio~yno~t.oIIis syndrom"'! in. elude l ome duaic synrlrOmell (Apert. CroUlon, Pfei ffer .•. ) lit" well as severn! newer enti t ies {Be3N>-Ste¥!MOn, Muenke, Jaclu!on·Wei!lS ayndromell}. All exhibit autoBOm31 do.uinlllll inheritance.
Tablu 4-4 Selecte-d cranlolaelltl dysmorphlc: syndrome. (modifiedf! '.'.....~
, ,
,
., abb,..,..,,!OnS: AO ., 1N1OSaTIaI~: FG/'~ ... bIobII~ grOWIi> 1at:lGf' r~ cso .. ~lOOIs: ~ ~ rlyl!f'OCOIl/UI"" ; UE "' ~PJ>OI a.
4.5.3.
~ed :
Encepha locele
Crsnium hifidum 18' defect i.o the ru~ion of the c:ranin! bone, j~ OttW"'ll in the mid· line, IUld ilmOll! (om man in th e occipital region . Ifmeuingelloo CSf' hern iate tllrough the defect, it i$ called I mening~le. Umenmgt'sand cer ebMlI tissue protrude. it (seni le!! an encephalllOllle. E ncephalocele AKA c:e pbahx,ele ;s an extenS;(lfL ofintracran' alatrllClUNII DI.ltllide of the normal coufiue! of the sltu ll. One C8/i1! wlU seen for eyery fi ve C/l\le.t ofspinal r:oy...[omen'Iij!oceles". A Ilasal polypoid mru;s in .. ~bolm should be C(lnaidered an en· e.!phaklcele until proven otherwise. See al&9 Differentio/ diag'lCfis. J>9ge 936
CLASSIFICATION
Systt!m baaed on Su .... n"'el. and Suwanwela1'O: 1. occipital: often involvu v8lleular structu res 2. c ranial vault: compri!!!!~ _ 80% of ene.!phalocelea in Western hemi sphe re A. int.erfrontaJ n. .nterior fontanelle C. interparietal: often involves vasculllJ" ~Iruetures D. tempo rn! E. po5t.erior fontap!!.lle 3. fronto-etbmoidll J, AKA $lncipital ; IS'N of encepbalOCftles, exterolll opening inlo face in one oftha following 3 region •• A. na,ofrontal: ex t;!ma.l def~ i.n the nll",on B. nQllO-ethmoidal: defect between lIasal bone lind nanl cartilage C. naB
4. Oevelopm!'t\tal a non,ali.,.
NEUROSURGERY
orbital fissure 5. po,terior fossa : WluBlly oontllins cerebellar tissue and ventricular component BASAL ENCEPHALOCELE
i'heonly group thatdoes not produ~ a visiblesof\ tissue ma8.ll. May present as CSF leak or recurrent meningitis. May be aS$OCiated with other erB niofadal deformities, in· duding: den lip, bifid nose, optic-narve dysplasia, coloboma and micropllthalmia, bypothalamic·pituitary dysfunction. In iencepbaly is cllaracterizW by defe<:ts around tile foramen magnum, rachiscllis is and retrocollis. Mo.st are stillborn , $Orne survi ve up to age 17.
ETIOLOGY
Two main theories: 1. arrested clo.sure of normal confi ning tissue sJlow, ll emiation tllrougll per.iatent defeo:t 2. early outgrowth of neural tissue prevents normal c")!Iure of cranial covering!!
TREATM Etrr
Occipital e ncepbaloce le Surgical excision of the 88e and its contents ""ith water-tight dur..! do.sure. It must be kept in mind thst vascular structures are often included in tile sac. Hydrocephalus is of\en presen t and may n
OUTCOME
Occipital ence pha locele The prognosis is better in occipital meningocele tllan in encepllalocele, The prognosis is worae if a significant amount cerebrel tissue is present in the sac, if the ventricle! extend into tile sac. or if tIlere is hydrocepllal us. Less tllan ~ 5%ofinfan~ with encephalooele develop normally.
4.6.
Chiari malformation
The term "Clliari malformation" (after pa. thologiBt, H.. ". ChiDn) i . preferred for tYPn . The Cb.iari malfor· mations consista offou r type!t ofllindbrain abnor· malities, probably Uilrelate
Table 4-5 Comparisons 01 Chlarllype 1 and 2 a nomalies
(,..
1~J.'''''''''
4. DeV1':lopmentalenomalies
'03
TypE. 1 CH1ARl MALFORMATION
t K..y fealllrejl:
• a het..rugeneou~ entity with th"l:Ummo" future of impaired CSF circulation through the fe".men magnulu a:rebeUar tunsillar w rniation: varieble, '" 5 rom below the f(>~men magnum is common. but i9 "at .. _ ntial nor d'agnOiltic ofth" co"dition t ....... tment, wh .. n ind icated , 19 ONrl(ical, bu~ aspeel8 or what tha t 8urgery IIhould e ntail areCQntroversial (enlB r~men t offonunen I1l.IIgIlum is Ul!ually In"oJved) • auociated with syrinsomyelia in 30-70% ",hieh almost 1I1",ay. improvell ",jth treatment of th a Chiari malformation AKA primarycen!bell~r ec t.Opia" , AKA odultChiari mnlfonnatlon (.ince-it tenda t.O be diagno&ed in the 2nd o r 3ni d''''''lde ofHfe j. A heterogenooWl grou;) ofrondiclons, with the u[\de.lyil\& eommoml]ily of disruption of normal CSF now throu!I"h the foramen mlllr· nurn (FM) . Sam.. ~.a."" Are ronB"olnital. but othef"8 are oClQuired. Clall.!!icII.llyd ... etibed a. a r8r .. . bnol"1l'l~lity restricted tu noudal dUiplacernent ofcerebeJlum with t.onsillar hemiation be low the foramen magnum (_ MRI belnw fur enl,,· na )and "~g-liJu, elnngation "F ton,it.". UnH.lr.r.Chlari type 2. the mooulla i~ Il.IIl. caudaUy di . plllUd (some lIu1ho., dillllgTe"'!)n thlS paillto/) , the brainltem is lI!)t invDlved. lower crll.Oial n erves.re II"t elollpted. lind uppe~ ceroiClitI li e""'" d!) not (ouI"Hcephlila d, Sy· ringamyeliD" of the apin al cord il pnl.ent in 3O -7Q'K.... Hydrotephah,s Oc:eu rs in 7·9% (lI
"r
EP,DEMIOLOGY
Ave, age agio: at prellenlatiM ie "1 yell"l I ..... oge: 12.1a yn). Slight female preponde .... anctl (female:mllie a 1.3:l t Average duration of.ympt.ows clearly rell'ltcd 1(1 Chlan malformalin" is :t t)'l"$ Irange: t mMth_20 )ITs); if non~pec:i fic eompJair,t/i, e.g. lilA , a re induded, this bec:nmes 7.3 ye/ml". Thi. lateCley is probably low~r i~ the MRI ef$..
CLINICAL Par.ient.6 with Chia ri type t malfonnatic)Il /lUI}· present due tu;my o r aU " f the fol-
lowing: I. oompreasion " f brain stem "I the level of the ftooraroen mllgnum 2, hydrocephalus A. True hydnom.y. l", probabl,y a_n'l O
.
""Ity ""tp"nibl. III find ....,.,un;........ bctw .... tl,~
, """
.,riA. ond
4. Oevel"pmental ano'nalin
1h~ ""nl .. I<.l1.O l lnChl.ri
I ""-
NEUROSURGERY
3 4.
5.
syri ngomyelia isolation ofthl! intracra nial PT1!5SUN! eompartmen! from the spina l CODlpartment causing transient elevations of intra crania 1 pressure
15-30% of patient.. with adultChiari mal. formation lIre asymptomstit""
SYMPTOMS
The most common sym ptom is pain (69%), especially headache which is usuatiy felt in the suboxcipital regilm (ut' Tobie 4-6). HlA are often brought on by neck e ~ t.ension Or valsalva maneuver. Weakne$$ is also prominent, espe<.:ialJy unilat-
Table 4-6 Presenting IXIIUllllIDl In CIII, nl mallolmllllOn
.,
(7 1 cases")
ned! (wboceipilal. ctrvica~
~"
eral gt'lIsp. U'ermitt.e's sign may nlsoocC'U t . L
See Ta.ble ;ng of signs":
".7.Three main patterns orc1ust.e r·
1.
fqrllmen mamwn...CSlluPrtSsjoD "xndrpme (22%): ataxia, corticospinal lind sensory deficits, cerebellar signs. lower cranial nerve pals; .... 37% have severe HlA
2.
~~~ ( 65%): diftsocia!.ed
!lens!)!), 105$ (lOllS of pain & t..>,mperature eentlltion with preserved touch &'JPS), 0<:caaionaleegmental weaknes9, a nd lOll!! tract signs (.yrilljl:Olllye lic sYlldrome"). 11% have lower cranial nerve pal.9ies 3. ~e(l!be!!ar 1Iv.ndt!wl£ (11%): trunCliI and limb al.a.ll.ia, nystagmus, dysarthria Downbeat lIystagmus is considered II charact4l ristic of this coooitioll. 10% will have a normal neurologic e~lIm wi l h occipital WA as their only complaint. Som e patients may pre.ent prillUlrily wi th spasticity,
lalnting
Iada! numbness
.,
Table 4-7 Preaentlng IilulI In Cillarl l ma lformallon
NATURAL HISTORY The natural history ia not known with certainty (ollly 2 reports on "natunlJ history"). A patient may remain stable for years, witb intermitt.ent period s of deterioration. Rarely, spontaneous improvement may OCcur Cdebat.-
od'.
E VALUATION
Plain x_rays Of10 skull x-ra}'ll, only 36% were a bnormal (26% s howed basilar impression, 7% platybasin, and 1 patient each with Paget's and concave clivus): in 60 C·Bpine x-rnya, 35% were abnormal (including assimilation of at.las, widened calla !. cervical fusions, agenesi s of posterior arch of aUMl.
MRI
d.~ -..beal ~1agmuIl oo...-..c:al _men~ and ftllalory nysIagm ... on horl· zenlal movemenI: no o.:ludes osellop$ia""
Diagnostic ",st of choice. Ea.sily shows many of the d al!9ic abnormalities described <1aflier. including tonsillar herniation, u well as hydrosyringomyelia which oceUrli ill 20-30% of Ca8U. AJ !IO demo!'lStrates venITal
NEUROSURGERY
4. Developmental anomalies
"s
brain nem eompl'
I.....'"
1:"
ClIiIIrI m.lronzaalicm IIQd
.
•
In itially." Ii DUll wIIs ddined 8lI dearly pathologic:"'lwith 3-6 mm heincbordt'rlina). Bark.ovieh" found t.<>n$illar positions 88 Bho .... n inTabl~ 4·8. and Tabl~ 4 ·9 ehow. th e ef. feet of utililing 2 Vt. 3 mm (Ii the lowe$t lIo rmal position . Table 4-9 Cri lerla lor
Chlarll ~
Crileri.lor Iownt Sensitivity Speclllcity e~t 01 tanslls at> lor Cll\lrli IorC\'ltlrl I cepled II norlll!ll 21T)1'T1 below FM t OO'l\o ~.5" ~ 0<1 ""' ..... 'emo~lS In 2(0 !III"I1a1s i
C/Ii/Id I pallenlilaken In "'11100 !D .,... Iow
The lnnsih. normally u<:end .... ith age" u s hown in Tabl~4· JO.
P.tUml.s with syrj ngohydl'Qnlyelia without hindbrain h erniation that responded til p"fos~s decomprj>osion have been deKribed~ (aGoC!l ll ed · Chi"rl zero Il1l1lrormatlon"l. Conversely, 14% of patienl.s with tonsillllr hernilltion " Ii mID al'flllsyrnplomati c'" fa verage utent of l!C\.OpiR in this group .... as 11.4,. 4.86 mm). POlentially moruigoificanl than the absolute t onail· lar desce nt i~ Ihe aU1Ou,n.l. or<:oropreuion ot the brainSleJll 81 the PM, best appreciated en axial T2Wl MRllllough t.l1e FM. Cnmplete.obliteration or CS I' Hignal llnd com pre56ion oflhe brainSlem at th e I'M by impeeted tonsil, it e (ommQll .'gtU!icanl r,nding. Cine. MRl : May oerooOSt rllte blodr.age orCS I' flo .... at PM .
....
Table 4-1D Tonaillar debelow FM related 10
s~nt
'
s.o. • _,d
"""'a\lon.
DIIsoonI:>2 S O. beyond
I..... !of loelilJ. , e<:Io\liIl
Myelogr8p hy Only6'Jb false nega tive. Muit 1'\111 dye all t ile way up 10 the foramen magnum .
CT CT baS dimwl ty eVlllu a t ingthe fom men mQgIlum region dueto bony artrfad. When combined .... ith intnllheca l ,..a\Jlr·IQluble contras t (myelogt'tl m). rehahility improveB. Filldings: tonsiUard .... cellt ""d/or ve ntricular dilata t ion .
T REATM£NT
Ind iclil lions tor lIurgery Since patientll respond best wilen opl!I'Hted on within 2 yea~ of the on.q el or~)'l1'Ipo tornli (!lee Operoliw f«U/IS bela....}. early a urgery ie reeonlmended for symptomatk pa· tien (4. A!;ymptomatie pa t ,enlll may be followed and operat.ed UpOn iraod wll~ they be<:ooIe s)'II'ptom alie. P8tieals .... ho hsVII be@nsymptoma tieandst.t.hle for y
Surgical techniq u ell The mllSL frequently pe:rformed operation is poaterior fOSlln decomp",,,,,ion (su bocd pitlll er..rue<:tomyJ, with or without other proced ures (uluaUy combined w i~h duul patch (l'aft inc aod ee rv icallaminect4my of Cl, soDletiml>fi to C2 or C3l. Some lIuthoT& advocate performing 8 tra"'30ra1 c1ivus·odon\.Oid r esection in eaSl'S wit b v@O lralbrain ·.temoompreBsioll.fiBtheyfeel thesepaLient . may pot.entia lly deleri . orutewith p
'"
NEUROSURGERY
UlrOor fossa derompression . OPERA TIVE FINDINGS
5ff Table 4·// . Tonsillar herniation is present in all eases (by definition); the most commo;>o position being atCt ( 6~) . Fibrous adhes ions between dura, arachnoid s nd tonails with occlusion of forami _ na ofLUlIChka snd Magendie in 41%. The toMils sepa rated easily in 40%.
Table 4-11
r
In Chlarl I
I I below loramen magnum
"
"
SURGICAL COMPLICATIONS
CO
After suboccipital craniectomy plus Cl·Slaminectomy in 71 patients, with dursl patch grat\iog in 69, One death due to s leep apnea oceurTed 36 hra post-op. Respiratory depression was the most COmmon post..,p eomplicetion (in 10 pa· tients ), ..... u.al1y withi n 5 days. mostly at night. Clou respiratory monitoring is therefore rerommended". Other r isks of the procedure indude: CSF leak. hern iation of eerebellar hemisphe res. vascular illiuries (to PICA ... ).
unspeciIied level
~"
inverted foramen m8ll"1lm keel rJ bone
Cl aret! alresia
.tIn"'....
vncular abnOrmal ..... P ICA d~ate1, ",ICA ollen deSOllnel' 1 0 _ margin or ""'$IIs"I: lor"" 11 ..... 1","""U, Iolta in 3
OPERATIVE RESULTS
See Table 4 ·12. PalLante with. pre·op romplain ts of paio geoarally respond well (Q s urge ry. Wea kness is less responsive 10 Sur· gery, elIpecially .... hen musde atrophy;s pre!lent". Sensation roay improve when th e posterior columns are unaffected and the defieit is due to s pi notha la Ulic involvement alone. Rhoton f""ls that the main benefIt DrOperation is to alTest progression. The moat favorable results OCCUlTed in patients with ureb<>llar syodrome (87% showing improvement. no la~ detf!rioration). Factors that colTelate ""jIb a worse outcom e are the presenc.e of atrophy, ataxia, scoliosis, .",rJ ¥Y"'I'wrn» 1~¥t;nJj IOll ger th"n 2 ye~,..,," .
Table 4-12 Long-term follow-up aner
surg ery lor CIIlarl 1 malformation (69 patients, 4 ~ea(S mean IQj!ow-uP")
lI1ese parionl6 dflhtriolah'
(none dele.."..lod 10000hllr) witt*l2-3 yews or S
_In
21 % ... In cenltal 00td IO)Indrotnfl
TYPE 2 (ARNOLO}-CHIARI MALFORMATION
Usually associated with myelomeningocele
CMM"!. Or rarely spina bifi ds occulta.
PATHOPHYSIOLOGY
Probably does ~ result from tethering of t he cord by the acrompanying MM . Pri_ mary dysgenesis of the brainst.em with multiple other developm e ntal anomalies is mOre likely"".
Major findin gs Caudally dislocated cervicornedullary j unction , poIll. 4th ....entricle and med ulla. Cerebellar tonsils located at Or below the foramen magnum. Replacement of normal cervicomedullary junction flexure with a "kink- like deformity".
NEUROSURGERY
4. Developmental anomalies
'"
Other possible associated findings: 1. !.>eaking of tectum 2. absence of the septum peJlucidum with enlarged interthalamic adhesion' absen~ of the septum pellucidum it thought to be due to necro.is with resorption secondary to hydrocephalus, and not a congenital absence'''''' ,,', 3. poorly myelinated <:e"lbella r folia 4 . hydrocephalu s: present in most 5. heterotopia. 6. hY'popIDS~a offal" 7.
mlCrogyrlO
8. degeneration of lower uanial nerve nudei 9.
bony abnormalities: A. of ~rvicomedullary junction 8. assimilation of atlas C. platybasia O. basilar impression E. Klippel-Feil deConoity: su page 119 10. hydromyelia II . c.aniolacunia of the Iku ll (8ft ~Iow )
PRESENTATION
Findings an d~ to brain stem and lower cranial nel"\'e dysfunction . Onset is rare in adulthood . The presentation ofneonataadiffs .. substantially from older childnn, and neonates ,,'eO! mON! likely to develop rapid neurologi~al deterioration with profOWld brain stem dysfunction over a period of several days than were older children in whom symptoms were more insidious and rarely as severe". Findings include>'·II: I . swallowing difficulties (neu rogenic dysphagia) (69%)". Manifests as poor feeding. cyan0!5is during feeding, nasal regurgitation, prolonged feeding time, or pooling of oral secretions. Oag rene~ often decreased . More severe in neonat.e~ 2. apneic spells (58%): due to impaired ventilatory drive. More common;n neonates 3. stridor (56%): more common in neonates. usually WOn;\! on inspiration (abductor and occasionally adductor ,'O<:al cord paralysis seen on laryngoscopy) d ~ to 10th nel"\'e pa res i.; usually transient. but may Progn'-SS to n'!!pi ratory arrest 4 . aspi ration (40'>\) 5. ann weakness (27%) that may progre~! to quadriparesi~" 6. opisthotonos (18%) 7. nystagmul: especially dl.lwnbeat nystagmus 8. weak or absent cry 9. facial weakness DIAGNOSTIC EVALUATION
Skull films May demonstrate cephalofscial disproportion from congenital HCP. Cl'II.n iolacu.
oi. (AKA liick.e nschiide l) ;n 85% (roWld defects in the skull with sharp bordel'$, &epaMlted by irregularly branching bands of bone). Low lying intemaloccipitol protuberance (foreshortened poIItenor fossa ). "; nla rged foran,en magoun' in 7()<.{,; elongation of upper cervical lamina".
CT and/or MRI find ings ("
,l
primary findings A. ·Z· I.>eod deformity of medulla~ B. cerebellar peg C. tectal fusion ("tectal beaking") O. enlarged massa interml?dia (int.erthab.mic adhesion )· E . elongatiolV'cavicall;zation of medulla F. low attachment of tentorium associated findings A. hydrocephalus n. syringomyelia in the area of the cervicomedullary junction (reported inci· dence in pre MRI era" ranges from 48·88%) C. trapped fourth wnlride O. agenuisldysgeneais or corpus callosun,·
'"'
4. ee,·elopmenLalal'1Omo.lies
NEUROSURGERY
E. cerebe llomedullBI)' e<>mpr"''''on Lary ll gOSCOpy Performed in pa t i.nQ with lirido r to rule out croup or other upper ~"piratory tracl i"fection. TREATMENT
inw,t CSF . hunt for hydro«pholua (or check funct.ion 0( ex ialing ahunt ) if neurogenic dy.phagia, Htndor . or apneic apell. occur, upeditious poiI~rior r.... l a d«omp~ .. lon i. recommended (_ Nlo",) (r.quired in 18.7<;\0 of MM pat ienll"); btfore recommending de«>mpression. alway. n,ake aurt tM pa~nt h .. a functioninB .h untl S u rgical d eco mp re.. io n NB : it hPi been arllled that part of the explon-'lon for t he poor operative r..ulUl in infants ia thot many of the ne urological fiedine' may be dut in part to intnnaie (u ncorrectable) abnorma litie. which l urgie. l decompreuion unroot improve"·", A diutnting view i. that the hi.tologic luion.s.1'1! du e to chronie bl'llin ltoem compreuion and conoomitant isehemia, aosd that upeditiou. brain I tem deoompreuion .hould bt:carned out when any of th e followingm t ical warning . ign. dev.lop: nlUr
Surgical t.ech nique: Oeoompreuion of cerebellar ton l il., ulually with du",l rraft to dec<)mpreu dur • . Patienll i. pla""d prone, wi th the neck nexf
68<;\0 h.d complete (Q' ne.r oomplete resolution of.ymptoms, 12<;\0 h.d mild to mod· erate residual de6eilll, and 2091: had no improvement (in Bent'r.l, neona," far1!d WO!'lle tb.n older ch ildrM )I'. Rupiratol)' arr«1 is the most oomroon uuse ofroorlality (8 or 17 patienll who died ), with the rell due to meningitisfvent riculitia (6 patients), .. spiration (2 patientl), .nd bi liary "l'8i. ( L padent)"". In follow · up ranging 7 m....6".., 37.8'\lo mort.lity in ~rated patienl!l. PreoOp It.a tUl and the rapidity of rotu.rologic deterioration ~re the Il>0l>1 important progoostica"" .... l'oIort.al"y ri te it 71'110 in in fants h."in, cardiopulmonary arrest, vocal cord panly." or ann wnluwas within 2 W(M!U of presentation; compared to 23<;\0 mor· ulity in pIItienlll with . more rradu.1 deterioflltion . Bil.teral vocal eortl paralysis was a particularly poor pr(lfrnOSticator for ,"poNe to . urgeryf'. OTHER C H1 ARJ MALFORMATIONS CHIAR/ TYPE 3
Ran. The mot! ICIvere IOrm. Dilpl&Cement of poIterio r r...u strudUreII, with cerebellum hemiated through fo"men magnum into oervic.1 canal, often with a high ce ....·;· c.l Of' lubottipiul ene.epha lomeningocell. Ulually inoompatiblt with li fe. CHIAR/ TYPe 4
Cerebell a, hypopluia without cerebellar herniat ion .
NEUROSURGERY
... Developmental a roomal ,es
,.,
4.7.
Dandy-Walker malformation
At.",;a offo' 8111'n. of Magtndie and Lu.c:h k,". Thl, l"uulU in &&frlesi, of tile ~r I I I~ posurior roan cyatcommunicating with lin enla rged 4th ventricle (.orne retrotl'l'i'beUar .nchnoid cyaU mi mic Oandy. Walker, but theAl! do not heve vennia" agene.is and the c)'IIt does I1l!& open into the 4th ventricle)
lObelia. venn's with
Hyd.""",phaJuaQ«un in 9O'Jl, ofcl.U$, and Dandy- Walker malformui(ln is present
of.n caHI 01 hyd.rocepha!IlI, Associated ab normalit ie. in
2-4 ~
eNS .bDOnJUllitia include .gents;, oft'" o;(Irp'" caIto-u rn in 17,.... a nd occipital encephaloc.le in 7<JIo., Olhu lindi"p indude heterotopi.., apina bifid" syringomyelia, microoephaly, den::ooid cyau. poreneephaly. and Klippel-Feil def
.il. microphthalmi.), and cardiovascular l oomlliea (e.l . septal defect.l. patent ductus Ir· teTiot .... lorti, f;OITClItion. clextroo;ardia). Note: be .w.~ of the lihlihood of I ean:ii3C: abnormality when considerinllurgery on theae patient.l.
Treatment In t he IbMn.. of hydrooephalUl. thue .... y be follow«l. Whell t~atlllent is ~8' lary.ihunt the pwterior f.... cyst-. In the rare patien t wi th aqued..el.llatena5;'. shunt the lateral ventri<:lea all(l. Sh untinl the venlric ln alone ;. c:ontraindiclted because of the ri,1t ofupwlU'd herniation.
Prol11ol i, 7~ 1 00'lIt tha"ce of l urvival . Only 00'l0 have nonnal lQ. Ataxia, spastic:i ty. and poor fine motor c:on t rol arl common. SeiIU,"" ooeut in I~.
4.8.
Aqueductal stenosis
Aqueducl.ll s tellO'llS (AqS) produces what;$ tometimn ",lied t rivenlri<,:ul ar hy_ droce phal .... charatteri~ by a normal .i~ 4th ventricle Ind erJerged third.nd lateral ventritLl'A on MR I or CT. Most ""." occur in ch.ild~lI, however some p~aent ror the first time in adulthood. ETIOLOGIES
a congenital ma lformation, ""y be nlOcial.ed with Chiari .... Iformlluon or neu· rofibromatosis 2. acqui red A. due t.o inflal"JUl)9tion (foll owing hemorrhage 01' in fedion, e .g. I yphil .., T.B.) B. neoplasm: esp~i.Hy brainstem utroeyt.omu{ineluding I«ta l gllom... ~ (XJgf 422), lipom .. C. quad rige min al pl ate a rachnoid C)'Ita I.
IN INFANCY AqS i. 8 freq uen tea UH of congenital hydrocephllus (HCP ) (up to 70!l0 ofca.es"'J.
but OC<:8BiooaHy may be t he WIi.Il of HCP. Patient.l with co.nital AqS ulually hive HCP at birth or develop it wi thin - 2-3 m~. CongenitalAqS may be due to an X·Hnked recellSive gene"'. Fo ... r t~ of ron genita l AqSdetcribed by Ruaaell (.um ... l riud'O); 1. forking: mul tiple channel. (o!\en n, rrowed) with norroal epi:.he UlllInina that do not ",eet. leparal.ed by normal nervous tissue . Usually uaod"led with other oon· genita l "bnonnaHtie.; (spin, biMo. royelomenlngo«le) 2. periaq ...educ:tlll gliosis: lumina l narrowing due t.o lubependymala.tn>ty!ie prolir. eration 3. t rue ltenO'l i. : aqueduct hiltologically nonn.l 4. u pturn
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4. Developmental aQoroBliea
NEUROSURGERY
IN ADUI. THOOO AqS may be on overlooked eaU5e of "normal preAure hydr
See Table 4·13 . Headachf 1'181 the QlOllI Cllmmon symptom. lind ~ ad charae .... rialiC4 of HlA 1I000000;au,d with e!e~ated ICP. Viauru ~h.ngu were next , lind liBuaJiy etlM'.lfod of blurring or 1051 of acuity End""rine cb lngu induded menstruol irre(Ularities, hypOthyroidism, aod h irsuti6m ,
Signs Papllledema was the most COmmOn lind· ini: (53%). Vi~ual fLel,u were normal in 78~, lM remainder having l'1:!duced puiphe ral vi, .ion, increased blind spota, quadranlic or hemianopic f'eld euta, or OU:Oto1ll8ta. lnwlle<:-lual impairment waa pruent in " I Ita6136'11o. Ol.her signs included; alma 129%), "pyramidal ttoct signi" in 44':; (m"l ld hem;. or paraparesilll2'2%), 6pntkjly (22"1. or BabUl$ki's (20%»), an(l3n';a (9W».
