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Lateralmedullarysyndrome(orWallenbergsyndrome),causedbyposteriorinferiorcerebellarartery
(PICA)occlusion,classicallypresentswithvertigoaswellasahostofotherneighborhoodsignsand
symptomsincludinghoarseness,dysphagia,nystagmus,Hornersyndrome,andhemisensoryloss(see
page68).Whitearrowispointingtothelateralmedullaryinfarct.(CourtesyofCarlosTorres,MD.)
Box5.5
Whenneurologistshearapatientdescribetheirsymptomsas“acuteonset,”thefirstthing
thatcomestomindisvascularpathology:ischemicorhemorrhagicstroke.Thisisan
absolutelyappropriateresponse,butitiscertainlyworthpointingoutthat“acute-onset”
vertigoshouldnotswayyouinexorablytowardacentraletiology.Bothperipheralandcentral
vertigocan(andoftendo)presentacutely.
Box5.6DisembarkmentSyndrome(maldedebarquement)
Haveyoueverhadatransientsenseofimbalance—likeyouarestillfloatingup-and-downon
thewaves—whenyoustepoffaboatontodryland?Thatunpleasantsenseofdisequilibrium
mayoccurwithyourfirststeponshoreorseveralminutestohoursafterabandoningthehigh
seas.Thissensationisnormalifitisbrief(lastingminutestohours),butifitbecomes
persistentitisreferredtoasdisembarkmentsyndrome.Formaldiagnosticcriteriainclude
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persistent,non-spinningvertigo(patientsoftendescribeasensationofswayingorrocking)
lastingmorethan48hours,withonsetlessthan48hoursfromexposure.Re-exposureto
passivemotionshouldtransientlyrelievesymptoms.Disembarkmentsyndromecanlast
weekstoyears.Disablingfatigueandcognitivedifficultiescanalsooccur.Theetiologyis
unknown,andtreatmentisdifficult.Scopolamine,meclizine,anticonvulsants,anddiuretics
havenotbeenfoundtobereliablyeffective.Somepatientsmayexperiencesomebenefit
fromabenzodiazepineorselectiveserotoninreuptakeinhibitor.Vestibularrehabilitationhas
notbeenfoundtobeconsistentlybeneficial.
ABriefWordonSyncope
Syncopeisthetransientlossofconsciousnessduetoinadequatebloodflowtothebrain.Patientspresent