E VALUATION
MRI ill lhe t1!~lof cholee. MRI will ~how ~be absto.oce of Iha nonnal now ,."id ill the Sylvinn aqueduct. Con l rn l s hou ld b8given Lo n, l~ut tumor,
T REATMENT (OF NON-TUMORAl. AOS)
Although treatmeo~ of the prifllllt)' leaion h."" been atu.Olpted (e,g , Jysi~ or"que· dllct.al ""plum), thi~ haa fall"" intndi. ravor with ~be improved """ QlC)' "fCSF ahunting. CSF i3 uRuaity ahllnled Lo the peritonellQl or the va!clll~r ~Yfit~m, however ahlintjng to auhaT8chooid ~pa~ Ie al~o f"'iL'l,bl~ (once. OMlroction at the levi. ofth a rad!llQld grtInu· latione iuLIJ ~n ruled out). A 'I'orkiltU~n 6hunt nloy work in adult eas£Sil, however pe. diatric patienlll wit h oba~ruet've hydrocephalus may not ha"11 >UI /ldlHlua~ly developed auhsraehnoid ~pace for I..,;. to funcllon properly, FoUow.u p o(ot iea..ll ...." y ... ars to rule-out tumor ... recQmllli!ndM.
4.9.
Neural tube defects
CLASSIFICATI ON
Various elassification IY6umS elDSt, thi' OIIe;. j\dj\pled from Lemire". 1. neurulation d@fl'
A, crani.. l
NEUROSURGERY
4 . Developmental Bnomat. ..il
'"
microcephaly: U~ below bydrimence phaJy: loss of significllllt portion of cerebral hemi· spheres which a re replaced by CSF. M u~t be diff.... entiated from maximal hydrocepha lus (Sft po.ge 180) 3. holoprosencephaly: Set bdow 4. l iue ncephaly: ""'f be/t)w 5 porencephaly: _ bdow to distinguisb Nom sch iuncephaly 6. agenesis ofl:Orpus callosum:.n b
'u
Migration abnorm a lities A I lightly different clauification scheme defines the following u abnormal ities of neuronal migration (!fOme are considered posl neurulation defect • . .."" "bow ): 1. l; _nce phaly: The most severe neuronal migration abnor mality. Maldevelopment of cerebral convolu t ions (probably an arrest of cortical development at an early fetal age). Infants are severely retarded and usually don't survive:> 2)'T11 A. agyri a : completely smooth s urface B. pac h ygyria: few broad &0 flat gyri with shallnw l ulci C. polymicrogyTh. , small gyri with shallow sulci. May be difficult to diagnose by CT/MRI , and may be confused with pachygyria 2. b et eroto pi a: abno rmal fod of gray matter which may be located anywhere from the auboortical white matter to the aubependymallining of the ventricles 3. ""hh.en ce pb .. ly: A. den that com municates with the ventricle (as lTU'Iy be
HOlOPROSENCEPHALY AKA arhin en ce pbnly . Failure orthe telencephalic vesicle to cleave into two cerebral hemispheres. The degree of cleavage failure rDuges from the severe alohar (aingle ventricle, no ;nterhemisphe,;c fISsura) to semilobar and loba r (less severe malformation s). The olfactory bulbs are usually small and the cingulate gyrus remains fused. Median faciOCilrebra! dysplasia is common, lind the
"
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4. Developmental anomalies
NEUROSURGERY
MICROCEPHALY
Definition: head circumference mare than 2 standard devia,jol\ll belnw the mean fnr sex and geSl.8tional age. Terms thalare sometimes used synonymously: mionx:rania, mi_ crocephalus, NCIt a Bi ngle entity, many afthe «mditions in TabU: 4-14 may be a!lSO
AKA macrencephsly, AKA megalencephaly (not to be confused with mru:rouphaly, which ;8 enla rgement of l he s kull (",e page 919)), Not a tingle pathologic entity, ~ enlarged brain wltich may be due to: hypertrophy of gray matteralone, gray and white mat.ter, presence of additional structu res (glial overgrowth, diffuse gliomu, heterotopias, metabolic storage diseases, .. ). May be soon in neurocutaneous ~yndromes (especially neurofibromatosi s). Brains may weigh upto 1600-2650 grams.IQ may be nnrmal, but developmental de· lay, retardation, spasticity and hypotonia may oc<: ur. Head circumference is 4- 7 em above mean. T he usual sigTI8 of hydrocephalus (frontal bossing, bulgi ng fontanelle, "setting sun" sign, scalp vein engorgement) are absent. I maging studies (CT or MRI) show normal ailed ventricles and can be used to rule out extra·a~ial nuid colle<:tiont. R ISK FACTORS I . early administration of prenatal vitamil1ll (especially OA mg offolic acid daily'"
") may ~~ the incidence ofneurnl tube defe<:kI (NTDs) (confirm that vitamin B" level~ are normal, see ptlgf 9{/4 ) malernal heat e~;lOsure in tbe fonn ofbQt.tubs, saunat or fever(but not ele~tri<: blankets) in the f,,,t t n mesler was aijsociated with an increased n sk ofNTDs" 3, use ofvalproic ac;d \ Depakene®)during pregnanty iss»ociated with a 1_2% risk ofNTO'" 4 . obesi ty (before and dunng pregnancy) increaee!l the nsk ofNTO.... " 5. m~l.erna! cocaine abuse may inCTease the nsk of mi~roo:ephaly, disol'de" ofneu· rona! migration, neuronal diITerentiation and myelination'" 2.
P RENATAL DETECTtON OF NEURAL TUBE DEFECTS
.5:«!.!.m a\pha-fetoprotein IAFP) (See Alpha .{rloprolrin On page 5-01 for background ). A higb maternal serum AFP (.. 2 multiples of the median for t he appropriate week of gestation) between 15-20 woou gestation cames a relative risk of224 for neural tube defe<:ta, and an abnormal value (high or low ) was associated with 34%ofall major congenita i detects" . The sensitivity of maternalseruIJI AFP for spina bifida was 91%{10 of II C8Se$), it was 100% for 9 cases of anencephaly. However, other senes show a lower sensitivity. Closed lumbDlj.8o ral spi"" defe<:ts , accounting for ~ 20% ohpina bifida patients" , will prooobly be miQed by serum AFP &ereening, and may also be I'\li$se-d on ultrasound . Since en. l.ernal serum AFP riee!l during nonnal pregnancy, an overest;male of gestational age may cause an elevated AFP in1Prpreu..:!~ . norm" t, .. nd JOn ,,"""r"8ti"'~te """Y """ ..... norm911evel to be inu-r_ preted 8S elevated..,
to""
U1tr aSOUDd Prenatal ultrasound will detect 90-95% of casea of spina bifida, and thus in cases of elevated AFP, itcan help differential
NEUROSURGERY
4. Developmental anomalies
4.9.1.
Agenesis of the corpus callosum
A failute of commiuu l'1Ilion oceurrin, ~ :2 weeu .ftu con<>eption. The COflI .... tllllo. I ll"" (CC) rorm. from l'Ol tn.un (gen u) to I pltnl um" . thu. in m08l caaea there mil)' be on anterior JlO rtion (genu) with noapienium (the conyerte «ell ... Lelia frequently). IW~ult.s in txp&Glion of th, thkd ventricLe and te p. ration orlh,late".1 ventricle. (w hich deve lop dilated occipi tal !lorna li nd atria, and concave mediol bordeTll).
me ide nee I in 2,()()()"'3,OOO ne\ul.rndioIO(ical exami nations.
As&oe ia ted neuropa tho logic fi ndin g." pore_ph_I), microgyria interhemi.phe.ric 1iJl2mu and li poma. or lbe COrpUI callosu m (Ht page 96)
art.ineo",*phaly
optic atrophy ~obom..
hypopl ••if, oftha limbic ' ,Item bundl" ofProbtt: aborted btcion inp of corpu. .,.Ikwum, b'Jlge into late",1 ven_ lrid. 10.. ofhorizonta] orien lation of d ngu late gyro, t<:hiz.en«ph aly (He pap 112) anterior and hlppocM)pal comm iuwu may be totally Or partiaUy a'oosent't hydroceph"lul o;y&t& in the recioll of the COrpUi a ll osurn I pin" hilidA witb or without rn~omeningocele "bsenee oftne septu ... peUUCiOUOl: ' " ptJgf 122
Po... ible p r esen t a t io n hydroceph.lul microceph"ly aei lu~1
(rare)
precocioUl pube rty disconnKtion syndrome: 100"" likely with ~ CC defect th.., in congeniUlI May be an in<:idental linding. and by itse lflllay have no clinical $igniflCar>ee. How_ ever, m.y be occu r al part ofa more ... mplex clinical ayndroD\f! Or chromoeomal abnor· mality (e.g. Aicaro i syndroroe: .nesis ofCC, seilures. retard ation , pst.<:hes of retinal pigmenUltion).
4.9.2.
Spinal dysraphism (spi na bifida)
DEFINITIONS" s;ma bffida oa:uIla CongerWII l absence 011 5jli'Ioos process and Yllri1b1e IIlIOOIlIsd Ie...... No ...iiii ~e oIl'I'IItWIges 0InN". b5$Ut ($NO b*ool.
The following two entiti es are IITOuped togethfor und er the term spilla bit'id a .pe.... III (aperla from the Latin fo~ "open") Or apilla bind. cYltic • . menf~te CorIgerWIIk!ed: in vertebrlllrtflH WIll epIle dISIe/I5IOn 01 mriIgH, Iii no .t>nQHr\ll~1y 01 ~Ml"'IisIue . C/rIe t.iftl1la'III _ rIIIIIOIogoc deId. myelomeni'lgocelt ~I 6e1ecl in v&!Iebr&Iardles wilt! eys!ic diltUlliorl of mri'oge$ and IlTul;luIil 01 f\n;IiOrIallbnormal'fy 01 spiIIl eord 01 caUIIIlQUN [see~. SPINA BIFIDA OCCULTA
OcCUI"l in ~ 20·SO'h of North Amt rk l.nl. Often.n inciden tal finding. u ..... lly ofllO clinical im por\llnce wh en it o«un alona. However. it II)II YOCClllion.lly beassoci.ated with diutematornyeli a, \.ethe...o cord. ]i porno, or de rmoid tumor. Wh eo IYlllplomatit from one of the IlUOc:lll\ed c:onditionl, the ~senl.&tioo i" tha t or
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4.
~veloproenUl I
anomalies
NEUROSURGERY
tettlered tord (gai t dinurbance, leg weakness and atroptly, urinary diaturba nce, foot d~ (oralities ... , see Tf/heret! t»rd .yndromf, page 120). The derl!(:t trUly be palJ.>llble. a nd there may be ove rlyiog C'J lsmloua mani festation. (.ee culo:rnrour " igrruolo: 0( dylnlphi,m in Tablt 4· 17, ""ge 121). M YELOMENINGOCELE
EPIDEMIOLOOY/OENETICS
In cidence of.pina bifida with meningo«le or myelomeningocele (MM) 1.1 1-211000 live birtlll (O.I .O.21to). Rislr inc rea_ to 2-31to ifttler. i. one previou. birttl with MM .•nd 6-81to after t ..·o "fTec\ed ctilldren. The nlk i•• Iso IncruHd in f. milies where cloH relllUvel (e.g.• iblinp) hav, given birth to MM clUldren, esp.<:i.Uy ...·hen on the mother'•• ide arthe family. Incidence may increllK in ti nle' of wa r . famine o. economic diu.atera. but it may be gradually ded ining overallt;l. 1'ran.million fo llowt nan·Mendelian geneticl. and i. probably multiractona!'
Hydroce pbalus in myelom eningocele Hydrocephalu. (H C P ) develo p' in 65-86~ of J.>lltlen u with MM , and 6· I()
PRENATAL OIAGNOSIS See P... dflKliOll o{nfurallu~ dtft!CU on fIOIge 113.
"",al
MANAGEMENT
AOMISSION
1. assessment and managemen t of lesion: A. mt!lsure she of defect B. aseeN whet her lesion is ruptured Or unruptured I. ruptured: . tart antibioties (e.g. n.reillin and gentamicin; DIe 6 hre afur MM closure. or continue if Jhunt anticipated in next 5 or 6 days) 2. unruptured : no antibioties neeesslU"y C. «wer lesion with toolfa. then ,ponges soaked in lactated ringers o. normal taline (fono a atenle &:I ute n ng around the lesion if it i. cystic: and protruding) to prll'o'ent desiceation O. Trendelenburg position. patient on .tomach (keeps pressure off lesion) E. perform su.-gieal closure within 36 hre unless there i. a contraindi<:ation to , urger)' (.io> ultanlOus . hunt if overt hydl"ll«phalul (HCP) at birth): see 1'I'm i"4 of MM dNU,.. below 2. neurological . . . ssmenl and manaK"mlnt: A. it.o.>ml related to . pinal lesion I . watch for l pontaneoUS roovemelltofU>e LEs(good spontaneous melVe-ment correlates with better later functional outeam"') 2 . alMss Iownt level ofneurologic function (_ TCJblf 4·1$ ) byd'e<:Iti"8 .... poole of LE. to paioful ltimulUl: .Ithough some infantl will have • cle... demlJ"Cl.tion between nor mal and abnorma l level" at leut l how.am. mixture of normal. aod a utonoroou. acuvity (...uing from uninhibited anwrior hom motor neurona)" a. dirrel'flnlil t ing ren.. movement from voluntary mly be difficult. In general, voluntary movement i. not . tere-otyped with repetit ive tti muh.. and ran.. moven>ent usua.Uyonly pe.. iatla.long .. the noxioUi lti mulua ia applied B. itema related to t h. commonly lllOdal&'
so..
NEUROSURGERY
ren...
4. Developmental anomalies
us
3,
3. check for .nspiratory stridor, apneic episodell ancillary alI_ment and management' A. evaluation by neonatologist to aue&S for other abnonualitiu. eapeciaJly thOM' that may prech.de l urgery (e .•. pulmol;lary immaturity). The~ i. an average incidence of2-2.5 additionalanomalin in MM patienr.. B. bladder. start pat,enton regular urinaryutheteriutiona. obt.in urological ronsultation (non-emergent) C. AP & lit s pine film" asses. KOI 'OOIi. (base liM ) D. orthOped IC tonUllalion for !leVI "" k,yphotie Or trollotic I plnl deformlti.. and for hip Or knee derormitiu Tabll " 1S
SURGICAL CONSIOER ATIONS TIMING OF MM ClOSURE
Ear ly dosu re of MM dered is.D.l!l. .... odated with improvement of neurologic fWlc, tion. but evidence luPPOrti lower in.feetion rate with early closu~. MM I hould be dosed within 24 hns whether or not membrane is iotact (afU:or _ 36 hrs the back le$ion is coloniud and there;, increased risk of postoperative inf~ion).
S imultaneou s r.W r e pair a nd VP s hunting In patients witbout hyrlroce phalus . most su rg..,ns wait.t least _ 3 days after MM repai r before shunting . In MM patients with clinically overt HC P at birth (ventriculomegaly with enlarged OPC andlor symptoms), MM repai r and shunting ml Y be performed in the &ame sitting without increased incidence or inreelion. and with shortu hospitaliz.atiorr" ·... It may also reduce the risk ofMM repair breakdown previoualy IIftn during the interval before shunting. Pa t ien t is pOl it ioned prone, head turned tori&hl Cto expose the right occiput), right knee and thigh ne~ed to e~Jl"OM right n~nk (consider u.. ing left. nank to prevent confusion with pppendeetomy..:a r l. ter in lif~). POST-QP MANAGEMENT OF
MM REPAIR
1. keep patient orr aJl illciaions 2. bladder c.atheteriution ~gimen 3. da ily OFC measurements 4. ifnotihunted A. regular head UJS (twice weekly to weekly) B. keep pltient nat to I CSF p~u~ on inci. ion
LATE PROBLEMS
Include: 1. hydrocephalus! may mimie - anything li. ted
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4.
De~lopmentaJ
.noml li ..
~low .
Al.Yl.6XSlW.LEJlJJ.I..
NeUROSURGERY
SHUNT MALfUNCT ION when. MM patien~ de~rioratn Iyri ngomyelia (a nd/or Iyringobulbil): lin JH16~ 349 tethered cord (8M rtlhtr~d corcl.,.lIdrome, plge 120): ~ all pfltient.f with MM d o. lUres hive. tethered cord radiographically. hut only I mlnori~y arelymptom.t.lc. Unfo rtunl~ly the~ i, no good telt to eheck for Iymptoml tie retetheriog(SSEI'. may deterioratel"" A. acoli08i. : early un~therini of cord m.y imp rove .eoli08is <_ScolU:illi4 ill u!lh~rfd <:rI,d, pfI~ 120) B. .ymptomalie tetherin, [, of\en manifNted 8$ neurological deterioration of delayed OrlMt-t. medullary compreuioo It foramen m.gnum (lymptonl!.tic Chi.ri 11 m.lforma . tion , 1ft page lG-71 2. 3.
OUTCOME WitboUlllllY t~atmen~, only 14-3K of MM inf.. ntl IUrvi~ infancy: these usually repretent thel...t Mve~ly involved; 70'10 will h,ve nonn.IIQ' •. 5O'lt are .mbulatory. With ItIOdem tre.tment, of MM ;nfanu .u rvive. The most common caUile of e.rly mortality are complication. frOIO the Chi.ri m.Jfonnati<::n (re.piratory arrest, as· piration ... ), where late mortality is Ulu,Uy due to . hu nt m.lfWlCtion. 80% will have no .. cnallQ. Meotal retardllion ;. mOltdoeely linked to .hunt in fection. 4(1..85% ar
as...
LIPOMYElOSCHISIS Carol.pm.1d yora phiam with lipoma. Six fonnsa re described", th~ fol1owing3a~ clinkaJly important .. poseible e.... ses of progressive neumlogj.c dysfunction via tether. ing (_ Td~nd arrJ .,.ndro",." pa~ 120) ancllor comp ression: 1. (jntra)du ral lipoma 2. lipomyelomeningocele (Ift~) 3. fibrolipoma of the filum t.ennina\e
UPOMYELOMEHINGOCElE A subcutaneoul lipoma. that"",_ through a midline defect in the lumhodo .....1 r... cia , vertebral neural arch, and du.,., and mell:" with an IIbnormlllly low tethered cord". 'l'hne .....y be tenninal. do .... l. Or trallsilion.t (between the two). The int.,.durel fatty tu",..r may alao be known 115 lipoma .,r thec:.uda equio • . l n addition to bein,lIbnormelly low. the conus cneduU.ri. i. split in the mid line do .... lly usually at the IIIIIJJe lev.el as the bifid Ipine, end th.i. dors.l Dlye!OIChi.il may utend su· periorly under intact . pin.l.rches'". There ia a thick Iibrovucular bend t.htjoin. the lamina oftbe most cephalic vert.eb"e with the bifid I .. mina . Tbil h.o.lld coDitrict. the cneniogooele aac.od neural u. .... e. caUtin, a killlt in the"uperior , "rl"II<;I of the menin· IIJCf!le. The du ra i. delli_nt at the level of the do ..al mye"-'hisi., and reflecu onto the pl.code. The lipoma paSSI!I through thill dehitoen<:l to hKoOJe attached to the dor..llu .. face of the placode, and m.y continue COI!phalad u!;lder intact arch.. with the pouibility of extenlion into the central ca.o.al.uperiorly to level. without dorsal lOyeIOlChi. is. The lipoma i. di.tinctfrom the normal epidural fat which .. looser and more areolar. The.ubar.chnoid .pace typically bul,N to the aide contr.I.t.ral to the lipocna. TheM lipomas accou nt for 2K of COV«ed IwttbOlacral maMU. PRES£NTAnDN
In a pedi.tric seriN, 56,. preaented with. back 1DIlM, S2";Il. wi th bladder problema., "nd 10,. beaoute of foot deformitiN, parelyoi. or le, pflin". PHYSICAL EXAMINA TION A1OlOIt an patient.l have cuta~ .li(O"lata of the auotlated .pin. biflda: fauy .ubalt.aneo.... pad. UoeIIted ovar the midli"" end usually extend, asymmetric:ally to one lida) with or witho ... t dimple" port-wine Stainl, abnomul] h.ir, derm.lsin ... open;ng,or
NEUROSURGERY
'"
akin appendages" . Clubbing ofth ~ feet (talipes equinova rns) may ~UT. The neurologic eKarn may be normal in up to 50% of patients (most presenting with skin lesion only) . The most COmmon neurologic abnormality ""as 8eUSOl}' 1069 in the sacral derma tomes. EVALUATION
Plain LS spine x-rays will show spina bifida in most cases (present in almost Bil by definition, but some may have sefOlentation anomalies instead such n butterfly vertebrae). AbnormaLitiu of fusion and sacral defects may also be se
Since sympl.Oln$ are due to \ 1) tethering orthe spinal cord. especially during growth spurta, and ( 2) compression due to progussive deposition offat, especially duri ng periods ofrapid w.. ight gain; the goals ofsu rgery are to release the tethering and redu"" the bulk of fatty tumor. Simple cos",etic treatment of the subcutaneous fat pad does not prevent neurologic defIcit, and lJIay !Oake later definitive repair mOre difficult Or impossible. Surgical treatment is indicated when the patient reaches 2 months or age, or at the time of diagn osis if the patient preaents later in life. Adjuncts to su rgica l treatment inel""' .. evoked potential monitoring and laser. Overall, with ,urgel}', 19'1> will improve, 75% will be ullchanged , lind 6% will worsen. Foot deformitiee olten progress regardless. D ER MAL SINU S A tract begilUling at the sk.in surfllce, lined with epithelium. UsuaUy Ioc8ted at ei· ther end of neural tube: ceph alic Or caudal; most common location i, lumho$ll.cml. Prob· ahly results from failure oftbecutaneous ectoderm to separate from the neuro~toderm at the time afelosure of the neural groov .....
SPINAL DERMAL SINUS
May appear as a dimple or as a sinus, with Or without hai rs, usually very close to midline. witb an opening of only 1-2 nun. SUrTl)unding sk.in may be uormal. pigmented (·port wine~ diseoloration), or disl.Orted by an underlying mass. The si nus may terminate s uperficially, may connect wil-h the coccyx, Or may travenle hetween normal vertebrae or through bifid spinee to the dural tube. It may wid_ ell at any point along its path to form a cyst; called an e p id e rmoid cyst iflined with stratified $quamous epithelium and containIng only keratin from desquamated epitheJium,orcalled a dermo id cy3t ifalso lined with dermis(conUiningskm appendages. such as hili< follicles and seba~u . glandsl and also containing sebum snd hair. Although inDOCuous in e ppearance. they are a potentIal PIIthway for intradural in· fection wlUch mlly result in meningitis (sometimes recu,nmt) and/or intra thecal abscess. Le"" serious, a local infection may occur. The lining dermis contains normal skin appendages which may result in hair , sebum, desquamated epithelium and choleeterol, withln th" t,·"d. lui. Ii re~ult, u,~ ..... "~" .... "f1.4,, .i"u~ trae1. ,.. ~ inil ..!i", ,ULJ ~ ...., eli""" ... ...,nl" (chemical) meningitis with possible de)IIyed uechnojdjtjs ;tit enters the dural sPlIce. InCidence ora prf's"med sacr al sinus (a dimple whO$
Thes-e tracts are NOT tel be probed or injected with contrBst U thiS can precipitate infection or sterile meningitis .
'"
4. Developmental anomalies
NEUROSURGERY
ElIaRI i~ dJrectt:d IoOw(utb detllClinllablIOnuali!.iee in .phincter runttion lanai and unnaryf. lumbosacral reflne.. and lower extremity 1le"0Qtion and function.
Radiologic e va luation
When lean at blrlh,llikJI.I~.lI.U'1 i. the belt meanl to evaluate for 'vim. bifida and a poIIsible nlll.. in. ide the o;8nlll. Ifleen initially followinll biM, lin MRI .hould be oOLained. Sagittal ima&d may demon$t.r~te the ttllct .nd ia pOint of.tWo.hmenl. MRlal*O Optimally demoNtc.t.eI m..uea (lipomas. epidermoids ... ) within Ihe CllnaJ. Pillin ,,·tII),1 lind CT Ira unllble to do:tmOr\&lrate Ihe fine ttllct which 01/lJ' .. ist between th skin lI11ld Ih
Sinuod abo ...e Ihe ,umbolllocral ,..,gion ahooJld be IUTfl""'U" ,..,moved. Mol'll ea udl1ly located ,;nusea ere I lillMly controversial. Although. 2&lI. o!vruwned Acnl.inUHI liel!n el birth will regreaa to II de~p dimple on follow·up (time not specified). il i, recom· mtlnd.,d th~t 11.11 dermallinnllell should bllur&ic.Uy explored and fully exc:.1Ied IIri!u to lhe development of neurolCliic delitit or &iJTl& oflnfKtlOn. The r Hult. foIlowi llil inlndu · r41 inI~tion ar, never _good. aiwhtn und"'rllken prior to infleUon. s...rgm within th4 week of djusnl)lli~ ilappropriate. Sinuu. that U!rminale on the tip oll.he COC<:)'l' Mlrely penetrate th"'du ra. ftnd may not need to be tnoated unlc~ local infKtioll DOeU .....
Surgical tecbnique CRANIAL DERMAL SINUS Stalk begins ....,th a d imple in the occipital
0' nasal region . CutAneoul.tigmata of h",mangioma, 8ubn,Jtaneout dermoid cyst. 0 ' .bnormal hair form.tion mayootur. Ottipila.lsinu&e5 extend <:IIU~.Uy, lUld ifthey enter th".kul\. theydosoel>udll to the wraoh., herophili. Preoentation rna,. ' ndud" recurrent toa.:t.Hi,1 (usually S. ""nrwo)or _ptio: men.i.11git.i, . .Evalultion should inelude MRI to 100». for intr!lCraDial ext.e"'lion I.lId .,...xi. lted ~ nomalies , including an intnlmonial dermoid cyst.
TJoeatme nt WbeD opetllt;ng 00 • cr.rrial dermal sOn" •. uroe II sagiualtv based Incision to pumit do-ep eJlploration. Th& tract must be followed eompletel)'. Be p~pared to enter th .. pOlIl,(!rior foasa.
4.10.
Klippel-Feil syndrome
Conllcni\al f... ion of two Or more«!ovic.ll1 ~e~rH_ Rangoeo froln fusion ofon ly thle bodiN (conpnltaJ block verubrae) to fuiion of!.hl mtlre veub' H (iocluding posteri· oraJ",mlnlll). Result. from fallureol nonnal ~tation ofccrvtc.1 sornitef betw~n 38 weeks llllation. In~oI vltd vRfUb...1bodies ueoften flau.cned IlQd .......aaIM dioclpllcU .... abMnto, hypoplutit. tiemiv",lUbraa .....y also DOeur. Neutlll forumina are 8malle, thin nonnaJ aGel oval. Cervka J lterlOSi.Ia ....... Com plitt> .bnn~orthe patteno, t iementa with an enlarged foramen m'fIlum and fiud hyperemmion postu re I.ItaUed In. ieocepbal)' and il ,arl. lneio;lenceofKlippo:l·r",il il unknown dIN! 100 iLl rarity alld tbe fact that it " frequrnlly uymptomatk Clauic clinkal triad (an 3 at'" pHUnt in < 6011>): low poIoterlor hairline, ,oofU""d neck (brevictlJli.), aod limitalion of neeIt motinn (may nDt. be e~ident if < 3 venebne ar. fuMd, iffusion illimitl!d only to the 1 _ et1'VieaIl_lJIO. or ifhYJll'rmobllily of non· ruNd """iI!n~ compensala). Limit.tiDtI ofmovlrnent I, more wmmon In rotation than flexion.f:lltanl1Dn 0' lat>eral bending, May a«u r in COI\iunction with othu fonpnital o:em",llpine al'lOmaliH t>a.eilar iDlprfiSion and atlanto-ottipitalhDioo. Other dini"",l.ssociftt;onl include fICOl;· 01;' io 6O'lto. f.eial .. sym meuy. tortinoUMi , _tlbinl or the ofCk (~aUed pttlryVwa colli wbeo II!Yrre). Spre~I'a d efnrmity in 25-3S'it {raiHd IleIPull due 100 fal lure o1the ",pula to properly d l'AOC'nd from ita recton 01 rarmetion !\ilh in the ned< to itt normal position lbout!,he Um' time elllle KlIppe.L·Feillnion oce ..... l. IY"k lnwl ]mirror mo·
.,,<,'h ..
NEUROSURGERY
n.
tions. primarily of hands but ()(Xuionally arms also) and less commonly fadal nerve pal_ sy. ptosis. cleft Or high arched palate. Systemic congenital abnormalities may al80 OCCur induding: genitourinary (the most frequen~ being unilateral abllenoo of a k.idney l, car· diopulmonary. eNS. and in ~ 30%deafnells"' (due to defective development of the osseoUs inner ea rl. No Iymptoms have ever bHo directly attributed to the rused vertebrae. however symptoms may occur from nonfused segments (less common in $hort-segment fusionR) which may be hyper mobile possibly leading to instability Or degene.ative arthritic changes. TREATMENT
Usually directed at dele(:ting and managing the aQO<:iated systemic anomalies. Patients should have cardiac eVllluation (EKG). CXR. and a renal ultrasound. Serial examinatio"" with lateral flex.ion~xtel\Hion lateral C.spine x·raY5 to monitor for illlltability. Oc.:lIsional1y.judicious fusioo oren unstable nonruaed aegmen~maybe needed at the risk of {urther 1000s of mobility. Alaosu p<J~ 142, for recommendations rega rding athleti~ competition.
4.11 .
Tethered cord syndrome
Abnormally low OOt""'S med· uHarie u90Ciated with a short, t.b..ick· Table 4-16 Presenllng aigoa and aymplom." ened filum term ina Ie. or with an intradurallipom. (other lesions. e.g. as Lipoma extending through dura. or diutematomyelia a/'1l ooneidered III separate entities). Mast commOn in n,yelomeningocele (MM) . Diagnosis must be made clinically in MM. 88 al most all of these patients will have tethering radiographically.
P RESENTATION PTes.enting signs and 8)'1llPtoms in patients with tethered cord are shown in Tabl~ 4·16. MYELOMENINGOCELE PATIENTS
If 8 MM patient has increllsing high irJ:idence oIscoliosis arid ~ due Ie incIusio:wl scoliosis, inen'!asing spaaticity. wo"', of series by HQltm:an enlng gait (in those previously ambu· latory). o;>r deteriorating uradynamics-: • always make lure thM there is II worlting shunt with normal ICP if painful. should be con~idered tethered cord until proven otherwise if painleSl. should be considered syringomyelia until proven otherwise may be due to brainstem compreuion ($ymptomatic Chieri II malformation. M~ fXJl:e Ion reqUIring posterior fossa decompression
Scolios is in tethered cord Progressive scoliD6is may be seen in conjunction with tethered cOTd; early untether_ ing of the cord may r~ul~ in improvement ofsco\i(l6is. however. untethering mus~ be done when the scoliosis is mild . When cases of s 10' S(;Oliosis were untethered. 68% had neurologic imptovementend the remaining 32% were stabil ized. whereas when scoHosis is leven'! (., 50°) ~ 16% deteriorated. TETHEREO CORD IN ADULTS
Although most casel of tethered cord present in childhood, casH of adult tethered cord have been reported (_ 50 publi.hed ClIseS a s of 1982). For comparison of adult and childhood form • • au Table 4·17.
n,
4. Developmental anomalies
NEl/ROSURGERY
" E VA LUATION
Rediograph,icaUy; low con .... medllilana (below L2 ) aDd thickened /ilumlerminaJe (nonTI81 diameter < I ".m; diam'ltars > 2 mm are pathological). NB : apP" ..... nl mum di· ameteron c;r-rnyeJogram m'IY '1Rry with concentration of room ' l mllt4!rllll. It isd'fficult UldifTenmtiate a t4!tbl!red cord fl'Qnl a congen itally low lyingrord {filum diameter i ~ &eo"'alLy normal in 18tlilr).
P re·op evaiuatiolJ PrIMlpt'rative ill.\I!(JlPttoIl1'Ol i •• trongly recommended, e8llecia II)' if the I'P(ient ronlinen! (po!Itoperalive chang es;(1 bladder function are not unl\Ommon, po6IIibly due to Itret.d".ing oflh" lawe. liberl! oftbe cauda equina).
_\til
T AEATMENT
Ifth t only abnormality
IS
II thickened, Bhortened fIlum, then .. lim ited lumbosacral
laminectOXl'lY may suffice. with diviSIon of the filum once identified. IfaiipOms is fa.und, it may be removed with tbe filum if ilaeparal.lla easilyfrorn neuraj t.iuul\I .
Di.lltitlJU illh in g feat .... res o r the filum tm nel"V~ roots by presenoe oftbllucteiistic lIquiggly vtssel on Buriace of rtlum . Also, under tbe micros~ope.. the filum 11M Ddistinctively wh i{er app"'aran<:e than th nerve roo-tII, lind li!/amentoua_like . 1I"IInda eaJI be ..."n ",nn;nlC t.hIVugh it.. NB : ;nl... -op .1"ctriO>ll a Umula Uon wnd "",urlling of.n .. l . phlno"", EMG .... e
more definitive. OUTCOME
In MM , it if; us ually ImpmlllibJe 1.<1 pennanenl.ly un tethera a,"I, however, In a grOw_ ing MM cbild, il may be thai after2·4 IlJltetherinp that tbeth;ld will be fi.nlahed grOwing and tethering may ees"" :0 be II problem . ClOSe. that are un.Let.Mred early in childhood moy rl<\:ur laLer, e.reciall, dunng !.be adole""en! growtb·spurt. !/lciden<.:e ofpollt-op CSF leak; 1&9&.
Adull form : " ".gica! relelOlie
.elum of bladder function.
NE.UROSURCERY
i~
UIIuidly good fo. pain ",lier. Howlflver , il is poOr for
~.
OevalopmenuoJ8J\omalies
'"
4.12.
Split cord malformation
There is nO uniformly lICCf!pted nomencllltu,"" for malfonnations characteri~ed by duplicate Or splil spinal cords. Pang et a1.'·' have proposed the following . The term split cord malformation (SCM ) should be u$ed for all double spins] cords. aU of which appear to have II eommon embryologic etiology.
Type I SCM Defined u two hemiC(JJ"ds, each with i1$ OWIl oentral eaRs]"nd aurl1:mnding pia, each within /I separatedurlll tube separated by sdural -sheathed rigid osseocartilaginous (bony) median septwn. This hll8 0ften (but not consistently) been referred to asdillote m· lllomye lia . ThereaTe abm>rrlllllities of the opine at Ihe level oflhe 6plit (absent di&<:, dol'* sal hypertrophic bone where the median ·spike" attaches)"", TwO-tll' roB have over lying skin abnormalities including: nevi, hypertrichosis (turt of hai r ), lipomas, d imples or hemangiomfl.'l. These patients onen have and an orthopedk foot deformity (neurogenic high arches). T're.atment: 6ymptoms are moat commonly due to tethering of the cord; and liTe uau· ally improved by untethering. In addition w untethering, the bony septum must be removed snd the dun reconstituted as a single tube (these spillO!s aN! ol'l
Type II SCM Consists of two hemioords within a single dural tube, sePllr ated by II nOllrigid fibrous median ""ptwn. This has 80metirnes been referred w 88 dlplomyel iR. E~h hemi· cord hlUl nerve root. arising from it. There i& usually no apine abllormality at the level of the split, but there is usullily .spina bifidll occult.n in the lumbosacrlll region. T'reatment: cons ists of un tethering the cord at the level of the spina bifida occulta, and occasionally lit the le'·el of the split'''.
4.13.
Miscellaneous developmental anomalies
Some anomalies that may be seen by the lIO!urosurgeon indude the following. Septo-Qptie dySpJ aS;8 W '.11 ..... ' .. AKA de Morsier syndrome. Incomplete early morphogenesis of anterior midline structures produces hypoph.sill of the optic nerves and possibly optic chiasm (affected PIItienta are blind) and pituitlll)" infundibulum. The septum pellucidum is absent in about half the cases. About balfthe cases also I"lave schhencepha1y (_ ~f 112). Pr-esentation may be due to secondary hypopitu.itsrisrn morufesting as dwarfism, i$Qlated growtb hormone deficiency. or pa.ohypopituitarism. Occasionally hypersecretion of growth hormone, corticotropin or prolactin may occur, and sexual precocity may OCCur. Most patienw are of normal intelligence although ~tardation !lilly occur. SeptO-optic dysplasia may be a less severe form of holoprosencepl"la1y. and O«lIs;onslly may occur lIS part Of lhis anomaly (with its attendant J"KIO",r prognosis for functio." or survival, Ut PQ8e 112). The ventricles cna~ be oonnal or dilated. May beseen by the neurosurgeon because ofeoncems of possible hydrocephalus .
Absen ce or the septum peUu c!du,m .. l. '7ll Absence of the septum pelluc:dum may oc:cur in: I. holoprosencephaly:.u pase 112 2. 8chizencephaly: IU ~e 112 3. agenesis oft.he corpus calloeucn: _ PGIIe 1/4 4. Ch.iari type 2 malformation: see page /07 5. basal encephalocele 6. porencepbalylhydranencephaly 7. may occur in severe h:odrocephalus: thought to be due to necrosis with ,"""rption 8. septo-optic dytlplasia: sON! .. bo"" 4. Developmental anomlllies
NE:UROSUROE:RY
References
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4. Development.al a nomalie$
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NEUROSURGER Y
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NEUROSURGERY
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5.1.
Contrast agents in neuroradiology
IOOINATUl CONTRAST AGENTS
Wlt4' ...olubla eonlralt ltfIent.l IIlve ,upC!.neded aide. llan-waWr·fQluble anee . uth .. Palltopaqudl (Mbyl iodophel1Y lundecyl.te or [ophendylate IIlE'glurtUne). • C. ution: iodin.ted Q)nln.1t (IV or inu-I·arterial) may delay tXenotion of m e!.romo.; o (Clueopll",t4, AVllKitmed), In 01"11 hypoglycelllic agent u,ed in di.betes ~ype II. and cln beu.odlted with I.ctic.eidosi.and renal failure. The manufaclun'r recommend. withholding metfonnill 48 hn- prior to and fallowirl( ccmtJ"lUt administration (0. lance. if the ... ito ev)denceordeclininc re .... 1 function following uae or~anlr8 !t). Metlonn_ [II .hould al50 be h4'ld ~ 48 hollrs before . ny I U.gery, and should not be reatllrted posWlp until the pIItient lias fully /'eCQvered and is eati'li Md drinking normally. INTRA THECIU. CONTRAST AGENTS
'J'he,primary .pproved ",eM e mployed ror intrntheal UBe t.oda, is iohex"l (0",· nIpaqudlH_ Mw...).
madverte 'lt intra thecal inject ioD or ion ie contrast agenu • C.ution: seriou1 reaction. can oa:ur with in.dvertent inlrathecaL inj!!d.iQn Ie./(. fill' 1Il,)'e1ot:JllPhy, cill.emography, ~entricu lography , .•) ofiodinated contrail media thai
.re not specifically indicated wrjntrat~lu8e (inclu.ding Ionic cont rast agentll II weI!
a. some non-ionic agenta I. -C. Optir.l)4. Reno-60 . _ )), Thil can ClUS<; uocontrol1able sei2.U"", ;nuace....,bt-.L m-morrh.tp, <:erebn.1 edema. <'Oma. patalysis, anchnoidit;', ml'oelonWl ftonJc.clonic ruu""le SpaII.,s), rhalxklmyolYl'i~ with .... bMqu4'nt n",al failure. hyperthenni. , and re&pi .... tory compromise, with a signiflCl nl r.talily nte'. Manag;>ment suggeslioQS ioc:lude: I . immediately remove CSf' .. conl . .. t irthe e rror il recogniled ... hlll the opportu · nity i. avail.ble (._g. withdraw nuid througll ",yelO(Tlplly oeedle) 2. alevllte be.d orbed ~ ":i"lto keep Qlntl'l.at oulol he.d ) 8. if there i. a question abuut whllt may hllve OI:CUlTed (i.IL it II not cartain ifan inappropriate conb'aat "ent ... at u-ed) JUld blood lind <:SF with contrest for high · perfo'1Il8n/it liqllid chromlltograplly fOf Idt:ntifi<:ation of "rent" 4_ .ntihilumin.., e .g. diphenhydr.mine (8en.drylill) ISO "'II deep 1M 5. respiret.ion : tuppl.mellul oxygen .• nd if needed. inluhlotM>n 6. conuol HTN 7. IV hydratio'l 8. TV l!.eroids 9, IftI.t.ion!fpat.ient;'qita!ed 10. Ir.at fever with eertaminophen and ifllftdftl with. coolinc blanket 11 phermacola,;c pe ..\yJ.i. if _ _ el")' 10 man.ga mu.de 'Cl.ivity (e , . a tomidate) 12. enuoonvultant medicauon: IlIOn th. n one ecent I'''y be nqUl .td (IL,. pheuytoin .. phenobarbital .. a benl(ldiazepine) 13. conaide r un.nhe l1(lt(l bndll CT tea n· m.y help..-. if((lnt,..,t h e dift'usad in· Ina.nielly, I7uI thi. requira plactnlf pjltlent nal e nd may 11:14. be .dviaable 14_ InH.tion ollwnbar l ubarechnold drain l~. monitor. electrolyte., ftntkonvulllni 1....11, cru tina kinaR.(C K) 16_ repeat EEGe 10 lileS' _iture activity while Mdat.edffMItalyzed
'"
5 . Neuroradiology
NEUROSURGeRY
lobeJenothiazines. e.g. chlorpromazine, prochlorpera· zine, and promethazine) at least 48 hours prior to procedure. Elevate Hoa .. 30' for the first few bours afU>r the procedure. Hydrate orally or TV. U..... witb cal,ltion in patienta with sej~l,Ire history, severe caniiovaacul.r disease, ehronic alcoholism Or multi. pie ..:lel"()5is. lohe¥ol uodergoea alow diffusion from the intrathecal "pace to tbe systemic circulatioo and is eliminated by renal excretion with nO significa.nl metabolism or deiodination . Ma:rimum dosage : a total dose of3060 mg iodine should not be ex· ceeded in an a.dl,llt dl,lring a single my_ elognlln (r.ome say I,Ip to 4500 mg is OK)(e.g. 15 C1: ofOrnnipaql,le 300. 15 ml ,,300 mgllmlm 4500 mg ofiodine).
Table 5-1 lohexo l coneentrations lor adl,llts
most centers use Op\or.y5. see reX! . lolow wolh 250 rnl tlOIvs ot 0."5"," NS I(> '$!\y
"",
180 WlII be ~ _ on CT,....:I $(Ime US(Il-3 rnt 01 1~ or r:f~UI'" l E1CPf. (dtIuIe apprnxrnalely 2 parts CO<>-
u.S! to 1 par1 praseNalMo-\r ... normal saline)
N QN· //IlTRA THECAL CONTRAST AGENTS
For inadvertent in~ralhecal inject.ion of contrast agents not intended fo r intrathecal
&te obo"". Diatrizoale mcgJumine (e .g.
1,18e,
Reoo·~,
Reno-di p!!)
Nol for ill lrathoc .. 1 ...." (sloe oboveJ. A tri·jodinated benzine derivative similarto Conray. BoO! have been avai lable for a long time . Due to tbe fact th at it ionizes, it is ~and byoerosrnoJar. Widely used TV, in nel,lr oradiology for TV Mnlrast enhanced CAT scan when there is no history of prior re· action to TV tQntrnst agenta (I,ISI! iohexol in patienta with previous reaction , sow obowl. IV contra8tenhanced CTscan of the brain in adult patient with no history of previou$ dye reaction: A. 300 ml TV drip of Renordip® (30'l1> 8Olution, i.e. 300 mg/ml) over - 15 ",ins B. 50-150 ml of Reno-SO (60%50]l,It iol11. 1'ypicaHy: ISO ml is I,Ised body C1': boll,ls of 150 ml of Re nor60® (3 vials of50 ml each ), followed e.g. by 250 rnl of 0.45% NS tohelp preventdehydrat;on. UsuaUy given more slowly lodiab'n· its and the elderly whe re there is increased risk of reDal fa ill,lre II
love r8(li (Optiray®) U~
• NOl ror int rathecal use (ret Clbot.!). and eoncentration$ include:
NEUROSURGERY
s . Neuroradiology
arteriography: Optirtly 300 (lov".80164%) or Optiray 320 (iovenwl68'l&). Total procedural dose should oot usually exceed 200 ml IV contrast enhanced CT I<'an of b rain : A adult: 50-150 ml ofOptiray 300, 320. or 100-250 ml ofOptilllY 240. Typically: 100 ml ofOpti ray 320 e. pediatric.: 1...J mllkg ofOptirllY 320
5.1.1 .
Iodinated contrast allergy prep
lndieated for patienta with previOWl hi8Wry of ..... action to IV iodinated eontrast rnB.terial. Minor prevIous reactiOf1s such all hivell and itching . hould Olen! preparation with this regimen whenever p<>6IIIible . Patienta with IInaphylsctic ! hock Or severe edema cauS' ing compromise of the airway ~hould probably 1>01 receive IV iodine e"~en with thi~ prep, unless absolutely lI~aaary. Caution: th e patient may still have serious reaction (modified'). utilize 'IOn-ionic oontrast medium (e,g. iohuo]) whenever poII.'Iible steroid ($I" pogt 8 for further details ofsterDid doting) • prednisone 50 mg PO: 20-24 h rs, 8-12 hi'll & 2 hra before study • equivalent dOlle <:Jf&>lumedrol® (tnethylprednisolone) f~r TV use would be ~25 mg diphenhyd ramine (Beoadry~) 50 mg, EITHER 1M 1 hr before, OR TV 5 min before st udy optional: H1 antagonis:, e.g. cimetidine 300 mg PO Or TV I hr before Sludy have emergency equipment a vailab)e durin8" study
5.1 .2.
Reactions to intravascular contrast media
BETA BLOCKERS Beta blockersea.n increall'l the risk of contrast media ,..,action8. a nd may mask Some manifestations of an anaphylactoid reaction. They al$o make use of epinephrine inadvisable since the alpha effects of epinephrine will predomioat.! (bronchospasm, vasoconstriction, increased vagal tone). If treatment is required for hypot.!nsion. OlS)· try g lucagon 2-3 rog TV bolus, followo:d by 5 mg TV drip OVer 1 hour (glucag<>n has positive inotropic and chronotropicefTect that i$ oot mediated through adrenergic pathways).
I DIOSVNCRATIC REACTIONS AND TREATMENT
For treatment ofinadvertent intrathecal injection ofionie contran agents,:lee fIOIl~ 126. HYPOTENSION WI1l-i TACHYCARDIA (ANAPHYlACTOID REACTION) 1. mild: 1Tendelenburg position . IV fluids 2. ifno response but remeins mild: e pin ep hrin e (use with caution in patienta with COl"Qllory artery disease. limited cardiac reserve, hypertension, Or unclipped cerebrsllUleurysm) A. 0.J.O.5 ",I "f 1:\000 SQ (0.3·0.5 !ilK) q 1:;...20 UJim. (...,w.: 0.01 "'~ ) B. OR, ASEP recommendations (especially for elderlyor patienLa in ahock): 10 ml of 1:100,000 TV over 5 to 10 min (put 0.1 ml of 1:1000 in 10 rnl ofNS, or dilute 1 amp of 1:10,000 to 10 ml with NS) 3. moderate to $f!ver~ or ....Qrsenin8" (anaphylaxis): add: A. rv colloidal nuids. e.g. hetasl.arch (Hespan®)6%(colloids a re required si nce there is extravascular shift.ofnuids due to seepage, then agents also caIT)' a Bmall risk of aOerg'ic reaction) B. epinephrine (..... ",00",,). May repeat It I C . 0 1 2-6 Urnin per NC. Intubate ifnl!<;essary D. EKG to RIO ischemic changes 4 . if shock develops: add dopamine, start at 51lg/kgllllin (~" page 7 ) HYPOTENSION WITH BRADYCARDIA (VASOVAGAL REACTION) 1. mild : A. 'I'rendelenburg JlO8ition
'"
5. Neuroradiology
NEUROSURGERY
B. tv nuid.
2. if 1>0
~IPOru~,
add,
A. atropine 0.15
mr tv. may rep~at up to 2·3 mg over 15 mille PRN. VII'! with
u ... t ion in patients with ... nderlying hean dillea.. B. t:KG andlor emiae monitor: eapedaJly if atropine OT dopamine are uBed 3. ifl>O rupon"": add dopam ine •• tart at5 I'r/kglmin (.n 1X'Il. 7) URTICARIA
L. 2.
ro.ild : . elf limited. No t reatment ne«Mllry moderate: A. diphe nbydramlne(Benadryl
2. if respiratory di.tr...: 0 1 2·6 Um;n . Intubate if nece. S&ry 3. diphenhyd ramine: I « ooot.oe 4 . eimetidine:.u o~ 5. if angioedemail accessible. add iee pack 6. maintain IV line B RONCHOSPASM I. mild to moderate:
A. epinephrine: I « obotot. May repeat up to I ml B. if respi ratory distreu: 0,2-6 Umin. lntubete ifne«sury C. maintain tv line D. inhalatioll3L therapy with a IJ-adrenergic agon;lt. e.g. albutenll (Pl"'OYen· til®) if respiratory therapy is availablll, otherwise, me\.ered dOH inhaler e., . pirbuterol (Maxai.rGl) OT metaprotertnol (Meta pre Ie). 2 purrs 2. MVere: l~at as above for moderate reaction. and add: A. aminophylline 250-500 mg in to-20a: NS.low tv over I~O miru. Monitor for hypoteruion and arrhythmias B. intubate 3. prolonged: add the following (will not have immediat.e e!Tect): A. hydromrtisone 250 mg tv B. diphenhydramine: 1ft obcM: C. cimetidine: I « oboue PUlMONARY EOEMA
L 0, 2·6 Umin per NC. Intubate if necessary 2. raise bead and body 3. futoMmide (l.asixe) 40 I'DI tv 4. EKG 5. if hypo>tia deYeln.. (fDlly manifest aa agitation Or oombativene.u), add: A. morphine 8- 15 ml tv. May ~au" rupi ratorydepreuion, III! prepand to in· tubate B. epilllpbrlne: _ oOOw . • CAIJI'ION: UN only if MI can be RIO all cause of the pulmanllT)l edema. Patienlll with acute ;n \.raeran'al pathology may be at riak ofneul"OKf!lI;c pulmonary ede.ma (I« pose 7) SEIZURES
IflH'ilUIl! is notlH'lflimited . • tart with [oraaepam (Ativ~) 2.... mg tv for a n adul t. Take precautio... for .t.lul epi lept;"" (IJft pof. 265) and p~ to othe r d ....p .. in· dkated (I« pose 266).
NEUROSURGERY
'"
5.2.
CAT scan
Attenuation of the x- ray beam on Ii CT Scan i.o defined in HGunsfield units.
Table 5>2 Hounslield unll,
'f'Iwse unit.9 are not abll(llute. and vary
'canner
'01' II I8mple CT
between CT scanner models, ..... ith II SlImple beiog shown in T"bI~ 5·2.
If there are no calibration marks on BClln, ooe Cll n estimate average adult globe (eyeball) is 25 m m diameter (through. its equator).
Hcl '" 23% wIH cause an oeule SOH to be isod_ with brain
5.3.
Angiography (cerebral)
Risks Risk varies with the natw"(! afthe patholOgy being investigated and w;th the e~pe rienee of the angiogTophy team. Overall risk of II complication resulting in II pennanent neurologic deficit" '; 0.1%. [n ACAS, there was II 1.2% oom plication rate ($u P"8~ 873).
General information' In gen<)ral: non-vascular deep lesions cause changes in venou s structures. BUperficiallcsions affect arterial Btructures. The classic future of II malignant neoplull'I (e_g_ glioblastoma multifonne) on angiography is an early draining vein. Bovine circulation: anatomic variant where the oommon CIlTotids arise from a comm<m tn.>nk "rrthc oort.o. Hypoid : having only one anterior cerebral artery las in a horse). Al lcock ttlat: evaluates flow through the posterior communicating &Tte~s by ver· tebral angiography during commoll carotid compression. Fetal circulatio n : 15·36'1> of patieoq supply their poste rior cerebralllrtery on one or both sidCli from the carotid (via p-oomm) inl tead of via the vertebrobaeilar syetem. 1'0 help find the middle meningeal artery on lateral ECA angio, follow the ante rior _O'<)p of the ~phenoid ai r einul.
Caroti d-baai lar anastamolJes A p"r eil
'"
5. Neuroradiology
NEUROSURGERY
may be auociat.cd with v'Ku l" anoOl.l ies.• uch U .oe urysnu Or AVM •. Rarely, aoeu· r')'1JII1 may directly im'Olve thelle venel!. May "$0 be ''''!leiatoll "'ith lri~minal neural· gia
(H~ pa6~
;119).
ANTERIOR CIRCULATION
--..., ANTERIOR CEREBRAL ARnRY ( ACA)
See Fi&""" ,5·2. BrallChee:
reeummt Dr\.o!ry (of Ue ubn er ): II01l. arise from Al (one of the largu medi, ) lenticulG11t riaU!8. re mainder of lenti .... ulOltriac.es mDy ariooe from tbi.1a~1 - head ofcaudote. pulamen. and an· !.enor internal capsule 2. media l orbitofrontal ,rtery S. fronl.opolar artery 4. cinosomlll"lin'" A. internal frontal brandle. L /Interior 2. mlddl, 3. poaterior 1.
8.
P'lnleentMlla~
5. peric:aUoul arte.,. (rootinuation or AGA) A. IUperior ,nternal parietal (pretunHte) artery 8 inferior ;nlernal P'lrietal.rtery
MIOOLE CEREBRA L ARn.RY (MCA)
See Fil/urt 6-3. Bn.ocbtll ....,. "'idely. 10 relativ. ly tOl1'ImOn ong: 1. medi.1 f3-6 per sidel and l.terallenUc:ul..tri.ta Aneries 2• • nterior telllpo ... 1
3. po$tenor I.ImporaJ "
l.tenolorbitofrontal
NEUROSURGE.RY
Ii Neurorad.ioloC
'"
5. ascending fronta l (candelabra ) 6. pn'!<:(!nU'al (p rerolandic) 7. cent'l.l (rolandie) 8. anterior parietal (postrclandic) 9. posterior pariellll to. angular
Figure 5-2 Anlerior cerebral arteriogram (lallra! oiew)
FIgure 5-3 Middle cerebral arleriogtam (Ia!eml _J
'"
5. NeuroriJdiology
NEUROSURGERY
(ltoprint«i CO\I~y of Euut>an Kodak COQl .... ",,1
, 1IlIeri'l' caudal' vein
w_
Ihalarn05IIia te VIII)
Slginal
:... I
..~
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dImdlateralv, i'lIemal cereblal y.
(
posIerior se-plal V grW~reblal
==~ ".::::- J -,~
""., (of Galen) vein 01 poS!erior hom - slnig-tdsrr"lllS
j
def!!l middle cerebrlll'l!ln basal cerebril
ve.Io (01 RosenlhilQ
'rIt~O/anastatnoIic
TC .. k,nsverse cauda~ vtUl$
Ve91 (oi llIbbtl
IVs = insoIar .... ins
Fig "" 5-4 In!emit! carotid venogtam (laWai vi ew)
llertebral arl,rIts
PICA SlgmefIt$ am 80letiormedrllary jon s laletal medullary 1m z lOr1SillorroeQ.rlary (W/Cao.Gal loop) M z 1~ (SUp!3tonsalil) (wtaarllalloop) cs" cor1ic:aI se~1 1
Fig"" 5-5 V"rI&brObaSll a, Merlogram (Ial."al v~)
NEUROSURGERY
5. NeuroNldiology
P OSTER IOR CIRCULATION
P OSTERIOR CERE8R.AL ART ERY ( p eA)
See
"'iSLl~ ~.~,
P OSTERIOR FOSSA VENOUS ANATOMY
g~al
cerebral YeI'1 (Galen)
1X~1 t41rebel~r ' .
- - - SI.p!ior pelrosai $inus
anterior mec1ul\ary y. pelrosal"m VM of I/le !a1~raI ..cess oj lhe 4lh ventricle
5.4.
Magnetic resonance imaging (MRI)
5.4.1.
General information
DEFINITIONS'
AbbrlWiations
..."
T!lble 5-.3 Range 01 acquisltloo dsl8
...,,, ""'''' "'" .."TR (la eSC)
(Ie ~ IKI)
(TfI< 1000)
prolo1 denslly
(TR~2OOO)
Of
"..,
SIlln denSIty
T , weighted image (T 1WIl Short T, - high .igmll{brigbll. ~Anator:nic image", !OmeWhllll'l'H mbles CT. Shorter ocquisi Lion timll than T2WI. ?roton nch tissue (e.g. H~O) hoslongT" 'The only objt(:\.S Urat appellr whiteon TIW1 are fat , melanin. lind subacute blood (" 48 hnI old). Whitematter is higher signallha o grey motter (myeli n has _ high fat con· tlmt). Most pathology is low signal on T1WI.
n.
5, Nosurorodiology
NEUROSURGERY
::::
::
T. weighte d im a ge ('T'l Wl ) ("'"R T l - hiCh 8igns J /bright). -PAtholcciullmRle", Malt pIIth<>lovy .bow. UpRB bilCk .l&r\i.I. in th.ldinr • .,rroun ding edema.
.."' ........
866) .... hi,h
S pin d e D9ity illUlKe AKA bIIlAnced ima~, AKA prGlon denait)' i~p. Part"'"1 bttwun TlWI",nd T2Wl, CSF. gr~y. app.ol
FLAIR Atrnnym for ~"Lu.id-Atl.l!nWl!.M lnve ..ion
~efY.
CSP appeaNI darlL MO'II I..icln.
Including" MS pla'luu. other wh ite matl.l!rle!liOOll. WrMn. edama, and acu\.e lnr.ret. apJM!or btillht. PetiVVltricular lesion. such as MS plaques becolllll! _ (UMlpicuou"
Ec bo tra in
{AKA rast spin echo (FSE» tr il held COf'Ist.anl . I.e ia progreasively inenued utilizing ",ulliple echoes (8- 16) r atber thlln 1. Image approawes T2WI but with lubsuintiaJly ndutad Iioeql.lisitiOtl IHne
(rat il brighter 00 FSE, whith m.y
~
I1!Ctilied by !'at IUpp~;C)n tKt\niqullll),
"GRASS" image Aero",..., for ·C rad ..."t Recalled Acqu~ltion in a Steady 5111W", A · fast" T2W I uti· pan.ial flip a~Je. CRASS;5 a GE lnIMIDark. othy manuradunI!n uSl!! dUfe"'"t nlme5 . a.1I FlSP. CS~ appelll"ll "'hit.. bone ill billdl.. lind nowmg vese" lire ",hil4.1'yp;CIII Kq\Ii"t;ot\ dar..: Til ,. 22. TE • 11, Mille 8", UHd e.g. in ca ..... ical MRI to pcoduc.a "ruyelogr8pllic" lmlge, imp ....u ability to deli ...... te bony ~Ur1l. ~STlR~ image Acronym ro.- "ihon. tau ;nven""., ....:overY'. Swnmate! T, '" T f ! 'gnala. CaLlIIeII rat to drop out (lOnIeti"," II-acall~ ra~ l upp rftOlloo imIIgel. , 11(1"" gadoliniu m enhance· rtlent 1.0 Ihow up belter in .~.. orfal. UtoefuJ primarily in spine and ...-bit.. H~inlla
MR" .
C O NT RAlNDtCATIONS TO MRI
P repaDcy aDd MlU, Dunng the lirat l rimuter. MRI can Clute rubtorption of prod·
.. UI orcon~pUon (..uK.mlte). Thera.A. no " udin to deta rmina thalOf\i tann efJeclli
ofMRI On a fet .... al'ler the firlll.nmester {the low ri,kofMRI in this,ilu8lion II problbly pl1'(... bLe 1.0 the knowo dan~rI orion;lin& rMfiRlion of.·ny.{including CTl"~ eadolin·
;wn eonlnll it ronlrW~tad dun",.U oIprflT\,nc.l', and lit not IPpt(lVed for u"" in
*II < 2 ye.n. SntNl·fftdinl mut' be inlem.l p\.Od for2 dly, after adminisuation of Ka· do!in;u," to the molbar.
NEUROSURGERY
5 Nlutor.diolorr
'"
Cont.nindieutioM to MRI! l . cardiae pacemaker, implanled neuro.ot;mulaL
6. sh ra pnel; BB', (tome bullets are OK) 1. reJlIliv~ oontrawdicatioua:
A. da"atrophllbic pBtieDI.S: may b., abl~ to sedat.> adequately to perfoftU nud)' B. rntically ill patH'!nt.s: ability to monir.o. And BOO!!811 to pllit.nl are impaired. SpeciaUy designed non.magnetit ventilator may be reqIlLN!d. Cannot Ill;(! m
ANEVRYSM CLIPS AND MRI
MRI conaider~tJol\I in patients ...·ith a cerebral aneu,),,,n, clip'
Tabla 5-4 Magnallc remnance 01 ane urys m cli p'"
I . the d/lnger oCthe MRllllilgt1et.ic: field clip to be pulled or torqutd off of the anellr)'Sffi or to tear the
..,k .rlifBC~ produ~ed by the metal or the dip in the magnetic litld 3, beatgen.era~ ill the regioo of the di p: not elinicRlly . ;gni»cant
2. the
Th" moTe fel't\lmagt11ltlc the dip, the IlLI'gu lhf rorce exerUld on it by thema(!llelic lield and the gre~ter the image di.lltortion near the dip. Sta inless ateel (55) i. da&Sifi~d PH m arteDBWe (ferromagnetic) Or IIUJltenitic lnon·rerromagnetk). CO)balt.-baSl'dJluperall(>yl lUe ru)Il·felTOm~gnetie and include ElgilllY (Sugit.a dipl), Phynox (Yesargil), and \·ari-Angle (M cfadden!. Tobl~ 5·4 ahow. thenUlgnetic ~mnall<:i! ofvariou$ dip. which it .... lelfd totheir lerNI.uagl1uic- proptrtiell, If in daub!. al the. time ofBMurysm lurg"I'Y, apply the following si mple test: nllo·ferTOrnHgnetk dipll canno~ be lifted Qr dragged with II 5II1.II11 magr>f'L. H EMOR RHAGE ON MRI One of the JtI09t c:ompjea: lll!lioll.ll to interpret on MRI . Su p~ 8ti6.
5.4.2.
Diffusion-weighted imaging (OWl) and perfusion-imaging (PWI)
O.inin8 usa8e in ischelnic brain disease (80me MRI machinu da not )'et have the n\!CUllary Vadien" fordiffuaion weightin8 or the apeed needed for both DWI and pv.'Ij. Dil'fusinn _w eigh ted imaging:. DWI illen.itive to random Brownian motion Ofw814!r molocule&, and an apparent diffusion roeffident (ADC) iA detennined for each areD based
OJ'
5. Neuror$diology
N~UROSURGERY
.n. number olvtrilblH {lime. I ll ..... unent.aliofl ... )". f'nety dlffuJin, w l~r (~.I. ill CSf) appta1$ dark on OWl. Areas of acute brain is~h.mia show up as lncnaRd algnallnll'! ... ity on DWI .. ithin m' nu~" I', DWI mlly also be able 10 dLstinguisli (')'totQllic from vBI5O~nit .,d em o"· " (_pagt 85), HI)W~"~r. racton o!.her lhan (Ot,1 i~~mi .. (.'1'. ,lobaL ischelllip, hypogly..... mi •• ltat\!5 epitepUcul ••. ) eat! produ<:e AOC decline lod 1.1>. OWl ;nlllgel rmllt therefore be interprtr.N in relalion to the dink.] -"lllin," . Some. bul nOlllll ", TIA .... ~iat.ed with abnonnalltiH on OWl . Oill'usion deftc\5 do not indkate irreveriibleirUury. bul imueates till$ue that ill !\ear cell dutll.
PerfW!;oo imuIPIll' P:o~idf!l infonnation rel.ted !.o the ""rful;"" '\.8LUI orthe microcirculation The ... are sewrll rnethod.eurrendy In u~e ...,th the bolUJI-<:llnlr1lnapPrtlach he; ... , t.he mo. t widely employed" , Ultrafangradient ima,in, UI u~ 10 foll_ the gradw.1 rtductllm loG nor m.1 fol1!IWin. Administration Of COn1rh1 (u!I.1II Lly •• dolinium), A .i~ nlll .... uhoOul CUf'I. is der.voo lind is compAred loG eonlrast In III lI.ury .
V •• In theQry. OW l nnd PWI maybeeomblntd I
5.4.3.
Magnetic resonance spectroscopy (M RS)
Thi • .e.:tioll . pec'f"ally COVerS protan (H'J MRS .... hith ~all be performed on .. lmostony MRI Kallller (eaped .. lI)" unita ., 1,5 1') ",im the appmprist.e INlnwllrll. Specl.ro6oopy ofo\.her Dude, (e .•. ph08phorou~) t.o be ev.lu.u.d only with s peci.lirec! eQuipmenL
T,bI.5·5 I
MRS
SINGLE VOXEL MRS
A • ..,.11 aR:1I is sel!'Cted 00 lhe '1l:OU1" MlU and the apeclrOfiCOpi<:. pe.... for that ~o . ... displayed in reltOnaOU as, fWlCt.i0Sl orparl4-per 'lQiUio~ ( ppm l,
Therd"o .... may be .u~ 10 IIImplill' emir. CUnielllly importllnt dwt· .cwn.tic pew uti d.hM.t
PATTERNS
Norma l brain; SH PI'IP''' 5 .1. T\uuor: SuF.,ure5-7. 1 NAA . C<'- _ _ _ _ _ ,.11actal.e, f li pId, f choh ne(ruJ. of thumb: WIth ,lloma., the lUther the choline, W hi. t - the rr-ck up to vade 3, thereal'..er nKfQIIUl reduce!! Ria. Ii"" choline Ievels."d U..lipid peak rull,. be ut iliwdJ. CVA: , lactate pe.k pn!domina\.el . Cholwe i, characllriuiclIlIy law.
Abaoceu" : Reduced NAA , Cr &: dloliroe peaka, and ",typic.l .,..aka" {$\I~nate, acetat.e.. .. 1 from bKtenal.ynthna il pathopomonic ror .b.ctu Inot always prellentJ. LacUI.e may to. elev.ted M o11tip le .cl!!"",;,: Bland pattern. NAA _llghtly reduced. Lactate and lipId _lightly fltvato-d . Choline oot el .... sLed.
NEUROSURGERY
POSSIBLE USES OF MRS I. differentia tin, .b_n from l'leoplll8m 2. poIt-op enh~nceD\ent va. reculTe nce of tumor 3. di.tingui,hinr tumor from M5 plaque.: occuioDaUy c.annot be differen tiated • . in AIDS: may bt: able t.(I help differentiate toxo from lymphoma from PML(PM L: I NAA, no . ignifie.nt illerene in choline, ladate or lipid) 5. !.he promie. ofd lfferentialinr tumor infiltration froID edema hll not been bonw
,"
6 . loroe utility in distinl{Uiahing tumor from ndi.tion MCfOllia (SH ~ 535)
"
" • " • ."
-'"
~
OJ> 15 U
U
U
... ' 0 01 00
(A) NORMAl SRAIN Figure 5-7 PrC>lOn MRSoI {A) nonnaI brain, ""II (6) high Ii''''''' glioma
MULTI-VOKEL MRS
Colorroded KIn with &eleded overlay for NAA, eholine .. [email protected]\>« risk ofumpling error.
5.5.
Plain films
5.5.1.
Skull films
Tabla s.6 Norrtull d imension. 01 . he aellll iurcica ( _ Pl9ure 5-8) ~
S ElLA TURCICA
o {depIhj {mm)
NORMAL AOULT DIMENSIONS ON SKULL X-RAY Teehniqua: t",. late.. L, 91 em u.r~1.O lilm
Max
Min
" •5 "
An
10.6
d.istan«, centnl AY 2.5 COl anterior IDd 1.9 tD1 lu penor 10 EAr.!. See FiI"'" 6 ·8 for mUSlTation oflbe diroentiont. and Tob/.~ 6·6 for nOr· ma l vIlu ... Deptb (0 ): defined .. Ihe gn!atel~ mua urement from Iloor IG diaphraama seliH. Lenrth (L) : defined a. !.he gn!att.t AP diameter. ABNoRIML FINDINGS
Pituitary adenoma. tend 1.0 enl'rfl:e lb. sella, in «Intnost 1.0 ~ ..niopha.ryngiomu whkh erode th. posterior clinoidt.. Empty ..lIa .yndrome tendll to bIolioon the sella . ymmetrically. and "so ~ noterode the clinoid..
1)8
5. Neuronod)oloty
NEUROSURGERY
al50
",f' ahspell seUa 8U.tiM'- opti~ nuve glioma. It o;an tongenitally In H urler·.$aynlirom
oco;,W"
polysaccharidosis).
MISCELLANEOUS Water's view: x-l1I.y tube ungled up.~S· lperpendiculli r to divusl . AKA submental verteK view. 1'91"01 '5 'tiel" ; K· ray tube angled down 45' . 1.1) vie .....
occipo,a. BASILAR IMPRESSION ~ure 5-8 M.nu,emaOl5 01 Several conditiol15 whose nomu are often (eNtlnem. sella IU'dca (lalora! view) OUllty) uged Interchangeab!y(eKact defirutionsara nOI en· tirelyagreed upon for all oCthe!;l!); 1. plnlyb8.llla, abcol"Ulal bll6ilar angle. OCHllle mOldkal i.:llporta,,~ (usO!"d in an· thropological dalal. May Cr may nOI btl s!i/!oci Med with basilar !mp~8!l ion 2. baallar ;mp", " ioZl (B1): UP"'lI.rd di&placeme nt offo ra men magnum IlIl1rglrur (i nc ludiog ocdpit(ll bon. l and urviclil ,pine lineluding :Jdo[ll.Qjd process) inlo Pfossa. Some use thi s term for upward di Splacement of dens only. May be selm in: A. tonpn"ital condilions (BI is !.he mon tommon congenilal anomaly of lhe crani
L
Down'. !\)'ndrome
2. KlipPl'I·F"il ~yndrome ]stt pGSe 119) 3 . Cbiari malfoMUation (.n fHlC~ 1M)
4. ayringomyeHa B. acquired conditions I. 3.
rhe umlll.oid ar1.hriti5 (in p.&r1. due do 'nccmp.elence oftransve .... e ligsmenL..aee Brullar imprtuwn in rh eUtllOlOid orlhrilU. page 339)
2 . ~t-:raumlltie basilar invaginAtion , AKA c r anlalseUli nl:: upward indentation of5kull b!W! usuallY due 1.0 a~uired IIOftenmg or bone. often allllodat.e
A. Paget's dlseDse B. o$teogeneliis imperfecta C. osteomala
E.
hype.tpan~idlsm
Same IO"asu remenl.& of ...... (rerer to FiG" '"" 5.10, page 141, and Figu,",,5·9 below/: I. Mc Rae's line ("MeR" in FiellrY. 5· /0): drown acTOlls rammeD magnum ttip of clivus (buion) loopist!Uon )'". Should 00 :> 19 mill ( avera~ : 35). No part of odontoid should be SOOv t !.hisl'ne (th e /DOIIt sccumta fo~ 2. C hnmber laLn·.lin e ("CL" in Figurt ';· 10)" : posterior hard pailite \.(I p05terior margill offonmeo lIIagown lop;&thlon,. l.cno Ilio n 3 mm or half ofdena should be above thill line, ..ith 6 mm being definitelY p,uhologit. Seldom UKild be.cau8e opi sthion i' ofu!n hard to we on plain film IIna mayaJso be invaginAted ) 3. McCre£or'. bilKli.,,, Flgu", 5-8 AP Yiew ih,ough ",al'k>cervk:al lul"lCtion' (~MtG·ln FisurI5·/0"f"': POi' FOOL ", FISd"IgOId'. divUlric line. F~L ~ FiScilgOld " tenor margin of hard palate _.~ line, " ~ , .)CIaI 0Wft>a/!g '" C1 CIt> C2 (SI!' to most CAudal poinl ofoo_oi~PIIIl·7Zl) ciput. No more thaI> ~ . 5 m.m ofdeDll shOuld be above thia 4 . Wac k e.,belm'8 e livu8-<:a. nalline t·WCCL· in FicUNl5.10): tha odontoid ahould be tangential to Or below the line that utends the coune of the cliy us (the clivus baseline). lftbe divus.is
ao
NEUROSURGERY
5. Neurondiology
'"
colICa"" or OI)nVU, this loNI!lin" ~ dr,wn to C(I"n""t tlte basion ttllhfl ba~e or~bll poIItarior clin
5.5.2.
Lumbosacral (LS) spine
LA-5 i, nonnllily the lumbllo. disc ,pace with thlllf1!lIlOSt "frttul height . Abo_
.,.g,
N(>J'mlJ/ LS .plM '"~twu"""",,,", 327. AP view: 1000k fur defact Qr non visualhlllion Ofthf ·Qwl'. IYes" " 'hkh i. dUil to peeli. c\e.erollion wlUch may oecur .. Ith lytic tumo •• (rommon with metutau( dl.waseJ. Oblique villw" look for diarontlnuity in n""k of"Srott)' dot" ror dero!(:l in J>WlI i"!.eran;eularia.
5.5.3.
C..splne NORMAL FlN OINGS
For radlolftlPhitllignl afO! ..... itlll ~pine trauma , _ Tablr 25.1, Plllf 706. and guidelintl" ror diagn~Jnr dinical h1/;tability,.see Table 25·11, pIIgc 13• .
ro..
CONTOUR LINES
On a lall1ral c.:.pine "·.Ili> tl>o. ... an!" ronl.Our lin ... (AKA arcu.!.e liOQ). Non..aU), f8C"h should ronn II smooth, pMle "" .....e UN FiIlUrl5·10); I . po6terior mIlrIPna.llin. (PML) ; _10113 poIIUrio. Cl)n~ lunace. or" ....I~b ....1 \.Jod. jllll (VB). Marks the uteriOf' llIargin or'pinal eanal 2. a"!.eriOl" margiruolline (.4.' 1L): alonr anterior cortical surfac:ee of VB, 3. "Pi nQI. ",;n ll . lin ", (SU,: along base ohpinou s proalS ...L Tb. pIIII!.erior ",argin of the . pinal eMal 4. pclllLerior spinal.ll line {PSW: akmr lips or s pinaul pro<:I!Io5N R~I..A TION OF A 1l.AS TO OCCIPUT See~
119 lOr erikrill for atlantoaaial di,IOCllu..n.
REl..AT1ON OF AnAS roAXIS Atl an to-d llDtal i n terval (.ADO '!be ADI il the d~tal>Ol! between the aot.e.rior margin of the dens and the clO6e!l1 pOin~ "rtheantemr arch orel ("Cl bUlton"lon alatval G-spine " .... ), Ute Figurl5·10). The normal mPill1ll1 ADI uvanoullly in the ran~ 01'2 to .. mmll.". Commooly a~· c:ept.ed upPf'r IiJ;a.ita ItA! show .. in Tabi~ 5·1. Atl . .. IoUi..... u bh.... lioo IAAS): Two POK;brlitiee: Tabte S-7 Normal I. AD I :> norma l' may Qtt.\I.I" ... itb inmmpewnc:eorthemns~e. . li"PlenL (Ammon in rheumatoid arthritis /.Ite AOI P"lII! 3381. may llao roUow uaumal_ ptl6C 722) , ....." 2 normal ADI ' i .. tb_ prllHTlUQfan odontoid rractura "" /X'R' 721) .,~ "'V" . b .pe
"'' ' eI!
, . ."....... ""'"
poII.terior lia'~tou' ~p,"
"'.
S. Neul'OT'adlolop
-- ..c,.
NEUROSURGERY
WCCL ,
..
AD'
KEY
(see
te~
fo,ootalts)
ACI" aUaNG-deolaI interval MeA " McFUe's ~ne MeG " t.lcGrego~s line CL",Chambeilain'S Une WCCL ~ Wackenhe!m's cf'NUScanalw
fl.gu r. 5-10 Sponul contour 1InII$ IIOI'l linus vsed 10 dilqlOSl'l basilar lIw ~glr>Bllon ult." ';ow I"""-'!I~ C>'~ jun<:IIorl
CANAL DIAMETER
Normal canal d'amew. 00 laternl Capine x-.ay (from spinulBlllinar line (SLL) to pullte rior ""neb.nl b".!.y with 6 foot wbe to mill dJstance)A: 17 '"' I!I onm, In lIIe p~noe of osteophytic IpW'8, ~BS\Jre from the back of the Spur to tbe SLL. Cervic al s pinal stenosia: varioul cutoffs for !.he normal minimum AP d iameter h,,·. been 8ugge!t.ed1;l. On a plain lateral C-a piM ,....,.y tbio i$UliualJy measured from the posterior vertebral body (or !.he posterior aspect of an o~t.eoph..vteJ to the spinolaminar line. Some ull
Abnonnnily increased preV1!nebral tissue (PVST) on late.al C_spine xr>IY may inditllte t.he preseuce ora verte-
1 0ft.
Teble W
NOm"lal preverlebral ,oft Ilssue
bral fractW"e, d islocation, or ligamentou l
,,
dis ruption". NonuaJ values are shown in Tobie 5-8. NB : the IU!Ilsitivity ofth.esJe measurement'! ie only ~ 60% a~ C8 and 5% aLC6" . Increased PVST i . more like· ly with anterior than posterior injwie5" . FaJae pD.itivllS may (Je(ur with bessl skulVfaci al fr8~tUrl!'B, e~peciaJly with
NEUROSURGERY
.5. Naurorad iology
'"
rTliclure of the pterygOid
pI9te~ ,
INTERSPINOUS DISTANCES C·~pine AP: a fractl,lUldi,tacation or [jgarnent d1i!ruption ","y be diagnDSe
spinous pr~ l"", Aleo look for II mallllignment afspinoWi PI'D'I'I'$!Its below" certain level which may be evidence ofrot.auan due to a unilAter1lOl1y locked fa .,.t . C-sp'n e lateral: look for "fanni ng'" or -n aring'" which ~ lin "bnotma] lipread of one p~iT of "p'nous pl"OCt'lSoOel that may alao iodicate ligllmenl disruption. PEOI ATRIC C-SPINE
C1 (ATlAS)(8e
synchondrosis: of sillflDUS Pfocess
F'1Iuf6S-11 C2 (All iS) C,.,e FIB"'" 6-12) 4 primlll)' naificat;on cenlers: odootoid P""""" ve.whr,,] body 2 nE:uml arches A ReCOndlll}' QSljl6cst,on oenlo!r 3pppaOl at till! iummit of tile dens between 3-6 Y"". Iilld fuse~ with dr.os by age 12"'. Syncbondro!iEtl: aonnaJly fusa between 36yeal'l;orag~.
AP VIEW
....
~ neura
P~I "'ieC1
g-
[alias)
neurocenlra)
s ynchondrosis
. """
.
derllocen!rtll syncnondrosls
~I ~ nau I1eurocenlral synchondrosis
FIou,. 5-1 2 pecillmlc C2 lllXls)
C3·7 Co!rvical bodies a.re normally wedge shaped in pedi9t.ric populstlon (narrower anteriorly). Wedging decrealu with oee.
5.6.
Myelography
L umbar myelogram Using iohexol (Omnipaql.lO"!® 140 Or 180) /U showo in
Thbl~
5-1.
Cervi cal myelogra m with wate r w lubl e contril8't v ia LP Use iohexo\ (Om.oipaque® 300 or 240) n sbown in Tobit 5-1_{moen spina! need(e into lumbtar !ubaraehnoid Il'pace, tilt the bead o(th~ my~lllgr.Im ta1>le down with the pa_ tient', n~k extended and then inject dye. Ita conlplelectrrvical block i.'I5~n, bll\"e pa. 5. Neuroradiology
NEUROSURGERY
t'~Qt flex neoek. Irttle block ClilIlJOt betTue~d , patient may need Ct·2 p\lllc:tuJe (IT MRI (firttobt.ain a CT ",hith may 8ho'" dye above theblock VtlltCWlnQ! be appreciated (In my. eIOlT,phy . IQQe).
Poet myelQgra p hic. CT u,errallY Iellsi tiY ily and l pe
5.7.
Bone scan
Te<:hnet.ium·99 ("-Ttl pertKhnetate ill radlolllOtope that IfIIJ' be attached to v_ io ... l ubst.r&te . r(lT use in bone .cllnninJ' It mllY be ueed to libel polyphOlphate(nttl,y uled l«I.y), diphQlphonite" (MDP), OT HOP (the rnot~ "'Idely agtnlll~ cutn'nuy). Bone ICIM ",ilh technetium·labeled compound. depend. 00 the p..sente of oateoblaillc activity for thede~iUon of tracer into the bQol~ GIlIium may alao t;.e uSl'd 14 look fOT mort d]fllnitOlt.oo~hti •. ApplleatioOi for bone 1C
infewoo
A. O!1teolDyeli U-l orth•• kull Dr I pine (verubral OIItoo Dlyeliul, aN ptJI8 244') B. dillCitis; 1ft pap 247 2. tumor J\.. I pine nle\.8$t_=ut p~ flJS B. primary bone lum ~ 01" the spi ne: _ P"P 6 12 C. 6kuU (uman: ~ fKlI/t f8Q 3. d;.\ot'QeI involvir.g aboormll bone metabol"m A. PlIJlet"5 disease; ohhe ,kul! (1ft PQ6t 3·U ) or Ipine (1ft" ptVJe 3411 B. hyPerOiltosis frontalis interna: ~ ~ 483 ... craniosynOl!-tQsu; ut Pfl#. 99 5. r.. tturn; spille lsu P<>I{t 701 ) or I kull 6. ·10'" back problema~: to help id~ufy ... me Dr Lhe above esamplel (.... p<J8t 2!U)
5.8.
References
R, ...._ £.Honl;I_ Io4 .W .." eK ....... _ _ orionc _ _
;,,",~
"""ond_,....... .....
I.
.
pIof. C_ .. IiDu. ,.. ........ ~,p<]"16 0I)·,. 19U. 8aI"OI Ii P. R,a L. Soijof.... J
••,iMII
noocI;.,.,.~
P'O(ICftIt. """ -... ....1_ J "" ........... lO. 'If>.ll . lm_
I I.
__
Il
_
"""
_~
.....1
,.. _ _ .... .-.joI
... )I 1"
U.
,..u~JM
,.
4.
A~ . . ."'"
.......,r.,.,...,""•.
..
Ij.
"""1OOo T H.,", OG. "odI.., ~ .. .... .C. y Mo!.llr.s-. ....... IVII
,hU_ .... "
10
~
9, 10.
_""tl1 "",,,,,, . loe. . SoII~.C. , . UUI\.. !IOOl: p"S6. 8010"".. .. 11. .......... s. 04."";' _ . . ..
.".,f'Irto ........ ,...) ,.. £ot:l J 101.,. m
''''
_ _"
NEUROSURGERY
"" H. 8 _ _ FS.S
_ __
$t_ ' W. ' _ f .s.Gctoo.ok>R G. .. ",~
.......
....... ,.....,. ..... "".
o..m-._1pI
~
S" ohlS. 1011·5. 1')97 "'t ll. a - FS. _ _ C . .. .,t _ l l l If· """""_;pit
top-""""'_
_"""puf"'_""~ MIII ~""oIOc!" n:
17
1792..... 1999 _otn _ _ ., _ _ .._ .. _ M .........,..' I, _""A."loMol .......
m.·_
,_
_16,
0II_1oI . \.JlOOC$<"~8 •• , ... ~ _ _ ,i_os .... _....,.,.
1<001-..11
1l69·76. 1991.
_IIF.Fe",..G .s...wt.." ...... ~ OompIka
IId_ ll F.e:;-..LLs.:_O f ... ., " .. _ .. MIII,.... _ _ ... .. sa..l1od _ ....Froe:;· .. _ s..''''''''''}·115-M. ___ I__ ItIllIt , ...
I\I\19.
UOi"'.
II
50""'. II ""·I(M)oI. 1"1
..... of.-bnl ... d .. _ ...... ofl"l>k. AJ. loOl. W·'J, I ....
1731)..6 .
......""'pIIoI.podI~ """"""-....~~:
W.'.'''1 Dooo I£ .G_p c: r ... "" . ..... o..uc.l_ ron...... ~~,
~ ): ·
PndIon1IW. G _ RI. "" ... _ Iot:~ _01 101111 . _ N.... '*'CJ 3l 11)l-6. I99'i
plOJo "Jl'IR U; IJ I)"9.1M"1
u..... 6C, IImtCC. T",-\. e.•, .." ,..,._.
f".."..IoI.AIt>«>GW· ...", _ _ of
It
R
10/1·
1). 1997. """'k VC.KopI.>MCE. M............. ..,".... I. . JII_I&fto
_1_,.,oI,,""'_."' ..
S. Neurorlldiotogy
".
M""", K A E.. ••• 6<"" ~ ""'()T , L")fi"J (jI W. .. 0;,10<", ... or.". """ltal .,.... w '", oot
ttI ~
l. fI'c1, ... O>i
p
~
......... II. MoIltt ,., O,I(nt]O<7I1.S.".,J , Tt.. _,~ •• "'fIOI .. ' .... o._ip, .. , Gi.lon"" •. :-...' -. .." ~,
" " "
':4.
JOI.' S.....
12_1,19'19
1Ioh,., s P. K,k," M O. M.oIIi.
...... ~ SIo: ..... , Rod,IoIl • .
W 'Y ' >I\lpc.j!>'l"
"
..
,
~
111-l>.lqsS.
ScI... od< k H tI, S" ... W H. (0.1>.). Op. ... ~ ........
....~rrI ..,l«h"'q_ 1...".G" ",, ~S"a"." . W .. Yort , lqll
£1""'" "', ......;.1 ",800,_ y . .. .!.. T_· it III)'< ..... OIIy io po"........,," ....... , ~ ""..,. ,'" wi.,."., f_"", ,,,d,_,,,o' ""<""",,or dl.,.-. . .............. , >ad _ i s. S..""" I'm) 0.:_ 1( , I{;,.-.:' C li1i1j" '" p
""-!IIi.
". " "
" "
111 .1 ... 1( A. FI .... ~ D.1'I"' '''cC!<:>I oof, ,...... , ...I~ .. . _1\11.1 •• '" I";""'of ... , ..... oa/ 'P<"'~ I I\I~'} 19' iTJ·9. 19II1. N';"i
bdloiolp' Ill: I iJ-
~,,'"' 0;....,.'100 .
6, Nellroradiology
" n.
" " "
IWl.
J~
G, 81«k..- H H; P.......,........... _of ....
.... IIIc:b.i~ . .. "' JII..... '~
.. DL
>OW ,., <~
Th<~ lnir"
n . 1z..3\,
__ .f.b_I".... ft ..
«n-kol ",' r><, ""II 70. 'D~, IQro. c..mben.,. VI E: 8 ..11. '"",,",,"'" I,p....,bbio): eiwre "" ..1o;wnon.1I ...,.".IJ' o f _ip i ~ _ ..... "~ r
0;"" ......u.a .._ .
1~J9 M~JOf II Tho \o& ..
'11-%,
f..-_ 01"""" " 111''''' __
"'II,,, of Ibo ,~.u in !lot dl'l""'" , ..... . ,J MNkIIZ,
'" w ,l .. I..,!I
""~_m""..,_IN.I~.""'.I" TkrnnIeo. nob, .. ju ..""" &lid ... . _!nOIiIlol. F......
I'\ItIIW,i • •• foI ... . 19J1.CM!U' l! pp19-61 lI ... t VC. llopl:J"' C E,S. .... nS. I>iI""",l<m·
"')1 ofl)l>.I... .mp<e .. ;OII •. - . . , . 15:!19. 1961
II.ondo I, V............. I. Mo,; .. II... al ~ _T<-pd,.. ~...,.,
99oooTc"",,.,.,.pIIo"" _
..1",~
t>Pl, ;" ...............1<&1 ptlaiI:< 500ra N.....all; lD1_ 10 . 197• •
NEUROSUR.GERY
6.1.
Electroencephalogram (EEG)
Common EEG rhythms lIrf!shown in Tobit 6·/ . The primary use ofEEG i~ in !.he diagnosis and n1DnBgemen~ ofseizur\'. diiordetll . Non-c:orlVuJ~ive use of BEG I, ellilcntiaUy limited to monitoOril1g for bun;~ 8upprullion <su~· /OI"! (e.g. during C1lrotid endarteuctomy )o r for d ifferential dingnOliis of diffu!'l! en~~phDlop.t.hy, Including: 1. differentiating psychogenic. unrespons;veneas from orga ni~: a n'mnll) BEG indioate!! either psychiatric unruponllivenu~ or locked-in 8yndrome 2. Mn_nvu!eive ~ tatU8 epHcpticus (seiJurea): R.b-
Ta ble 6-1 Common EEG rt'IY1hml
sente or romplex partial sta tus 3. Bubclinkal focal abnormalities: espetially in patients too ill to be u-anBported 1.0 CT. Loo~ for e.g. PLEOs l.su ~Iocu), foclil slowing ..• ~ . specific pattern, diagnotltic for certain pathologil!'ll; e.g.: A. periudic laterali?;ng e pileptiform dillChargeJ (PLF..D8): roay occur .... ith any ac:ute focal cerebra l insult (e.g . herpes limple.: entephillitis (R SE), lIbM:ell$, tu mor, emholieinfancl: .een in 85% of calleS of HSe: (on~t 2·5 d ane.. pf9Sentation), if bilat.et"sl i • • disgcll'ltie orHSe: B. ."baeut.! adel"Ofting pDnen~ephalitil (SSPE) (paihognnmonie palt~m): penook b.lgh volUlge with 4·15 ~ IieparntiOll with Il«Ompanying body j{:riur, no ~hange with psinfulatimulltion (differential djagnosia includes pcp overdOlic)
C. Creutl{eldWakob diseAse b«J108f 227); myoclonjcj~rks. EEG ~ bila!.CrAI ahArp Wave 1.1... 2 pe r iecond (~a rly - slowing; lal.l!r- triphMie). May ~ semble PLED •. but are reactive to p~lnrul Itimulation ImOtit PLED. au not) D. tripha.ie WAV"'" not r~ally sp""ific. MIIY beseen in he""tieentepbalnpnthy , post.-lInoxia, and hyponatreml9 5. obje-ctlve mea3ureofaeverity of encephalopathy ; u8ually used for anoxic enceph. alopathy (e,g. periodic spike. with sei~uNS ind.katf! &<:;'" chance ornonnal neu _ rologic outcome , with high mortality), Nphll coml1. bunt auppresaion, and electrDC(!rebral 1 ilente an all pOOr prognO'$ticatorl 6. differentiating h.ydrllnencephaly from severe bydrocepbll.1Ul ( _ Hy1ronf"",pll. 01.1', poge 180) 7. u a tUnical eonnrmator.l' test in the determinat ion of brain death (,sec pall~ 166) BURST SUPPRESSION I_Je<:tric intervals interrupted by bursts of a..~2 Hz e1~;rir.a1 activ ity tha~dimin· iah to 1·4 H ~ prior to ~lectrical s;l~no;:l'.l . Often u~ed a8 an ~ndpoill t {or titrating fleu r(lprtr teo:tivt drugs such as barbi turotes, etomidate... (e.g. IU page 807).
6.2.
Evoked potentials
Cli11 iCIIJ indica tiollll for !voked potential. (EPa). 1. di agcosu. : (MRI has largely replal.'ed EP. fnr these 3 indica tion,) A. acoustic neurOma B. "" bdinic:.a llesiolUl of multiple , dl!l"Ollill C. brn ins!.Cro lesions
NeUROSURGERY
6. EJectrodiagnostia;
'"
2. ,nlra-operati ve U$oll bu ~Iow) Table 6-2 Evoked pot ential waveforms (nOle ' values may diller Irom lab 10 lab)
",."
''''
Possible- geMrtlou M COChlear microphonic
.~"i~1 ~!\I'P5 P7 .
II~' I V
P, cislalVllinerlll. Pl Jlf(liltnal VIII or ooc:hIear I1\.1dOO$, P, lower jIOI1I (1 superior olivary c0mplex).
P, mid·uwer pons. Ps upperpoosO:- inleriorcolIiculus
IOmS
Nv (on frEp where E. ~ E.Ib"S poi01)
UESEP
~
C3'-FP---""-"" '"' < . ~~9_VP22 eart1 -
~f\-N~~
~
"" ~ 1"''-/
AKA EP: o:nIry 01 volley ..' 110 listal braclial pieXIIS, 1'4" (on CvrFpz); roo! entry lone (cervical region), N13 cerW;orneCulary~nc\loII. N" prinarysensoryearlex, P~ (early) motor earle., P~ (\al.) IPSP "reaction" to NIS
Fz-Ep
I
I II I I I "mS
LE SEP o.7.fPz ~ ~ r--.~ N2S-WN27 -:- I,U5uV
Cz.FPz
~ 40
l5-TI2~ t
I
I
I
~12 (on ls·T,ll; luml»S¥;fal pIexvs, POll (011 CrF.z); sensory COIle. (analogous 10 N18 i1 UE SEP, reYeIStld in polarity !or ? reasorr), Ng (on CvrFpz): ' doIsal column rlId~
N
t
I
~mS
..-
100 striale & pre·striale occipital C«tex. wilh contr\bUlOnS !rom 1ha1amoixN'-
PAVER
INTRA-OPERATIVE EVOKED POTENTIALS
Also, ste p<JB
...
6 . Ele(:uodiagnostics
NEUROSURGERY
o:>Verallaplnal tord funeliOn and ar~ a lTetted by manipulation or the .pinal cord and l~ chemie ev~nt.l, they mal' r~in un~hanged with aome injuri" to the anterior cord, ~anscran;al m otor e voked potentillia (TCMEPa) : tran&a"anial electric .. l or magnetic stimulat ion of O'\otor cortex and delcending motor 0::<0n8 with rerording ofmGtor pltentiuls from di8~1 ~pinal cord Or m.uscle group!! , OUl!. to thl!. large potentials, th e acquisit ion tim" is shorter lin d feedback 10 the iurgeon ia almas! iruooediate. However , due to pa t ient m01li!ment from tho muscle contnlttion s. contim:oous recording: bI Uilually not possible {except with mOn.ilOrinjl' the re1IponU over the spi nal !;Old ), U~ful for iu rgel')' iovolving the sp inal cord (tervlcal or thoracic), 110 utility filr lum bar spille surgery. In addition to g:en~raJ EP anellthet.ic require",eolJi, n.mmmugcuhH blockade ,,,u8t mini_ n:>i~ to pennit a. 2 out of4 twitchel. De-cending evoked potentieJ. (D£P): (form erly refelTed to by the misleotding tern:> al oe uropnic molor eyok~ poten t ial,). Ros~al5ti!l\ula tion of w apina l corti wit.h n<:ording ora caudal n~"rogenic response from thl! s pinall'OnI or peripheral nerve, IIr .. rnyogeni~ r"l'lponse from a di1t.a1 mllKle. OEP. can be medial....:! primarily bl' IIIl1SOl')' oervt'B IlIId the""fOJ"~ do not rep.....senl true motor poten lial.s. HQweV8, ;r.lwwn 10 be sensitive to s pi nlll cord chlL~gft5 and may be "...,ful wh"" TCMEPo cannot be obtained , ABBREVIATIONS Abbre viationll1.l5ed below: BSAER .. brain ~tem " " di1Al1')' I!voked rO!b~: lJEILE SF.P .. upperilowI!T ..xt .... l'Ility senso!)' evoked pot.ential: PR YER _ pe tte rn ""versal vi · Bual evoked response whi~h cequir"" patiP.ntcoop
"''' UESEP LESEP PR YER
(nole: values may dl Ne< trom lab to lab) Pamnettfl lIINaumi -- Normal ¥8111tS --
"".
~2.511k1
Com~'
dey
I-Y pe.D: !alelq'
4.01 mS
H.pe3~ 1a1eflC,
l.\51!1S
4.63m 2.&61115
V nbsolote I~~ IH-V peaIIli!1erqo
S.1mS
6.27 m5
tfs·N'1 pealIlalMCY
9.38 m5
PuP. pe~ 1i1!!ocy
IS.62mS 2O.82mS
jlIOklrqaIion $U~ lesion belween pon$ inO coIicuII.G. oIkt" K_11e neu,oma proklngallOn ~t:; Ie:siotI be~ IoMr pons"& midbrain, may be seen irllo1.5
11.35111$
P.., absoMe lalerq 3720mS 44.16mS P,oo absolule laWlCV .3 S.D. P,OO i!118'~)'e dfle,ence 6-10 mS
1~·eyeddll'l$'lOO is mom~"~hluil
field Slimulauon. Monocular delect so.ggesli ~ de!edif! Ul.loloplic oerve anlMor to CIli~5m (e 0. M.S" g1i..eoma, ~pre$IOOI'Il8lifW cegeneralion). Bilale!i! de!ect does nc)llocali.l:e. ••
IIOIm" ... Iues on _ _ ... cnliCl l "alUM
6.3.
u~
as eutaW I... aDnDImli _
Eleclromyography (EMG)
There lire two po.lrtionB 1.0 thl! EMG exam: 1. conduction lI1eI,,;Urements 2. o .. edle exam A, ioserti01l1d actiVIty B. activity at refit: IhouJd be I lle nl when need le Is activity iuo3l1uieteoi
~talionary
a nd in :\Crtional
DEFINITIONS
Fibrillati on potenti"" a: following den~rvetion ofa
NEUROSURGERY
mu~rJe
6. E lectrodisgflostica
(""condnl')' La II n!!;TV"
'"
injury). individualm utcll llbe .... begin firing independently . .EarliHJ,()/\IIet 10 daY".
lOOIetime. up to 3·4 week.. after denervation. If tM nerve re<Xlye ..... it llIIIy reinnerva~ tM mu..:le. but with larger motor units N!. uiting in longer duration a nd de<:rea~ num· bon. F. .....,.: ~rve it . timulated. cauling or1hodromic and antidromic cond uction. Fwa"' l latency may be prolonged In rlldiculopathy (not IIIn.ili"'I). SNAP: te nlOry nerve nction potentiall. M YOTONIA
TheN! are . number of myot.onic condit.ion•• induding myotonic dystrophy. There i. sustained con traction of the mu scle. Clnuic EMG IInding: ·di .... bomber" IOUnd due to myoton ic ditcha rgu. LUMBAR OISC OISEASE
Al.o .... pagt 292. SNAP may be normal ... the injury i. proiUmal to th. ~n body which ruideli in the do ... al root ganglion (in the neuul foramen). Pa. upi nalmullclo! fl· brillntiona mllY Ottu •. Following disce<:tomy for udiculopathy: motor poll'ntials retum fi ...t (if~rvl injury were · compl.te·, it would u.lle 1 month to return) if lost. senlOry potenlial, retum lut or may not relum paraspinal potentiftl. m.y no longerbeuseful because the muacll'tl are cut during surgery PlEXoPATHY
Reductioo of SNAP with DO paraspjDw. musele fibrillal io,,- (the donal ram i Gi t proximally to innervate the paras pin. l•.• nd aN! involved ~ W with rootl..iorv). MISCEt1.ANEOUS EMG PEARLS FOR NEUROSURGEONS
The short head orth e biceps femoril in the LE;I t~ Ii.... t muscle inner-lted by t~ po!rooeal division oftbe !(illic ne ..... e at or just above the poplil.e$l fOUl ju.t after the aplil4l offfrom the sciatic ne ..... e. In cases e.g. offoot drop it is a good muscLe to tesl to determine if there it a po!ro"",al neuropathy VI. a more proximal lesion (i.e. above the plplit.eal fos •• ). EMG ii not extll'JDf!'ly sensitive for radiculopathy te.g irritative radiculopathy m"ht not be picked up). more !<) in the cervical region than in th.lumber region. However. when positive , it i, very epecifi<:.
M ......
6.4.
References
o.-pnl_ H """_ ...to ...... ;' ...... '1'_ ""IcIoI _ I. M_. ~"",,,,"
8 1i" C D. (ed.l. o....t..ll u, ... _. New YOtt.
19":,. n}-ol).
'_af_'_ ..
1.
c;",toriEM.~S· Lo<"i ..
1.
J""'~"-"''''''''-''''''--''C-. _'-'11 6, . lOO1·S<. ' 9I< WooIM,C N.Enol _TC.O_ WE; Lo
148
,_ .. _ i r ' " - l ' ..... _ _ af-.-
6. ElectrodiagnOitiea
«tdnI_ ........"""bydot
a..-KH:E""'ocIp
l r... af_ J*U) NI:IoiIIJM«I lO6: 11000-50.
""_K".£_"'..........
int
1_",,_,...1 NE",IJMotI lO6: ' 21»·11.
'9$1.
NEUROSURGERY
'"
-"
---
..
~
-', Also_ pag.153 for plumbi&m (lem! poilloninll frotn At.al ned bulLelli.
7 .1.
Ethanol
The acute and ch rOJ1ie errKU; of ethyl alcohol (e~h.nol , E tOU) ebuH on til, nervoua .Yltemal1lprotean •• andarabeyondlhI8l:opIofth.i.te~t(nol.tolDl:ntion the Afl'ecb; of EtOH QfI othar ol'la.o .ylteml). Nell1'(lmulC:war a fTKUllndude l .cute in~""Uon: ' " ~IIIW 2. ~fTecUi of chronic. .leohol abu ae A. We .... ;e..e·. e a e.
ACUTE INTO)(lCATlON
The primary elfKI ofElOH 0<1 the CNS 1& depresaioo of neuronal eaTa ble 7- ' Blood ethanol coI'ICantratlonr. citabWty. impul5f! cond:.octioo •• Dd (In noOoi' 1oo/1oIIc pa.lletl1I) neu rolr.nlmittu releaH d.... I()ditlKl (blood EtOKJ CIInkll lIIlCl elfecaon the cell memlW'ano!S . Tpble 7· 1 MI;MlI the clinical elflKlI .-0,,1«1 Ibm IIlIIOO, ....·ith Jpeocifie EtOH aHlcenll'1lUoos. Mell.aby erred; the J~trilY of in. pniqnlcion. ~ !!Iaication i. greater when blood.1eo,. 21.1 1feSIo"..-:I oerebt.... ~ hol leyelll al'
.
...-, .,. '.' "
". ..,
_1IIIOlI:RI:!an:
~
-
ALCOHOL WITHDRAWAL S'I' NDROME
Compen18lion for the CNS tllpraua nl elrecUI or EtOH aco::~ in mrooic alcoholi. m. Coneequently, rebound CNS hyperactiv ity m.y rellull from f.mng EtOH level •• Clinical !IIena of EtOH withdraw.I,,1I duaified .. mador o r minor Ithe devae 6r.tltonomic hyperKllvily lind the pr~noe(.bHnceorD"l'll difl'erenlilltel lh_ l, as well .. ,.,1)'(24_48
NEUROSURGERY
7 Neuro!OX.oology
'"
hrs) or late (> 48 hl'1l). Signslsympt
BeozodiQzepineli BenzodilUepines (DOls) are the mainstay of treatment . They reduoo autonomic hyper. activity, and IDay prevent sejzure& and/or 0Ta. All BOZo are effective. Initial dOles are shown in Table 7·2 and are higber than those used for treating anxiety . Symptom triggered do~ing with repeated evaluation utili~ing a standard· iud protocol (e.g. CIWA-Ar") may be mOre efficscious than fixed ·dose ttchedules'. Avoid 1M administration (erTatic absorption).
Table 7-2 BDZ doses lor EIOH withdraw sl·
.,
Adjuncti ve m e dica ti ons Associated conditiolUl commo
3.
badofen: a Smallsludy' fouod 10 mg PO q d X 30daya resulted in rapid reductioo of sympt
'"
7. Neurot
NEUROSURGERY
A. phenytpjn (Dilantin®j: luad with 18 mglkg .. 1200 mgl70 kg rUe page 271) 6. eT.ha.nol drip: nol widely u.~d . S% BlOH in 05W, ~tart at 20 cdhr, and tilrat
When D'l'~ occu r, they ~uaJly begin withill4 d8y~ of the ~t ofEtOH withdrawal. and typi[8lly persist (0.1·3 days . Signs and eympUlms indudJ,: profound disorienta tion, llgitation, t remor, imwmnia, hallucin!>tiona, severe autonumieinaubility (taoby-cardia. H'l"N, diaplwre3w, hyperther· mial". Mortality i8 5-1O\\' (highor in elderly). but can be reduced with tnlotment lintlud ihg troating ~ted medical problem~ and t~t.ruent Ibr aeizures). Haloperidu] and ph~nolhia:unl>B mAy e,ont ....1 hallucinations, but can luwer the Mli. lui'll th",shold . HTN BIId tachyarrhylhmial . h""ld be tnsted as outlinellbove under Ill , c..,h"l ..-itbdr8walayndrome.
WERNICKE'S ENCEPHALOPATHY ( W E )
AKA Wemicke-Konlakufl" eneephalopalhl'. ClallSic triad; em:ephalupathy (consistIng of global ""nfuaion), ophlhalmllplegia, and atula eNS; 11113 are pT1lllent in only 10· 33% ofcuu). Due to thiomine de ficiency. Body 510",5 ofthhllninearead~ua~ only for up to - 18 daya. May M seen in: I 0 certain BIW:~pl:ib]1! Bubset ufthi!lllljn.defici~ntaIC(lbe>l.i<:ll . Thiomln.. dencieo~ here iadue to ~ tombinal;un ufinlldequatl! iotake, reduced absorption , decreased hepatic &wal:'l, ~nd impaired utili~ation 2. hyperemNi8 (oa jn lume pregu ancieU 3. s tarvatioD: including anor~~ia nervusa. rapId weighllo~. 4. gastroplicotion (bariat rk surgery) 6. neotOdialysis 6. can"""' 7. AIDS 8. prolOllged rv hypera.lin:..~nt&tion Oculomotor abnormalities ..,....,ur in 96'11> and indud .., nySt"fPtlu8 (horizontal > "ertj~ cal), lateral rectu s palsy, cun,jugat.!·gllZe palsies. Gail aLaXia i.IIseen In 87%, 8fld results frolll a oombination of pOlyneuropathy. Cl're· t>ellard.Ysfunctiun. 8fld veatibular imwirroenL Sy.t.erruc 8ymptomo may indu de: vomiting. fever . M1U : May , h(IW high s iJ,'llal in 1'2WI and FLAJR images in the pa'raveDlricular (madlal] thalamus,.the floor ofth1l4th ventricle. and pe ri aqueductal ~9y of the midbrain . These changes may resulve with t realmeDI'. Atrophy of the mammill ary budj"," may alao be seen. Normal MRI does not RIO the dlagnO$u .
Treatm ent
Wernicke'. entephalopothy (WE) III a medical emergency. When WE i. $ullpect.ed, 100 mg thiarnine$hould begiven 1M Dr IV (oral route ia unre/iable,sa llbow)daily for 5 daY8 . • IV glucose CIIn pr..clPltat.e acute WE in thiamine derkle"t patients, :. give thia·
mine fint.
Thiamine adJUini~trat.ion improves eye findings within hl>Urs to day,,; ataxia and confusion improve in day~ to weeb. Many palienlll that Bumve are left. wic.h horizontal nystagmus, ataxia, and 3Ih\ have Kurnkoff'...yndrome {AI<..... Korukorr! psytho9i,1, a diubling memory disturbancc Invoiving retrograde and 81Iterograd~ amnesia.
7.2.
Opioids
Includes heroin (~hkh i. usuolly iTueeted rv, but the powder can M 1ID0rteQ liT' ... moked) aa "'ell a. prellcription dru~, Opioids produce small Jlllpiis (miOllIll). Ovenloee produces: 1. rerrpirll.1Ory depll!$!ion 2. pulmonary "
7.
Neuroto~icollliY
'"
3.
0;:0l1\.li
• . hypotension and bradycardia 5. ."izurea may o«u r wit h: propo:q>phe ne, m eperidille (!leme rol4t) which may .1&0 aoUM deleli um, IIf>1i the Itreetdrug combinatioll of"'1'". and blues" (.et pagt 259) 6 . r.tal overdOMl may occu r with 8ny agent, bu t I, mOTe likely with Iyntheticopioids luch as (ent a.nyl (Sublimate'll) among "'$era unfamiliar with t heir high potency
IUver sa l 0' into:dcation "
A tQt dOH ofnalol
piloer"Ction, yawning, l oeezing, rhioorrhea, nausea, vomiting. diarrhea, abdominal cramp!!. m....,l" spMm •... which an! uncomfortable but not lire !hr..knin,_ Clonidi ne (Cata pr.f4) may be h~ lpfu l for &ome narcotic withd .. waJ lymplOme. With long
7.3.
Cocaine
The ineNn ing use of.:oc:aine in its vanou. forme (including Crick) is resuJtinc in a n se in theinciden... and reeornitioo ofib deletHio ... eft"ect.son thCNS . Effecu.on other body '~tem s (tachycardia. acute myocardial infa rdion. al"thythmias, ruptureof ueend· ing aort.a (aortic dissecuon), abruptio placenta. hyperthermia. intutinal ilOChl!mia. Iud· de n dea th ... ) are well d()Cum~nte8p. uc adn!nergie n,,"'e term inal,. It ;. avaUabl" in 2 formt: roeIIine hydrochloride (h
Ac ute pharmacologi c e ffe<:ts o r cocaine Acute pha r maoologic eff,.., .... pertinent to the .... "'OWlI~t
NOD· pba nn lco)ogic effects related to the ne rvous system I . pit .. it.ry deteneration: from chronic intra.naaal use 2. Cflebral vuculiti., leu common than with amphet.ami ..... 3. Hi zum' pot,ibly rel,ted to the Jocalanesthetk Pl'OP'!rtieI of cocai ne 4. cenbrov_ular a~ident (CVA. ttrok,),' A. intra~erebral hemorrhase: _ 1"'f"Gce..,bral Mmorrlw&e. E;1,'olOtl~1 on page
'50
B. lubarachnoid iM!morrhage'" OJ: pouibly .,a mult of HTN in thII, however. sometiroH no lHion it demoMtrated on angiOflTBphy". " Iay pouibly be due to ",rebral vaKuliti. C. Itchemic strolee'': may mult from vasoconl t n ction D. thrombotic s troke "
E. TIA" 6. .nterior Ipinalartery syodrome" 6. P.ft"_ of maternal rouille ..... on the fettli ne .... ou. lyatero include": microceph· aly. di.eorde.. of neuronal migration. neu ron.l differentiation and myel ination . ... rebra! i.. {amioo. s" ba..chnoid and intraee rebral h"mol"tll . . . and l uddeo in·
'"
7. Neurotox.iooloey
NEURm,'URCERY
fant death syndrome (S IDS) in the postnatal period TREATMENT OF TOXICITY Most cocaine toricity i8 too short-live-(\ to be treated. Aru
7.4.
Amphetamines
Toricity is similar to thotofcocsine (1ft obowl. but longer in duration (may Iallt up toseve ral hours ). Cerebral vasculitis Dlayoccur with prol onged abuse (Ut pogt 63)wl\ich may lead to Ci!rebral iruorotion (u<' poge 774 ). Eliminotion ofamphetamines requires adequote urine output. Antipsychotic drog& such 06 haloperidol (Haldol®) should not be used bei:aWle ofrisk ofseizures.
7.5. •. 2.
).
References
a..m... M E.Simon R P.C .... nb<'. DA . Ethanol
j 27.2(0).
Md tho...,..."", . y... m. ~ EqJ J M«I )2 •. "'1·)01.
10.
""
H.
Co«h .... P9:AI
,in _
i 2.
'W}. •.
l.
"r"'......
Il.
SlH. I990. }.
S.lh."" J T. Syk ... K. SCM.ide',.,." J. "QI.: /IS.. ......... nI ohleoh<>l .. ;I/Id...... , 11>< oe" ..... '",i
'4.
"'''''0'<- .. ;""""wol . ........... n' f.. 01e0/l<>1 "",.
(CIWA·At). B. J Addi
~.
s.o~. R . M ~S"'i th
.'do.,iu
fco-._ If"'.
M P.R~ . M 5 ......... I»4i.
wi ....... ".. ' A' .... JAMA 2n'
'S
""""...,dOubk·OIilOl .... roIl«I
"9·13. '994.
~"" "oC.~ ."""
F.Col'fillO E.
.,Q/, R",i
'OPII""""" of .'<0lI01w,IIJd,.~. ' ,y""""",, by boo I, 9
elokn, AmJ Mod ' 12() ~ 226-9.2001. TtalO>CII' o f o _ w~t.lrow" . M..r Le'I<.ll. 75·6. 1986. W"_WD.Y""" ..... len.nMJ •.,.I, MR1," 0<>1 .. "'U1Ii< ••·. <...,..'{'!IIII~7 . N.. oo\ou 611~):
NEUROSURGERY
'6. 17.
dtu"
MO'.,.O
A,etI_ .1 : 22' ... . ' ~ . O},<>,k. N M. CciIo/l" A R T.lIoonow D L. <1<>1.: Co<. ,"<-.»4oc«1 .,.,...,.,..moI N","~ A" ",...i .. f..... in , ... "",onl ~jllO<)' of 1nt/t<1anl'I _0'Y'''''? N'"1"OW'J<'131: }'Sol6. '99' . 5th......, K .... CohonJ ... : S" _ _ b<mot· mOl< lR"'piI''''' I»'
7. Neurotoricology
,_,,,"n,
'.r).
'"
8.1.
General
COntciOllsnUlI hao two romponenta: arOU8QI
Bnd conl.ent.lmpairrnenL of ar0Wl81 ca n vary frolJ'J
mild (drowsinet5 Or som· nole llce), to obtundation,
to nupor to coma, CO nlll is the seVfn!st impairmen! of arouul. and;B defined Il$ the inability to obey I'Ommands. speak, or open the eyn to pain. The GJosgow Coma Scale (GCS ) is shown in Tob~ 8-1 (note: the scole Is inUlnded UJ asseu level
.lrttdoonsclcu&n_.
tect>nically, tnIs io • SCI'" 01 _ e •• "O(> ma'lmpI'" ""''''PQnI~ range at I<Jt.II pants: 3 """"01) 10 ' S (nmnaI) _ _ i$$1Ihg eye oper.1<19 ., pHI, ...... perl!>l'e<»llllimukl, (ll'le \If\'"""" associelecl 1'11111 CItIIUa! ~ may e....se eye _,e) ~ ,.., mo\OI' ,eJilO'!S4l. imp,manl 10 ~ ..,;nal <»rd lIan_11On
ofconsciouHoe!18 and ;s not designed ror following neurologic deficits), Some cenleni realrd a "T" next to the toUlI&eo", far 111'tienta whose verbal axis cannot"" Ullessed becau,", ofintuha t ion', 90% ofpatienl.s with GCS s 8 and none with GCS~ 9 meet the above definition of (Omo. Thus. GCS " 8;1 a generally accepted ope rati(lna.J definition of coma , A $CIOle for use in child ren is shown in TobIe 8·2'. Table 8·2 Clllidren's ComB scale' (for 8gB <4 yfS)
,
same as adull Glasgow <:OmO sc:alot ,~c epl Joo _ , ,e$pQflM:' "nge clklIlII 1l
'!'he following: term, an iMccurat.e In the Implication ofm. location of the lesion. DE«Irticate posturing implieo a more. rO$traJ le~ion and prognosis mly bf' better.
ikcorl.icote po~lUring: CIBmliea\.b' attributed to di$inhibitiDn by removal OfCORiCO$pi· 0111 pathways above the midbrain.
'"
8. ComA
NEUROSURGERY
Overview: abnormal flCltion in UE lind cxte!\ljion in LE. Oi!t!lU: 8low nexion of wrist end finec<'" with edduction in th e UE_ Erlen,ion, intern,,1 i'Otalion, ph.ntarfluion in LE.
II,.,,,,
Dece r e b,.lI t e po8turin g: Clfl&4icelly attribu~ t.odi,inhibitioo ofveslibulospinallrACI. Imore cAudill) ~nd pUCltloe reticulAr formotion IRE) by .-emo'l;n, inhibition of medullary RF (LrD03C!ctlon lit iot.ercollitular le~el. between vestihulllJ" and rlld nuel@i). Overview: abnormlll utenaion in UE and LE. Dctsil: opisthotml!ls (head and trunk exteoded), teeth clenehed. anNI extended , ...uducted lind hyperpronstfd (InternAlly rotated). wrists nexed. fingel"l nued. Loegi ClItendI!d lind ,nternally rotated, feet.plnntarflexed lind Inverted, ton phmUlrnexOO. ETIO LOGIES O F COMA
T OXIc/METABOLIC CAUSES OF· COMA I. elHtrolyte imbalance: ""pe<:illliy hypo- or hypematremia, hypen:a.J.,...mia, renal
failure with elevated BUN St creatinine. liver r"jlu", with elevated fI/lImonill 2. endocrine: hypOglyccmifl, nOhketotlc hypefOlimolar 8tate, DKA (diabetic Iotl.OacidO$ii , AKA diah<>tiCCOIl,a), myxedema COIllII , AdditotUILIl cn$is (hypoILdrenaliamj 3 . vll!lcula.r: va~ulititi. ore, hyperten&ive encephlllopathy IHtl JICISt 64) 4 . !.oxic;! EtO H, drug oYerdOl«! (including nllra>ties, iotTOfl:eruc polyphannsq', barbi~ tuute.), ln d ioto>(iclltlon, carbon lD(NJox.ide (CO) poisoning. cydoe-porine {CfLIlUI ftn encepha\opetby that.l>owB white--mat~r d,,!.ngH 011 MRlt./Iftti.e often reve rsible with discOUWlufttioo of the drug ) 5. infectiouslinflftmmatory: meningitis, en.cephRll tis, ~~psi. , lupulte rebriti~, Ileumu rc:oidOlli6 lUI /XJgt .',6), toxle-ahock s yndrome 8 ne-opllllltie: leptomeningeal carcinomp~i.t, rupture of Mopl8lticeysl 7 . nutritional: We,." lco's erwepltalopethy. vi' lUIlio Bit deficiency 8. Inherited ro@tabo\icdliOrderl: porphyria, la~ti~ aeidosil 9. ot"!(8n failure: Uremia, hypo" emia. bepat,ic en.,...phalapathy, Reye'& s yndrome , an oxic enc~phalopalhy (e,g. poO t. re8USCillLlion &otlll;l).rdill~ arrest), Co, oaTCQlli$ 10. epileptic: st.atll.ll ('pileptklUl (including non-.(:on'lu\aiv('datWl), poSt...icl;:llat.ate'1s-pecilllly with unob6ervtd !lei~ur~)
SrAUCT1JRAL CAUSES OF COMA I. vaS<;LLlar:
2.
A. bilateral cortical Or subcortica l jnrarcUI {e.g. with canlioembolism due w S8E, mltrlLl &tell08 li1, A-fib, rnuraltbrombu • .. .l B. 0«Iu810n of venelsuVplying both ce rebral hemiaphare.g (e.g. severe hHate ..... 81 earotid sle n{)lli.) C. bilateral cU"n~phallc In llirrta: wdl des<:ribe
3. neoplutic: primary or ro~ tlL-ilt.atic 4.
LrILU'Il I: hcmorrhILgic conl~ion5. e!lemtl, hem9tom9 belo,ul herniation from maM effect: presumably br8in8wm compreuion C.8uses
5.
6. 7
NEUROSURGERY
8, Com8
Table 8-3 Ettect olla te rshill on level of con scl0ll8nesr
a'
>S,
P SEUDOCOMA
Diffe r e ntia l diagn.osis; I.
2. 3.
locked-in syndrome: ventral pontine infarction psychiot ric: oOt01OnlO, conversion ",oction neuromu&Culor weiliest: myasthellio IP"lIvia. Guilloin·Bo:rrt
8.2.
Approach to the comatose patient
The follow;ni covers non t raumatic oomo(see Hood Irauma, paie 632 for thllt topic). Initilll evalulltion: il>(:ludea ITH!O.!IUre:!I to protect brain (by providini CB r , 0.. lind iluoose), assesses upper brllinatem (Cr . N. VII I), and rapidly identifies8urgica.l emergencies. Keep "peeudoooma" os II pol-lJible etiology in b8ck ofmilld. ApPROACH TO COMATOSE" PATIE"I>IT, OUTliNE" 1. cardiova~ulllr stabilizlltio n; establish airway, ched< cil"(:ullltion (heartbea t, BP.
cllrotid pulse), CPR ifnece56sry 2. obtain blood for tests A. STAT: eJectrolyte& (espe<:ially Nil, glu<:OSe, BUN). e BC + diff. ABC B. others as IIppropriate: toxicology screen (serum & urine), calcium, ammOnia, IIntiepileptic drug (AEO ) levels (lfpatient is ta king ABOs) 3. IIdminister emergency supportive mediclltians A. gluoose: lit lefUlt 2::' rol ofOll> 1VP. Due to potentillily h arm fuJ e ffect ofglu· OO/Je in global ischemis, ifpoallible check fingerstickgluoo$t! first , ot herwise ilucose i8given without exceplion. Wllesa it is known with cert8inty thllt Be· rum glucose is normal B. 118loxone INarcan®): in case ofnarootie overdose. I limp (0.4 IIIg) IVP C. flumatenil (Romll!ieon®): i.., case ofbenzodiuepine overdose (ue page 36). St.Ilrt with 0.2 mg rv over30 secondR. wait 30~, then give 0.3 mgover 30 sees lit I millute intervals up to a IIIg or until patient llrouge9 D. thiamine: 6()..lOO rng IVP (3'10 ofWemicke'. pre!!
'"
long tract sign$ , i immedillte-
B.
.,. move
".
6. Coma
NEUROSURGERY
Ol"gl.t. (LP in thilletting m~)' Iw risky. lee u,mbar puncll<~, p.gto 61 $). seilurel if pn!lenL If sLu l u8epih!pti~", i'."6'-~, trut ... in. d,tlIted on pa¥e 26510b18111 &merg
t.n1I~III!ll~TB!I,eC
CORE NEUno EX~M (FOR COWV A. rel (!i r ll.tory rote hod pa lttm: the moat rommon disorder in impaired ronac:IoUllnltlll 1. C h eyn ...S t oke.: breatbin, gradually CN!IiCendo.ln amplitude a.r>d thlll! ~~ trail~ off. follow.d by lin ,.pirnt.oty L p9U61!.and then the pIIl\.trn repeat.a. ," H~rpne~c phue iJ, ",uIUy. lon~r Che ne-Slokes r,S!li'-'Ory panltln thftn .pnetc. Uliu.lIy Ief!1l with dl~V oopba!lc lesion. Or ...n.teral ~bral henu'pbere d),.ful>elion ('!IIf1 •• ~U;cJ. e.,.. ea.l)' Increased ICP or mt~boho .bnormahly. Rault.l fnlm anllKtea"d vMulltory ' ''I!pon,e toCO:! 2. bypene ottlatlou, usually in reapo"" 1.0 hypou:mia, m~botic !Icidof;i., .,pi... Lion. or pulmonuyede",a. True ef1ntral neur~nic hypo""Mtilal>orr .. raN, and ",u.lly results fromdywfundion withm the poria . [fnoothe. bnunst.m algmare present, mRyaUUe6t pli)'Chialric di5Ol"l!tr 3. du.ter bre.thi ng: penodlOf rapid ilT1!gula. breathio" &epa. ral.e'd by apneic lpellt. WIly IV· pur .imillr to Cheyne-Stoket, UI'\J' merge with pat· tern. of gupin, roespiratloll5. High medulla cor lower pont~ ,iOl}. Often an 0"';nou5 eign .. . a plleuat;o (rare): e pause It full iD!lpiratlon, Indie.\86 poll' tllle lesion, ~" with b .... il •• ar_ tery O«Iu,ion
-I'''''
v.nou.
,
a t .. le (Biot'. bN!athini~ no pattem in rate ""depth of ..... pi_ raliOI\l. 58" With medulla.,. le.ion. UlUlUy pNltarminal
Alax"oc .espifaliol"$
*
B.
""1.11 (,ioe in mIn) in ambient light. and U! roeacLion to ditftlltnnsens.uallight
"'lua) and. reactive pupillindiuole to~i~n-...llIbol;c CII~ WlU! few el
,1_
NEUROSURGERY
8. Com8
'57
diate post,.anoxi~ Or hypothe rmia (core temperature < SO' F (32.2' C)) midposition (4-6 mm) and fixed: more ex.tens;ve midb".ln lesion , prosumably due to interruption ofsympatheties and pare.sympathetics C. extraocular muscle functi on L deviations of ocular a>:es at re!lt A. bilateral conjugate deviation: 1. fron tal lobe lesion (frontal .::.enter fQrcontralateral gaze): looks toward aide of dea: n.octive lesion (away from hemiparesis). Looks away from side of /leiZllre focus (looks at jerk.ing side), may be statu s epilept icu &. Renex eye movements (ru below ) are norm al 2. p..",tioe leaioo: eyes look ~ from lesion and tooYards he miparesi s; caloriC!! impai red on side of lesion 3. "wrong way gaze": medial thaJamiehemorrhage. Eyes look aw .... y from lesion and towards hemiparosi . (an exceptioo to the axiom that the eyes look ~a destructive $upratentoriallesioo )' 4 . downward deviation: may be associated with unreactive PUpil8 (Parina ud's 8yndrome , oei1 pag~ 86). Etiologies: thalamic Qr mid brain pretecta l lesion s, metabolic coma (esp~iaJly ber bitW"ates), may follow a llelZUro B. unilateral outwa rd deviation On side of Iaeger pupil ([II palsy): uncal hemi· ation C. un ilateral inwa rd deviation: VI (abducens) nerve C.
O.
!;k~1l!i.2n
1. III Or IV nerve/nucleus lesion infratentorial lesion (frequenUy dorul midbrain) 2. spontaneous eye movements A. 'windshield wiper eYell": random roving conjugate eye movements . Non·l .... calizing. Indicates an intact 1lI nucleu s and medial longitudinal fasciculua B. periodic alternating gaze, AKA · pin&"·pong gaze": eyes deviste side to aide with frequencyd - 3·5 pe r seoond (pa using 2-3 &eC$ in uc h direction). Usu· ally indicates bilateral cerebral dysfunction C. QCulp r bobbing: repetitive rapid vertical deviation downward wiU, slow re~urn to neutral position. Pontine lesion (Ht paIJ' 588) 3. intern uclea r ophthalmoplegia UNO) : due to Ie. ion in medial longitudinal fasciculus (MLF) (fibers Cr0531ng to contraiaterallil nuc\eua are interrupted ). Eye ipsilateral to MLF lesion does not adduct on spontaneous eye movemen~ or in response to reflex maoeuvers (e.g. calories) ( sa P<JCI' 585) 4 . rolle .. e)"l mo\fflments (maneuvers to test brainstem) A. c>cw Qvest ibuln r enel<". AKA Ice water ca lori ea: fi rst rule-out TM perforation , then with HOll at SO', irrig8teoneear with 60-100 ml of ice water. NB: ffl9po
(..
A. ""ulov"'. 'ibulor ...,n~" Joliet): tho ...,'icipourl reo""" ... i. commonly "'i . und ~ ....toOd. In • norm.! oW "",ie nt Ib..,., i•• Iow de';. ti.n towordl lhe . id. oflh.
».
'"
8. Coma
NEUROSURGERY
wilh 3rd nerve palay (herniation). UaullJIy OInjntllmed in !.OXic/meta· boUe«>tna 4.
1IJI$l.IIrmul without IOnic deviation (i ....Y" f8ma ;n In primllry lion) virtually di/lll(n08t ic of l'IIychopnlc wIDe c:ontrelatualaye r.u. t.o adduct: [NO (MLF lealonl
6.
~i.
B. optoki ne tic ny ltermu, ~oc. atronal, IU!;!;"ta paychOgo nic eon,' D. UlOI.Or: mu aele tone a nd l'1!f1exea, t8lljlOilBe W pAW. Bobinski (no'" a.ymmetries) I. ap propriate: impJi" cortiw.pi nlll trleta lind eort.x ",tact 2.. 8.ymmuric; lupl'lItentoria l lylon (t.one Ulually IRcrenO'd). unlike ly In meUiboJic S. incon, i.tentJv.ri.blt, HilU ~, POIytroBtnc 4. eymmatric: metaholic (""1I811J' decteJIl!f:d). Al tarixil , l!"tmor, n'yoclonu, rnft, tw present in metabolic: coma
..
5.
I!yporene;<.ia: ooNide r myud.'lu, «Ima, nPHililly In patient ane '1Taa.uphenoidal 'utte ry
6,
pattem. A
p r~ ti [\1
wHIl$
_ decort.iute po&tu.rinr; 8nM nex, lell ..,u.end: large co rtieal or ",beu rtlcaJ I&-
B. dfetrfl>l'1IU1 """tllnn,: . n . . .nd l.pcst.tnd: brainatem iDjury.t Or Mclow lower ruidbra
8.3.
Herniation syndromes
Cla.ic If'Ichinr:: hu been that shiJ\l; in bnlin mue l",r::. QI\I500 by m8He1 or inintmer.nilol prasulll) throuCh rir::id openi",. in thll . kv ll (hemiatiaa) c:omp..... other Itzvcture. oIthe CNS prodlldog theobMrved 8Ylnptoml. Th.. view hal; been challf-nCed'. .. ith the h)'pOthuis lhat hf-mlelion may be an epi phenonllnon that !XCu" lau in the pr_and i. notaetu.lIy thetaUSlloftheomllrvau.",.. Hp~er. Mmia tion moder- al ill H .... e III ~ul.ppro~imation •• ThUf- are many popible hen>i.tion ~dromH. the » vI JI)OIlc..mmon ....: J tentral ltranlt.tn:.orilll) bemlation (_ Pl'B" 160) } .... p ... teotori.l 2. uneal herniation 1_ pG6I161l herniation 3. cinguJatl hf-miation: ci llCUl atll eyru. hernlal.eoll und~ rab (AKA l ubralcine .... mia t.ionJ. U..... lly ....ymptomalicunle.. ACA k.inb and ooduducaul lng bi· ofimpenliina: lTan"III1LOriallworniation frolltRl infardion. UsuaUy 4. upward Of-",beJlar (we bdtN#) } inr.. telltori.1 hf-mintion 6. ton. mar lu.miotion 11ft. t>tlow ) ~
".roII
COMA FROM 5 UPRATEHTOfilAL MASS"
CantrllJ and unul hemlll t'or. eoeh ca U_ " differtil t fOND ofroatraJ-eauda l ae' . ";orlltiol1. Cfll tra l htm,at>on , H uh. In Nqlle"ti.1 ra;tu", 01' dian«phalon . midbra in. pon •• medulla (arf fHl6' 160). For ul\C8l herniation , 11ft' pap 161 . ' CllIu ic" l ien' ofin· o;rellSed ICP (HTN, bradyurdia , .1te~ ,,"piralor), patUom ) lUIUlllly MIn with p-fOM. Inionl may blabHn~ 111 alowly d~'optl\( au pflteatoria.! mlti",:tion betWl!tn cl!tl lTal and until hl!rnill ion i. ditTi~ull .. hen dysl\.lncti!>n ruche. Ihf- midhrai n 1,..1 gr below. Predielin. the roeatlGn a rthale4k)1I bAM
m._.
hemiat.lon ayndrome ill unreha ble.
Clinical c horacteriat iu d ifferentiati"'i u ",c.a l from ctmtrlLl h e rniation dt\:reaH
•. c...
'"
Differential di agnosis of s up-catentori a l etiologies L 2. 3. 4.
va8Culu: CVA, intracerebral hemorrhage, SAH innammatory: cerebral abs<:ess,lubdural empyema, herpes simplex encephalitis neoplastic: primary or metastatic traumatic: epidural or subdural hematoma. depressed skull fracture COMA FROM lNFRATENlORIAL MASS
NB: it is essential to identify patients with primary posterior fos~ IKions (lee To · bit 8·4, page 156) u they may require emergent surgical intervention (Iff poge 772 ).
Etiologies of infratentorial m ase L
vascular: brainstem infarction (including basilar artery ocdusion), cerebellar in. farction or hematoma 2. inflammatory: cerebellar absceS$, central pontine myelinolysis, brainstem encephalitis 3. neopl"sm s: primary or metaetatie 4. traumatic: epidural or subdural bematoma HYDROCEPffALUS
lnfratentorisl masses can produce oblltructive hydrocephalU$ by campreaSing the Sylvian aqueduct andlor 4th ventricle (Iff page 404). UPWARO CEREBEt1.AR HERNIATION
Occasionally lOOn with p-fosla masses, may be exacerbated by ventricul ostomy. Cerebellar vennis ascends above tentorium, c:ompreuing the midbrain, and p
Cerebellar tonail s 'cone" through foramen magnum. compressing medulla - respi· ratory arrest. Usually rapidly fatal. Otturs with eitber Supra- or infra·tentorial masses or with elevated 'CPo May be precipitated by LP . in many cases, there may simply be pre$9ure on the brainstem with· out actual herniation', There are also cases witb significant cerebellar herniation through the foramen magnum with the patient remaining alert'.
8.3.1.
Central herniation
AKA tranatentorial herniaticm AKA tentorial herniation. Usually more chronic than uncal herniation. e.g. due to tu~or. especially offrontal. parietal or occipi(allobe •. The diencephalon is gradually forced through the tentorial incisura. The pituitary stalk may be sheared, ",,"ulting in diabdes insipidus. PCAs may be trapped along the open edge of the incisura. and may occlude producing cortical hjjndness (see BliJ\dnu, from hydrocep/w.11l8, page 202). The brainstem suffers ;"chemia from compre!!5ion and shearing of perforating arteries from basilar srtery - hemorrhages within the brainstem (I)"...,t h .. m" ....h .. g .."') CT or plain x-ray criteria
Downward disph.cen'ent of the pineal gland may be demonstrated '", Perimesen. cephalic ei&terns are compressed. DIENCEPHALIC STAGE
Early_May be due to diffuse bilateral hemisphere dysfunction (e.g. from decreased blood flow from incN!""ed ICP) or (more likely) from bilateral diencephalic d)'$funtion due to downward displacement. This stage warns of impending (irreve",ible) midbrain damage but ill frequently rever$ible if the cau"" is treated .
".
8. Coma
NEUROSURGERY
COi'iio.igalf'o:iiligll1fiOMiiOml tIM"'il 'Yen w:,ugaie t!iiiT&aiiStem 11'.l:id. OSU/IRVIiCiSib\ie
i
OO!..L'S EYES and conjIJgaltl ipSiIalMaj response LO OJI~ w~tGfealctb (CWC~ ImpaJred III)galt! _ to corrcresOOn 01 SlJPllrior coIIiaJ~' lina di8!\Oellha1ic pt(Ite<:Wm (Pariodli Ii)'l'l~romeSllflpa~86)
MoIor
a-"f"pjjlopOiajilii~~liSelO r.o~6itiIe"""'iirBitiiiiil<j. iiiljiiitiil!ili (paralOnic .. ' iiS!anu). II pltVicmly hemlpar~K:conl!aralelallO lesion: ma'J I'oOfSoefl. h~ mo!lonlessness &.JI!.a5P ,~, Ihen OECOATICA!E (rilia/Iy ~JiIltira.!.!<' ~ n~_caS:!'.5~
l
MIDBRAIN - UPPER PONS STAGE Wh&n midbrnin s igns fully d ..... eloped (in adulu), pl"Ojj1\os is is very poor (extnme is cnemia of midbrain). Fewer than 5% ofcase:s will have a rood tf!COvery ifl"'H tm ent Ie
successfully IIndertakl'II a t this st8g~ .
Aespiii1loiip~ ~
""""""'in panlinll lllmo"noge poIlPQltlI PllPis lQIIItat IIKaus- I'" 1M$0I sy""",l~ C5 ""'_IIIePllr~l In n~mill\ron. 1M f'II!I,.yrn~lh.tIcs ... USUAlly 10$1 , too (3nl "'''''''1,*,'11)
Ic!I Uf'OllllllS8
_
LOWER PONS - UPPER MEDULUoRY STAGE
ISIOW, Ifii'9Ular r~lti arid deillh: Siijhs/gasps.
!Oocasionilly /lype1)lOU I llelOlt!og OoTaleMdityWilh~'" -
============
wl\tl apnea
OUTCOME AFTER CENTRAL HERNIATION
In a series of 153 patl"nbwith sigNlofe(!nlrai herniation (altered l..... el ofellnlicloU$IInss,ani!!OCoria orf",ed pupHs.aboQrf!Ullmol.or fiodings) 9% had good recovery. 18~ had fun~ional out.come, 10% weI'\!! wverely diubled, Bod 6O'l> died" Facl.ors fls&ociated .... ith II better result wera young age (eapedally age,. 17 YJ"IIJ. anisoeoria with deteriorating Glasgow Coma Score and n<.lnflaccid motor function. Fa",. lOra .... soeiat<>d with poor oukorneo weI'
8.3.2.
Uncal herniation
Usually oc<;u,.,. in Olpidly expanding traumati. /oemat.omal. rr"'lue utly in thalateral middle'{08'8lI Or tfimporal lobe pUHhing medioJ unn.S and hippocampal gyrus Over edge of tentorium . 0D trapping tbird nerve and direetJ,y eornpre~Bing mi~brllin . PCA DUly be !>c. duded In with central herniation ). For CT criteria se:B. below. Impaired consciousoes5 ill NOT a reliable ea rly sign. Ea rHut I:OnSi9tolnt sign: llOi· lat.erallydilatinr pupil. How~ver. it is W1likel, Illsta patient uodergoinr e8rly unca l he ... niatioD would be completely neurologie.ally intaet ex~t for anisoc<.>ril (do not dil;mias confusion. agitation. etc..). Onoo b",ir.u;w,o findinga app"ar, deterioration may be rapid (deep coma may OCCUr wlth;U hounot
CT criteria" Tentorial incisura surround~ interpeduncular and prC-pOntine cisterns and b!"8 IO' stem. There is gteatinterpersonal variability in the amount ofgpace in the incisura . Impending- uncal or hippocampal bemintion may be indj~ated by en<,:r08~bm~n t on
NEUROSURGERY
8. Coma
'"
lpterala sped ofsupras.allar cIstern - nMterung or normal peuLIJgoIIP I ~ hape. Once herniftt.ion OCCUMI CT Dlay "ho ..... , brail'lStem di'& pl at~ment and natten;n" coroprellSion of tont ralateral eerehral peduncle, midbrain rotatilm with sligh t i~ase of ipsilateral s ubarachnoid spate. Alii<), ¢olltralateral hydl'Ol'ephalus may occur". Oblitern tiol). of par8S1!ll.r a nd interpeduncula r cisre" •• ~rB as uncus and/or hippocompll. are ror~ed th ....ugh hiatus. BrainBtem c,ompres!lion - AI' elongation. Since du ral s tl'\.lcture, enhance ..... ith IV contrllBt. thl~ msy be"SEd to help del ineate tentorial IJ'IsrgiD,S .... bell neC@$sary.
EARL Y THIRD NERVE ST.AGE (tj:QI A eRAlHSfEI¥I FINDING, DUE TO 3AO NERVE:: COW'RESSION)
LATE THIRD NERVE STAGE Midbrllin dysfunctioo llCCW"S almost imlJ'ledjal..Jy aJt.er symptoms extend beyond th06edue to foc.l cerebral lesion (i.e. mayskipdiencephalicslage. d~ to lateral pressutE on midbrnin), Troatment deJ a}'s may result ill i~versibl e damage.
From Ihi~ point ,,"wanl . the unM I synd ran... is indis lingurnru.ble mill untrnl herniation (u
8.4.
Hypoxic coma
MOllie eneephRlOptlthy rna)' b. pO.) or a.nemic 1I"."tia (following e..'(du. 10 .... 0 • ....,;" auo.i .. (drop MyoIn IIDnguination or canl iac IIrreat). donU! i. commlmly seen. Pat hology: Jesiona pre-dominate in 3rd oortica l loyer (grey ma~r): Ammon'. 110m ~ 1.1110 vulnerable_Whi ta me lter;1 u~uaJ1)' better preserved (due to lower 0 1 requirement) . In tht bani g.nglia (8G ); enollernie anoXil eave",]), affects globus palHdu.; " ""mie ano..u a aITec;ts th e caudate nucleu s and pUI8Ulen. In the eerebeUum. Purkinje «1I~, daniel
'"
8. Coma
: '~'~'~.~'~"~~;~~~~~~'~";';'~'~'~'~'='~~~
NEUROSURGERY
Mu Ltiyariate analy"s yieLds (XIt· come prognosticator'll shown in Tobie 8·:; &06 Table 8·6. NB: this lI.Oaly. l, ~pp1ies on.1:t to hypo,ao;..ischemie coma'· More r~nl6tudinwnfinn the poor prognosis of ..mrellct;ye pupils and !ack-ormotor ..,sponse loG pain"; if /lither orthese findi np 8M $/len within a rew houllI lI.l'ler cs,rdillO!aTTffl tbfte ;11. 8n 8O'lI ris k ofduth Or permanent vegetative I Ulte, 8nd if present at:1 day. these th'. r8le t(l9f 1(1100%. Clurocortiooids lau-roids) hllY~ ~n ahown tohll.Y81\O bt:neficill ~trKt on ~ urviy.! TaU- or neurolog'iC
Ta ble 8-6
al>b""vlat;o,u,: \Oo?iL .. ... 'lhin no""oJ Il",H••• GCS
• GlUt_ Com • .su..101''GCS-
lIN IIIOICT ""..... .J: BOM • ".-craocula< mlUC.!.:
8.5.
References
t..-loItC.J .. _
R. ... " ...""' .. of .........
PAI' ... _ _, ......... JITXU
,,n.....
~, ":!
11"' , 19").
>. V',_ A B. H"'Y" R Il!C""'V.o;)" ............... .,,'''',,, I/w 1onJd.J";""" p<1IWoI I. 1'I'~roI"'''' ..... ro . . y'l'R K. Wi"",,,,,, J E.on
,. • ,
, ,
II",," V l.aw..,C.YonIo
"<"""""...,...." •%,
a..."...-uw.2uOS V...l6oolAlltJI ..,in .., .... ",..,po, ...... ,.,.d ,..." .. 46' )6 1·), 1W9 fi",...CM """"1ni.I>&ni,....., .... ........... _. .. pt . s.... N• • rnk>tl' " 0\ 1·11. 198-1 . _f._.JII.Th
." •• •
AIII<.C M.PI<w EH . " - .... "81~ ......
...."'.,_.'.J
NBul/OSURGERY
'"
" " " "
"
., n
J9U. H.... r. c..... yI cr"""..r.. _ _ ndl •• ,,,,,,.. _1,,,,.1Il>0
"'odrc"'" B T. PI&> L H' FIoO<1iato>,
"",,",oM! ~"'ion. J'I_fIH}' 19l :!ll.,JI.I'Il1 0
_by
<,..j/llC1".Ilod~ IJJ, ~J-6.
I~n
5IOYn., I, o ..<-,i", .......,/11 ..... ,.,...,: FUId" "OII«><" pYldiu<1l...,."".rb, !'Itu'.... dlolor;r
14, 101·f. I!fIT. Me"fo_' W, nf!O< J C. B_ W H. "..s; E.< ·
.....", ...
Otoot", ....... OIl
!~,~ _
....""'" 167·n.I'lfI/). D f ,c..o... 1 I.S' ..... 6 K.m./J l'I«iico
L<.,
1~1O-6.19t)
~EG I, .II ...
RI. S,"".. _C,.."
.1. ' S,.,.""'o<,....k"ofeotlJ'pttd~GI
__
""I<""' " ,•.,.,.io>--«
,..". ...... M. ' '',/Io·T,..!! K._ v ..""", p." ..s. O_ialldIlUlmmlClod _ ............ I""inl _ . " iot ...... ,", <.. d,,,,, __ J AMA 2~ ' JoI!1·JO. 1919
8. Coma
>OJ
·
~
-
"
-
~.-
'.
...
I
9.1.
Brain death in adults
MOS1Uates l
a""'n
BRAiN DEATH CRITERIA
lWcommendatlOD sL. ' : 1. !lbsenc&0I DrI_irISIem 'elIeUS A. absen'-"!of brai"8Iem refl ca·e.: A. liud lll4"~ t. lXular eumination: 6 BbsenI comeal relle.(eS A. rlX~d pupila: no ....~Pllnse. to bright light (tau: ion alter reaus, C. absfnI OC\IIOYestIWar ~tle~ citation: H e bflow). usulIlly midD. absenI ~hallc po!Iitiou (4·6 mID) bll ~ may VB"" E. BCseoI gag &. w.; gil IeIlel: 1.0 dilated rlngeB (9 mm) In size 1. B. abJ;ent corneal Taflel"'I!lC 3. no r~ to d\'ep~ pain C. abHnt lXuloc:..phalie (doll's 4, 'oi\I1~ eyes) reflu (coll lrain dicated If C·spine not elesr edj, _ {XlB" A. tori! temp > 32.~' C (90' F) /58 B. SBP '" 90 rrrn t'Ig D. ,b....moc ulovQ tibuJllrren .. '" iccld wewr ca Jo rl ell): it1lltill6(l.. 100 ml iea water into one eaddo not do ifTM perforal.ed ) wiLh HOB at 30' . Brsin death is e~duded ifan), sya moveroent (... ~ IJ06('. r58l. Wailalleut 1 minute for responBII, and::t a min before testing the op~it' side 2. absent oropharynC .. a! ....1'1,:< (gag) to stimulation of pDJIt.erior pharynx S. no rough TeSpOnsa 1.0 bronchial auctloning B. a pnea leat AKA apnea c:w.Uenge: nospontan~ou~ respirations D lifter diSCOM&L:!tion &om ventHaloT (aases_ function or ln.duJla). Sioce elevating f'aC01lncrease.ll tCP whl~h oould prec:lpi tate h
,.!le.o
....
'
.I.. no<e: EEG .. J!PI "'.1\11. 10'1..... ...... ,~_ntkd """""', ..... ~ .. . pop 16:1 B. Cl M ... I-rmp. ,heli. p. thw.y. ... r ' ..... in "' ... " C. cornul ... ~.,~. d
IhIon r. . ny '1LLoe$"Gn • • ' p'n>moler "'.Y ~ conn"""" 10 II .. potkn,'
'"
9. Brain deaLh
.<Wq""'" tid.1 vatu",..; If NEUROSURGeRY
2.
3.
4,
6,
6. C. no
high". £'aC0 2; lIot valid with aevere COPD or CH F) t.o prevent hypoulIlia durin/: the ~t'w:il.b. tho>.da.ngerQf cardiac- 8l'ThythmI~ or myncardilll ,,,fa rtl;on), p~e th! te!! with 16 minutes f>fwm tilation w ~th 100$ 0, prinr!.Q th .. le!!t, a.;ljual the ventilator W bnng!.hl! PaCO, 1< ~ O mm H~ (to ~hOrWn lh~ teat time and t l!1.L8 reduo;:e th~ n.k of":lypoKemia) during the len. hay., p!l8IIive O2 fi()w IIdmini5!.ered at 6 Umin through either II pediPirie-oxygen cannu\anrll No. H Frencll tracheal suctioncathcter (witb tlleslde portCO\'end with altha.lve tape)p:lped r.o the estimated 1@~1 of the carillll etllrtinl from nOmlOCRpnea , Ule aVeJ'lIge tim" to re8ch PaCO, '" 60 mm Hg is 6 miouteM (cl9S81~ tuelling is IhIIt raC~ rises 3 mm Hglmin, but ill act.uality lJtia nne!! widely, with an average3. 7 2: 2.:1'; or 5.1 mm flr/min ifst.8rting III normocarbia'j, Sometimes allong os 12 minutes 1Il1I)' bee necessary the t.<'1;t aboJrted !»"emlltu",,'y if, the pa.t;en: blllat hu: iOCODlpatiblf! with brain deflW significant hypoterll!ian {)<:Curs If0 2 uturation drOPfi below 80% (on pul!MI a>dmeter) significant cardi"e arrbytlunias IICCUT U' patient doea not breathe, lend ABC at regular intervals and "t tM complet.ion often rl'gardl~u ofre!l.8On far ten;ainatian.lfthe pa tle.:lt doe3 nat breatM for at least 2 minutes!l.lhr II P/ICO. > 60 mlf) Hg is doc:ulI>en:.ed, theo the tnt i8 valid and ill compatible with brain death (irthe patiMt is steble and ABCs resull.!o Ir~ Ilvailll.blewithina few minute8, I.h~... pnea challenge may be IIOI'Itinued whiJewaitin\! for Tl'suJt8;n aase the PaCO. 'i C 60) i fPaCO. atllbilii:es be low 60 wm Hg and tb@pOl rem"in.adequate,tryreducing the plIIS!!ive ~ flow rDte .ligbtly motor function
u.
""'~::?;~~~~':'i~J;:~~~:~post uring Of seilure8 afE, intODljl8tiblfl with !hOldi.
2. I . :'
3.
o.
I.
•
Ii .1
3.
<.
; ;
5.
6. E. I.
; II
condition i,
a single valid brain 2.
NEUROSURGERY
9. SrIOio death
'"
3.
ifan in-eversibJe condi:ion ;1 well establiJibl'li Md no clinical confll"lllat.ory tntl ...... ..ed: 12 hou", 4. ifdiagnoosU i. ulll'Utain and nO clinic.1 confirmatory ~I.t: 12·24 bour. 5. if anoDe il\lul)' ill Uw! mu~oCb~ain deaLh: 24 hou", (may be . homned if ce.N· tion of CBF ic demonllnted)
CLINICAL COtfARMATORY TESTS
CEREBIMI.. ANGIOGfY..PH'f
Crit.lria: at.ence orinlJ"atrallial now al the level o(the carotid bifurution or eircl. of Willi,'). f"ill.i.al o£lbe supe rior "giual . inu. 0fX\U" in a delayed (a,hlon. lnt.lroblerv
IQ..,
EEG
ell! ~ do,", III bedlitl... R.eoqui... . .,;.nced int.lrpretar Don not del.a(:t brain. • t.lm artivity. and elec\rocerebr.l.il.nce (ECS) d ... not oelude the pouiblUly 01 ..... venibl. coma. ThuI, I I leul a:..bmu:a ~rvillon if, n!()Ommendad in cortiUDc;tlon with ECS, U.iDg RCS . . . cliniu] Ql)nUru'lltory t.I, t . lIould be doni only in pui. nu without drug intoxication, hypoth~ru'li. , nr . hoek. Definition of . leetl'O«lnbral all.nee 011 EEG: no elec:triwltCfiYl t)",. 211V with th e following rtquirelDl!nt.l! reeord inll'r
t. IIn"U peAk , in early iystole withDUt di'itoli, flow 2.
fir reterbe.ating now (indica. tiye of tlfnificanllj Inereased ICP) i"iultl "bllence ofdnppler ,ignal. cannot be UIIM U cri!n"1a for brnin d~ath since 10'110 of palIon II do no~hl'" temporal lS()nPtion window!
CEREBRAL FVoD/ONUCLIDE .foNGIOGRAM (CRAG)
Can b, perfo.medllt the bed"lde with a general PllJ"PO'M! Kio tillatlon camera wIth a low enerl,)' collimator. Ma.\' not det«l minima! blood flow 1.0 thl brain. esped,l]y b.min. l!.em, the ... fo ... i.hwu:J. obsen·lltion in cortiunction with CRAG i. ~ed un.leu there fa • clear etiology of o"erwhehnilll br.in irtiury (11.1. lnIlSlii .. e. helllorrh'g' o.r CSW), May be ll.O~ruJ to conlinn dini;a) brnin death in the foHowinl"u.inp; I . where complicating conditions are pNlSent, e.g. hypotherm ia. h)'pOleJaion (e hoci<). drug intoxication 2 . ..,veN:' racial (numB where ~u lar findlnlllQ.lY bt difficult 01 con~lnl 3. in "",tienll with "'vere COPD or eHF when apnea teating mill"! not ~vRlid " 1.0 s honAtl th~ observation Jl'l'.riod. " pt'Ciolly wheu organ don' lion i," poIIsibUi t)'
Technique 1 acintiUation came,. fa poIilioued /iI. lin AP heAd and nKk view 2. i~ 2Q..30 mGi of99",Tc·labeled _rum albumin or perte(:blletlt.e In a .. olum. of 0.6--1.5 mllnto a proxlmlll lV pOrt,ora ~ntr.J Une, f
'"
9 Brlin death
NeUROSURGERY
Findings No uptake ill brain pllni!oohyms 1I
n
SSEPs BilAteral abseocaorN20,P22 re· sponse with median "'Il'\'a stimulation, ATROPINE
In bra in death, an amp ofatrop,n8 ( \ Oli) sbould not sITed. the hellr! rllte due w the absence of "a gal t,;mel it normally in· crease the heert rate). Although atropine in usual dOM!! doolll>Ot ""use pupilllll")' dililtation'''u, iti, prudent to '!)Carnine the pupils first toeliminatl! uncertainty,
9.2.
Figure 9-1 "Hollow-.kull" algn on CRAG ISLOIIc: AP _ W<enl~ .....".. .~.er IrIjecdon)
Brain death in children
Criteria for death : ;:'ravarsibla loss ofcardiopnlmonary or enUre b""in fUnction 18f1 in adult), but the (clinically unproven) auumption that (I child'S" brain it mor
absence of buLbar mU8Cui.aUlte movement: including oropharyngeal lind facial rnu~cll'll; absence of corneal, gag, \lOugh , $uck, lind r!lOting reflex 4. llhoien"" of ro~pi ... tor:v movem0at (usually tl!;lted alter other erilens met) 5. flacci d tone aod ahoieoceof5pont.aneoua or induced movemenUi (spinal myoclonus and spinal \lOrd I.llOvemenu, e.g. renex withdrawlare not included) 6. examination r""ulb should remll;n clllU"leZl~with brain death t.hrllughout observation periOd observation period~ according to age, A. in nawborns born at or art.er term (:> 38 wks): 7 days B , age 7 days · 2 m(llS: 2 l!Jl3mination.s and 2 EEGtl48 hn apll.r! {repeat ualn unnOOl!5Sary ifcerehrnl rlldionuclide IIngiDgl"Bm (C RAG) (ai .. to y;suali ZOl cerebralllI'tl!riesJ C. ai" 2·12 nlll~: 2 ua..rninatiofUI and 2 EEGs 24 hn apart lr"peatexam unlJ<'<. essary if CRAG negatiw) O. ai"" 12 mO$: if irre"ersiblt \lOndit;",n ~~iBt~, labora\.Ory tesling is nnt nee· 3,
NEUROSURGERY
9. Brain death
'"
usary, and 12 ~n observat iOfl is sufficient (undesr conditions, especially hypoxic· i&chemic encephalopathy, are difficult to assess, and 24 hra obser· vat ion is suggested unless electroce.-.ebral s ilence Ofl EOO or a negative CRAG confirm dillgllosis) confinnatory test3: A. EEG: standard .-.equin:'ment for 10cn, electrode distance<SU!X'llt 16n may be decreased in proportion to site of bead B. CRAG: applicability to patient" 2 mos age unproven
9.3.
Organ and tissue donation
State and federal laws require families of individuals satisfying criteria for brain deatb to be approacbed a bout the po$sibility of organ donation. ~'acts that may be con· veyed to family in order to help their understanding about orglln procurement: 1. any Or allauitable oTgaJl.S may be individually specified for donation or t.o be ududed from consideration for donation 2. organ procurement may be done in such a way as not to interfere with an open casket funeral (Le. disfigurement can be avoided) 3. families tan receive infonnation lUi t.o tha ultimate use of any recovered orgaJlll
9.3.1.
Criteria for qualification for organ donation
Ge n era l el:(l lusio n ary criteria for organ donation (modified") l. infection A. un~ated septicemia B. the following infectiona or oonditiOfla: AIDS. viral hepatitis. viral encepha· litis. Guillain ·Bam ayndrome C. current rv drug abuse D. activeTB 2. malignancy: brain tumors represent JlOS8ible exceptioJl.S ('ff ~kIw) 3. relativeexelusions: chronlcuntreated HTN, hypotension (desired SBP> 100 with normal CVP) 4 . dise_ of the organs coosidered for dOJ)ation 5. anencephalic newbom~ : recent consensus is that the functioning brainstem in these inf8J1tJ; (e.g. spontaneous respirations) di9qualilie~ them froro the diagnosis orbrain death (furthennora, few such organs would likely oonefit others)" G uidelines for inclus ion (some recommendations from refe rence" included) These guidelines are constantly being revised . in part due to '".proved results with the use of cyclosporin in recipients. In general. consultation with. a transplant coordina· wr ia recommended to determine appropriateness of donation . 1. brain death in a prev ,ously healthy individu~1 2. organs: A. kidneys, age> 6 roO! (because oftize). Normal blood pressure, BllN.serum creatinine & UlA. No S I..E (because ofposllible lupus nephritis) 8. heart and beartlluolr. ageideaily" 40 years for males and "45 for females (above these ages. a cardiac catb i8 usually performed) but up to 60 yrs may be used depending on condition of heart and potential recipienta). Euro by cardiologist ind ,eating 00 heart disease (cardiomyopathy, valve defect. reo duced ejection ('-""tion. severe ASHD. SIP CASG). No roDM C. liver: age > I ".os. Nonnal hepatic fuTlCtion (nonnal Or acceptable AST, ALT.I..DH, bilirubin (direct , indirect & total) 8J1d nonnal dotting studiea) wit~ no history of liver disease D. pan cre as: age 15 - 40 yrs . No history of disbetes. Nonnal serum glucose and oroylase 3. tissues : A . J:lI.m9!: age" 1 yr. Neither cancer oor sepsis di5Qu"lifiea (rabiea and CreutzfeJdWakob disease are contraindications) B. Din: "ge 15-65 yrs. E~c1uded if cancer C. il®l::; age 15·65 yrs . E~duded if cllncer D. bone marr!lw: age ~ 50 yrs
'"
9. Braindeath
NEUROSURGERY
E. hgart valves: age" 55 YI"II
9.3.2.
Organ donation in patients with brain lumors
Asnong patients with a hrai,n tumor: 1. those that al1llll!t tandidnIQ fOT O'l[1Ul donotlon : A, meta"talk tumol'll to the b ra in B. brain tumcr~ rnat hllve been manipulated (biop.sied Or I!:lcised ) C, p/ltienl..$ with br/lin tumor" who hlV(' been 6h..."ted 2. tholle thAt m;ghtbe candida~, but eonlide.eII higl,..",k donora'" indude p,;p"IDtitd: A. glioblaslOtnIl rtlultifonne B. a naplastic astrocytoma C meduUnblBllOmp 3, unmlln;pulpted tumors that might not be considered high nsk A , henlsngiobiastnma B, meningioma
u.nma:.
Optimally, ifno mela!l!.ll.seSOI1l seen on CT,chest, abdomM lind pelvie) And no meu are found /It tune oforlClin procurem~nt, a brllin bioP"Y would beperfo,m"" II..fl&t Lhe 01" gan , aTil procured li t the same anesthetic bnd the organs would not be ~ele3Sed' until the biopsy prove... which of the 1I1x we o:IIU!gonl!.!l applle.s ,
9.3.3.
Management after brain death for organ donation
Note: OnCe b""i n dea th 0«Un. cardiovBlIC ular instability aventll.lly ensu e., genef'/Illy within 3·5 dayo, and management with preSSOI"ll; 8 u"ually requinod. Fl uid and elKtrolyte imballlnces from 10M of hYJIothalamk regulation mun be non:r"dl ~ed.ln eQme instanrtlS a beating·beart cadaYH can be maintbined for months". 1. ~osent: mustbeobUlined from donor'. legal guardian. NB: mUlltala<) be obtaoin
5.
nouo ADH (vasopreain (P;lreMin®J) if J1OSs1ble . ;nett th~ ri8k of renal s hutdown increase!. i n brain·death A. start with(:r)'l l alloid (05 V4 NS + 20 mEq KCIIL is gtntrally a good choke a ln ce. it repl~teS frO!t' water}, replace urine t'C for « plu. 100 cdhr mainlA>.nance B. un c>olloid IFrP, a lbumin .,,) if unabla to rnalnlem BP by replacement C. UIIe V"Opruso ", if 8tiU hypotensive. Start with low dl'lSll dopmrn;ne, h~ c.... a~e u p to - 10 l'gikg/min, add dobutamine ,(still hypot.enS;VD at this d!l!le O. If UO;s still,. 300 mUhr afte r above lIleaSUri!$, U!iC ADH anlliog ' aqUfOUli v8llo prea:l i~ (Pitretl>in®J i. prefem!d over DOA VP to a void ren8J. hutdovm ) thyroslobulin given TV convertl50me cent from anaerobic to ,,~robic rneta\lolillm which rnay h elp .. tav~ oll'CIlrdiDY3i1Ctlla. collapse
LABORATORY EVALUATION U
Gel)er a l iDitiallabll I. 2,
3.
serology: VORL ~r RPR, H8&\(, HIV, CMV,ABO blood group, HLA tiS8ue Lype dtellli,t.ry. eleclrolytes. gJUCOOle, BUN , ( reatilline, cal(iulII, phOllphate, liv~r f..."I:' t ion t.ests, U/A ( unn~ D.nD!y~i$) hematology: c ac , PTIP1"r
to"
A, h,fb ·rlok ."...ns ".1 b< OOlI.id
NEUROSURGERY
9, Brain deaLh
".
4.
microbiology: blood, urine II l1d sputum cul t ures; sputum Gram stain
Kid ney donor l.
2.
in addition to general labs (s« (Zbo~). check BUN & creati nine ~ q day check electrolytes ~ q 12 hrs (m!l(liry as appropriate )
Li ver do no r l.
in adwtion to gene ral labs ("'eabo~), check LDH, AST, ALT. bilirubin (di rect. indirect, and tota l)
Heart don or 1.
aU require an echooardiagram prior to don alioo
9.4.
References
""......... Comm,..'" roo III< s..~1 of !;lll i
,
,.
.. ,.
_ " " 10 M«I"I ..: C"jdoh ... rOt'~ "1r>1II;' ~.\6; 118H•. 1981 . Qualroy S~. 5"1><"",,"",_ or
fIMioo 01 .... ... . J"'M ..
t>r.,. ""..
GI-o<"".,
"",'"rio, ..
po'"''''
.fIe,
",'
'"
9 . Brain death
Tit.""" .. ofh,..,.....",.. • . M", ........ , 36: 116-7. ,~
"
.. RI .... ,.. t-l . o.p"'" 0 E.M""n.,.lC: S"",i.a! . I· .. ' "",Ioo&«! h1p¢O~nni • • N El>aIJ 101«1 HO: 119.
"
Goc>dm>." J M.1l«1; Ll. M<:><m B o ,conr""",.,. 011n;,.0..1I1 wIlh 1IOf" '" _ .., """,,",' " ..... wof2G4_"',"" ....... N.. """..." 16: '91·7,19U 0"",,"" J .l'nud I. Comparison .1 .... 0l."Il111 <1". lecliOI"""";"_J!)'<"Of)'mof ... .. ill1 ''''' IV",. ""
"
"
"""'<-of.
•• He,.."'
"
"
" " " ".
,~
0.""""'''''''"
cIcroOt. J...........
261 :n21 ·8 . 19~9 .
She..-""", I) .!..Co""", A M .fU
"'p. _«u'
NEUROSURGERY
10.1.
General information
~Mbrospi.nal 0 ... ;<1 (CS F) aUm;lUIlda Lhe brain lind _pinal 001<1, and !lilly functi,,m liS II shock 9bsorber ror the CNS. ll may 11150 storYe 9n lmmunoklgical fundion BllalQious to !..he lymphattcsYlltem'. i lcircula.tes within the subarachnoid space. between tlleanch·
noid lind the pial membranes. CSIi' is normally" d ..... coloillfflfl fluid wit.IL ~ 7.33-7.35.
Production 80%ofCSF ;8
for-9S~ (>fCSF
produ~
in the
....
--
s"""ific gr"y,ty of 1.007 lind
II
pH of
Table 10-1 Normal CSF production yolumea and prBB8Ure
prod"ted by the choroid plel(uSi!$, 10catA!d in both Jat.e raJ ventriclee (acoount.&
1\
P"",,,, IO\aI voIUIII! ( ICl(lT1;IIion ral'!
.......
I 5
25nWd
."
•
,.,,'
H OYTl
ISO (50"1. inlritcta
choroid plnu""s) (em o/nlid) lind in thl! 4th ven· tricle. Most ofth as me .... JI.a I" tl>lllumbir .,..OQf.ch_ opac9 ...ttII1h. 111"01 ,. I . ~.a In t~. rest of intrllcrania 1 "'leIal GeoJbolus posiTio(I production O«UrBin the in t.e rst.itial SPl>Ce'. A $l!IaU III[IOunt lI\ay also be produced by the e pendyma l lioing oflhe ventrieles. [n the spine, itis produood primarily in the dura orthe nerve root $leeve$, T,,(Jle JO-1 show.. properties o{CSF production, volumes Ilnd PTeiSureS.
Produdion rate:: In the adult, CSF is produced (It" rote orabou~O.3 mllmin (Re Table /o.J). In tenus thMared lnicollyrelevlUlt, this lipproximate54SQ mlt.!4hn, which mU M that in an adult, thJ, CSF i. ·~umed over' _ 3 times every day. The rate of fonnation is inthf"lnd~"t orthe inlrac ranial pressure!{except in the limitinK ~ when lep beeomu sg high that ce rebral hlood now is reduced').
Absorpt ion CSF is ~bsorbed primarily by ~rachnoid vi lli (granulations) u,alext..nd Into the duo ral venous linuses . Other s ites o[absorption include the-choroid plellUSf:. and lymphat.iC!;. The-rat.. of Bb50rpt;on is pr8S$ure dependent".
10.2.
CSF constituents
The cornpoiiition ofCSF differs lli,htly in the ventricles wb~re the produced compared to the lurnbar5uooruhnoid spare.
rnajori~
of it ;.;
CELLULAR COMPONENT
In norm al adult CSF, there are 0-5)ymphocyus or mononuclear cell5 per mrn~, Bnd polys (PMNs)or RBCs. ln the absenceofRBC., S· 10 WBCs pttr mm' is sUspicious,lInd .,. ~O WBes per mrn 3 is definitel)' abnoTmal , 110
NEUROSURGERY
10. CerebrospinalOuid
HI
CSF CONSTITUENTS Tab" 10-2 CSF .01111•• II, ,.." ,
_ T_ _ _ "...,.5(0) 0111 ""'" T_ s., 01 "Ce_oo.p;n.l Flulclln 0iIN_ oil"" N _ Syatem' "" ~ A. F l - . M.O, 11orCEA.AfP.&~CG .
CI'*. w. 8.~Co., I'IIiItM:IeIpI'~.P""u_w;",~
--
_tiIoIpIa<mo NoIe: CSfPfOl_ II ........ , in~ 1ILoicI,1wI1n....-..--.
_"""CSF_ns.s . ,
~_,...oI.In
... _
Ttbl.1004
.,
hble
1~4
CSF IIndlngs In varlOUII pathologIc conditions (ad ult vllu_)" (comlnued)
_,_iono OP
. _~ ~ :
Ine C6F!irlclir9t1ll n
__ IMI tJaciII .. _
I
... . _
. I . - . , , ; t ......
_ : ~. ~nItr
... """9a'" CSF ...a......*olI _ ••pIIl~_IIWr_U/ 1II~2!h1OlIo_.
1O~_Ir1_IICI~1tom
SAt1, eIto_
~
SAH __ ".......,.,'IA _
173
t "" ....... ~""\IIICSf' .. Io!S. _rMf1I51 TRAUMATIC r AP
D itre r e DtialillS' SAlI ITom traum Ati c tap For typi~.l findmg5 in SAM. JU ~ 784. Some f'''l!.l m W I from 'IT lire f howo in 7b~ 10-6.
NB: ""- candl!i<>r\s.CIaI' _
b~lpful
in difrerentiatiol!
oWI\fIoCIwomil
Differentioting tr1l e le ukocytollill l'rom tTlio umatie ta p
When many RaC. a nd WBC •• "" preo.en t In t he CSF d ue 1.0 a t raumatic lap ('rn, It may be important to eell if' lhll wa C. ar. elev.~ or 'fthey are prl..,nt in the ...... OItio 81 in ~he penphenll hlood. In non-anemic patieDia. Ihlll'1l shou ld be - 1·2 (01' every 1000 RBC. (alII. tonec:uon'" '''': l ubt' lIct 1 wac for livery 100 RBCIf .. '>II), In the presence of .neroi. or n Opbltal l.ukDeylolllli......1 f'i.hmlLQ'. fCN'Olul. lI • ''',oo''n in Bq IV. I toUtrmaUolhe origi nal WBC eountlo the CSF ~the TT.
wee.
I .
~BCC:UOIUG/I...L . WklC ar -
NEUROSURGERY
WIICB!.OOD" R8CCSI'
-
-
I!q t e. t
RHC,t.OOO
m
where wnCCSf'O~ICI"AL
K
wnc lXIun~ in ~heCSF befall!-U\e 'IT, WTJCcs., &; RaCcst''' WIIC
& RBCcounts mellllured in theCSF, Dnd WBC8I.OOI)& RBC8l..OOO" WBC & RIIC ~r mm1 in the peripheml blood ,
Estimat ing tru e Lota l CSF protei n CO l:ltel:lt with a traumati c tap If the hemogram lind peripheral protein are. normal. then hllve ~he c~11 count alld pro\.e.in content run an th,urne Lube, and the IXIlTection is'I.""': • 8ubtract 1 mg per 100 ml ofpro!ein far evel")'.1000 RBC per mm'
10.3.
Artificial CSF
II numberof famlulfttiOll8 ar "artificin l" CSF have hean Pl'1)posed OVer the yell1"8 in (lrder to mon dOlely mImic the pH. ~l1lolarlty. COt, and membr."e a~tiv" ion roncen· tration ofCSF . In many instance$, normal B8\.iDe (NS) 118.0 he~n used in brnin Burgary. probably without ronijequlmre. Howliologlc ",,\utioo8 when large vQlume8 Qf n uid lire exchanged . 8B <>CCIII'II during 80me Qr th~e procedLlres . An actu'" reliction tfth~.oIl1lJon".
E Uio tt's solution AKA Solution a or ElIiCK: lin d Jasper"· II. lin used in the pRIlL
10.4.
eJaborli~
formulillion that WAS widely
CSF fistula
AKA CSF leak. Two major J;uogrOllp.l: 1.
ijPQO~DeoLl$:
rare \,rE~tow)
2. post- procedura or poettrau.ma tie (mare I.'Ol1lmon): 67·17\\1 orcases. Including ~I. transaphftnoidol sUlgery snl,! poet skull b8ge s urgeI")'. Subgroups, A. immediate B. delayed CSF fistula should be BLlSpected in patient1! with otorrhea or rbmorrhea Bf\u head trsum8. orin p~tient.C wfth recurrent menillgitis .
Possible routes of egress o f CS F I. 2. 3. 4. 5
Ii. 1. 8. 9. 10
mastoid ait ~elll \u!'e<'ia llyaf\e. p-lOesa 8Urgery.e.g. forac:oustic neuroma (ANI . '"' PDlJt 435 ) s phenoid air cells (""perisHy po!I(..u1.n9Bphenoidal sU'l!el")') crlbrifornl plate/ethmoidal roof(Ooor of frontal fOSS.ll) frontal air cells hemialion into 1'trou~ ridge I). intenl.al auditory .,.,..al, fLlnowing tc'mporal bone fractUle or acoustic neuroma ,"rgery (,u pag~ 435). 'Then either : A. rbino .... bea: throllgh middle &Dr - e ust.achian l.ubli - nasopharynx a . o to .... b e a: via perforawd tympanic mertlhran. - ellLernal auditory ean&!
T RAUMATIC FIST\l LA
Occur in 2-3'l> of all p8tienl.! with bead ;'I.1UI")'. 6O'lb <>ccur within days oftrallmli. 95% within 3 n,onthBU, 70% (of CIl$e;I oI'CSF rhjnOlTheo It.op withIn I wk, and uSLlally within 6 mOl in the N5t . Non·traLlmatic fQeS cease apontlllleoualy L'I only 33%. Adut~child tati" il 10;1. 1':Ilrf! beroN ago, 2 yn. AnO!lmJ.a is.eommon in trallmatic leaks
'"
10. Carebrospinal Ouid
NEUROSURGERY
(78~),
TIIN!;n sponLln.., ... " . Moet (8Q.85%) CS F otorrhu ceases in 5-10 days_ CSP liltull oecurred in 8.9% of 101 easel of pe netrating trauma, and intreases the infection rat. over those penetrating injuries without fistu la (50% vs. 4 .6~)". It i$ reported to occur flO't·op in up to 30'l0 of eases of .kull·ban surge.,,".
SPONTANEOUS CSF FISTULA
Nontraumatic leaks primarily occur in adul", > 30 y .... Often inald iou5. May be mil_ ""ken for allergic rhinitia. Unlike traumatic leaks. these tend to be intermittent. the ...... of 1mell i. ulu.lly prtHrved, an d pneumocephalus ia Wlcommon". Sometimu lMOCilted wit h the following" 1. Igenesi. of the noor of the anterior faua (eribrifonn plate) or middle f _ 2. empty leila . yndrome: prima." or poSt tranAphenoidal ,urp." (He poet 454) S. increased ICP andlor hydrocepbalus 4. infection of the p8ranU81.intUles 5 . tumor: including pi t ui""ry adenomas (He p4fe 438), meningioma. 6. I pe ... il tent rtmna nt of th crlniopharyngeal ClInal" 7. AVM" 8 . dehiscenteofthe footplaLl of the ."'pes(a congenital Ibnonnality) which can produce CS F rhinorrh.. via the 11lItachian tube"
P08terior fossa 1.
2.
pediatric: usually preaents with either meningitl. or hearinc lou A. preserved labyrinthine function (hearing and b.lanee): these ulually prtsenl with meningitil. 3 ulull rolIteaofr.. tula: 1. facial ~llJlal: oan fistulize into middle ear 2. petromastoid canru: along path of arteriallupply to 111lI<:0II8 0( Olutoid air sinuses 3. 11yr1I's f1.-Uft! (AKA tymp.nomenillpal f15l ure): Hnka p-fous to hy. potympanum B. Inom alies oflab,.-ri nth (hearing IcMt ): ona of ..vera I typea of Mundini dy._ plllSillS, usually pru'!'nting with rounded. labyrintw-:hlea ~hat per mi'" CSF to erode through ova l or round willdow into auditory eIInll adul t: u~ually pn'senbl wit h conductive hearing loe5 with sera... err.... ion, OleningiUI rolW n follo";ng an epi.sode ofotilis media ), OTOIIreb1ll1 aba<:esa . Occurs most commonly through middle fOll&8. May be due to arachnoid granulationl erodi ng into lir linus compartment
Spinal Olkn pruenta with postura l headache associated with ned .tirrnea and Io!ndem e5S" (He pogf /78).
MENI NGITIS IN CSF FISTULA
InciderlC$ with !»'ttraumltic CSF leak: 5--1~, i~ ulealr. peniata > 7 da)'L Menln,giti.1t more common with aponl-aneoul fistula_ Ri$k may be highe r in poiSt- neurolurg'icil CSF fistula than in porit-traumaticdue toelevatad ICP comJllOn in la ller( fon:es CSF outward). If.ile ofl",k uoidentir.ed prior 10 a t loompted .urgical treaunen ~ 3O'lodevelop. ~rftnt leak !»'l-oJI, wilh 5--154 ofth ..... .re.eloping meningit il! bero", leak is stopped". Meningi til may promolo! in1lammatorychangH at the l ite of the leak. with a r ... "I1.alit oeuation ofthelealL Pneymoc;og:al menIngitis;' the IDOIt common pathogen (83'1> Of Cll$eS1O), mortali ty 1,lower than in pn.eumoo;oc:cal meningitil without unde rlying fistula « l~ VI. 50%). poaibly becauH thelatlo!r il frequently leen in elde rly debili""ted patients. Prognosis in children il WOrM".
EVALUATION
Dete rmining It rbinorTh ea o r otvrThe a i . due to a CSF fia tul a 1. ~h.racteriltka orthe nuid luggesting the preeence ofCSF A. nuid illII dear al Wiler (Wlleulnfected Or admixed with blood) B. fluid don not ~I"'H eltcoriat ion within or outllide the nose
NEUROSURGERY
10. C.rebTOllpioal nuid
C. pati,nte with r~inorrhea describe the t.R'U as l aity D. collect nuid and obl.llin qUBntiLa ti"8,' u<:oee (urine rlueoM detection I trip:t may be positive even with exceu mue ... ). Teat the n" Hi abortly .ner collection to roinillliu ferme nulion. Norma! CS F' IJluc_ 30 m&"lo ( .. I ... lly lower with meningiti l) wlMreaa lacrimal "cretions and muc ... are ul "ally < 5 m~. A negative test i. ~ he lpful , ince it ruin out CSf" (ncept in hypoel,.:orrhacbi a), but the .. il ' 4S· 16~ chance of r.IN poaitivet". ,.-, E. BrtrllUlafe ITln: pr,..nl in C$ f', but at..enl in t ...... u llvB. nu,,1 exudatu and IUUrn (except for newborns and patientl with liver diNu,)" ,", The only othe r !IOun;e i. the ,,;t ffl(lU I nu id of the eye. Dfttec~ by p. atein elec-
i.,.
t.rophores;I, . 0..5 ml needll.o be
pl.~
in aluri le conla inu, packed in dry
ie., V1d .hip~ to a lab that can p4!rlorm thilstudy F. "ri ng ' ign": when' CSF leak i. I Ulpe<:ttd but tho nuid i, blood tinged, allow the nuid to drip onto linen (Iheet or pillowcue). A ring of b]ood with. I.rrercooeentric rina of dear nuid (-.;I CII lled "double rint' or haloaign) luaant. the PftSenH ofCSF. As! old. but unn!1iab]", sign 2.. r.diographic ,igne of"pneumocephalul on CT or akull ~·ray 3. cinemogram; intrathecal inJectiQl"l ofradionuc:lido trlcer followed by lein tigram or il1jection of radiopt.que cont.rasl followed by CT lean (... ~ Mlowl 4 . atl(ll mia i, pre.enl in ~ $'lo ofCS F leaka 5. followina l kull ·baM "'1lI:ery (especially involving greate r l u;>erficial p 1.3 ,ulI"ta leak . If no le~k , the no. can be repacked and the study repea:.ed the following mom ,na· Leak. into fron tal sinul will ampt.y into nllOpharyu anterior I.<) the middle contha , unlike leak. throuah cribrirorm plate. RN C identifies the .ita in only 5O'A-. May be milleading" with pouible contamination afU r M veral OOUnl (rom ablorption o( rad ioilOtope into the bloodstream and accwm,l ,. tion in the muOOP] /danda ofthe turbinatot. Patient ~itionina mly ~ COQtaroinate other pled~ta C. intrathe<;l] (.. i" ble) dye I tudin, some luoeIII witb indiao aormine or Ouoreacein (1ft PfJII' 699) witb little or no complicatlons (II methyLene blue il neu rol.<)";c and should not IN used, _ PQlt 599) 5. MRl : ha. litt.le to offer in Jocalilation of"CSF fi. tula 00 below)
'"
10. Cerebroepina] fluid
NEUROSURGERY
WATER-SOLUBLE CONTRAST CT CISTERNOGRAPHY
nu. te$~ i. perfQrmNl lf: no l ite. identlfied j:1n pla in CT (with coronals) when patient is leakinlC clinically (the. aite 15 only 5Qmetim .... identified in the absence of an active leakl 3. when m\l.ltiple bony d,,(ecu art! ident;(ied. and it i. enential to determine which site is actively leakine 4. if a bony defect SlOen on pla;n CT does not have associa~ chan~ ofahnormal enhaocement_o( adJacent brain parench)'IQa
Prot~u .... ofrhol~.
L 2.
Tecbnique"" Use iohe~ol lue pu.je 127, which hill! geoerally replaced metriuom ide S. 7 mt or 190220 mp] ) [njerted iOl
via CI-2 punc~u",). Patienl positioned in -70" 'l'rendelenburg _ 3 min prone with nee). gently n""ed. in CT t.hey .rekept p[lme with head hypereKtended wi~h I> mmcoronal cuts with 3 mm overlap (U6" 1.0 IOIJl CUtii if necessary). May n...ed provocative. maneuvers (coron al llCIII"\8 prone {brow up) or in position o(lellk, iotratilecall8l inei.n.fu.sion (requirea Harvard pump)'·" .) Look for aCC\Jm ll l~tiQn ofconLrast in air sinuses. Apparentdi5conLinuity ofbone on CT without extraV8$S8tbn of contrast ;. probably not the ~ite of leakpge (boRe_diseootinuities may be mimicked by partial Yolume ll'·f.r~ging on CT).
MRI MRJ pro..-ides little additional informa!'!on for IOC/Ili:wtion , but it CStn RIO I>"fo""l1 mo:;.s , tumor, and elllpty3e1!~ bo!tter than CT. Both CT and MRl C"IIn RIO hydrocephalll6. TREATMENT
AcutA!ly a~r traum8. ObuNation ia jWltilied as IIIos1 fa ....8 ceo"'losp"[ltatll!Oualy. Propbylactic anti bioti C8: Con tro~ersial . There w;;u no differencc io !.he incidenfe Or n)Orbidlty ofllleningltia between trtlMed o.nd untreat«! patients"', Furthermore, the ,;~k of8~lectiug reaistaolst.ra ins sppeal"l real" and i3 thoenrorflll8ulllJy avoided . FOR PERSISTErff POS17RAUW.rJC OR POST·OP LEAKS
NOll -s urgi ca l trea tllle llt. I. rnellsures t.o low~r ICP;
2.
A. bed retlt ; although recumtMtncy may am eliorllte SYrQptom l, !.here i~ no other benefit froro bed rest'"' B. avoid atrair,ing(stoolsof\enel"l) and avoirl blowing nOlle. C. aeet.a:u>laIOlde (260 mg PO QrD) t.o /"l!dUCf! CSF pN>dudioll D. modest nu;d A$tridion (eaution po
OR
3.
B. continuous JumblU" drn..in.age (C LOt via percutaneous catheter. Ke-ep HOB el""al.<:d 10-15" ,."d drip ch8lllber "l shoulder le"",1 (,;4ju8t down if leak pil .... iatli). RequIres leu mouitoring. Irpatient oeUlrioratQ with dr"in in place: illlmedi awJy st(Jpdrainage. place patien~llat in bed ~or !light fun· ~lenburg), st.art 100% 0 •. ge~ CT or bedsideCTOEsH lible .kull x-ray\to RIO tension poeumocephalu8 due. to drawing in orait) lurgkal treatment in persistent cases (...-e below )
SURGICAL mEATMENT
ind ica tions ro r
8lU"gh~al
1.
CSF leak
2.
leaks
3.
inte rve n tion
h
P e trou a bone May ptellent 1111 otorrhea or 8S rhi noIThea (via the Imstachilm tube ). NEUROSURGERY
measures !U r gety: UIIU_
po,.,
1.
following poat.erior {()$.'ja ~u r g1!ry: IIf'e 435 for treatmen t following aco.>ustic neuroma eurgery 2. following mastoid fractures: may be approached via extensi,·" mastoidectomy"
Leaks thr ough c ribrifo nro
pla t~/ethmoi da1
roof
Extradura l fl pproacb: Gi! neralJy preferred by ENTlurge0n3"' . lfa frontal craoiotomy is being performed. an intnadural approach should be used ,ince problems may M$e In dissecting the dura olfofthe floor oftbe frontal fossa, wherein the dura almost slwllY~ tea", and then it is dilTtcult to know if an identified tear is the cause of the leak or ifit is iatrogenic. Fluorescein dye m;"ed with CSF injected intrathecally may helpdemomtrate the leak intraoper8tivelyICAIITION: must be diluted to red uce risk ofsei.ures, see pog~ 599). Intradural approach: Generally the procedure of choice". If the fistula site is uniden· tified preoperatively. use II bifrontal bone nap. General techniques of intradural approach : Post op: lumbar drain after craniotomy is eontrove"'ial. Some reel CSF preuurt may help enhance the seal". Ifuserl, place the drip chamber a t the level of shoulde r for 3·5 days (for precautions. $ff COOtie). Conside r shunt (LP Or VP) if elevated ICP Or hydrO)a'!phaiua is demonstrated.
Leak s io to s pbe noid ,;null (incl uding post-tranllllp be noidai a urgery leak) I.
2. 3.
LP BID or C!..D, as long as pressure" ISO mm H~O or CSF ¥anthochromie A. ifleak persist$ " 3 days: repack sphenoid sinus pnd pterygoid recesses with fat. muscle. cartilage Mdfor fascia lata (must reconstruct floor of sella, packing alone i$ inadequate). Some recommend against muscle sin.::e it putrelles lind shrinks. Continue LP or CLO as above for 3·5 days post-op Il if leak persists ,. 5 days: lumboperitoneal shunt (first RIO obstructive hydrocephlllus) mOte difficult surgical approach: intracranial {intradu rall approach to medial fI9peet of middle cranial fossa consider transnaspl..,llar injection of fibrin glue under local anesthl!$ia"
10.5.
Spontaneous intracranial hypotension
The syndrome of spont aneous intracranial hypotension is cha racterized by the fol· lowing in the a.brwlg; of antecedent trauma or LP (or epidural injection ... "': 1. orthosta t ic headache 2. low CSF pressure 3. diffuse pachymeningeal eohancement on cerebral MRI In most cases, the underlying etiology is thought to be II sponlan eousCSF leak from a spinal meningeal diverticulum or dural tea r".
Clinical features Most patients have orthostatic hel>dache. Atypical patients bave been described withcu.t lilA , or HlA tbat i$ !)On_pOiIitional, without pa.;hymeninll:e.. l enhancement on MRl", with dinicalaigns of encephalopathy, cervical myelopathy, Or parkinsonism". Sinti! some plltients roay have normal intracranial preuuTe. the term ·CSF hYJlD"olemia" has beeo suggested'". MRI evidence of brain df!S<'enl oceurred in 36%"'. and reyenl!ible pi tu itary enla rgem~nt with a eonvu superior margin" may also be . een . Subdural bematomas may occur as a result. Radioisotope ciste.oography was abnonDal io 90%, and showed 8 leak in 40%".
Treatment l'reatment includes: 1. bed rest 2. analgesics 3. hydration 4. epidural blood ""t.ch (EDP) for appropriate cases:"" poge 46
Outcome Complete resolution ofHlA was pchieYed in 70%, and was higher in patients ,""ceiy. ing EBP, and W illi lower in patients with multiple .ites ofCSF leak".
'"~
]0. Cerebrospioal fluid
NEUROSURGERY
10.6.
,.
,. ,
References , _01_.,.._CSI'~
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n.-t J l ' ·191·'. I991. E>. ... ~ _ _ '"
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s-O.II< .... JEA
~"" Iloid. '''''N<", ' 9 U~$. 1961
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679-91, IknII&EA.S.... O' H~· c-F· .. b·
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,~
Grilf.~
118. 1...".,.... ... 8: no. .... _ol
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I. J
1910.
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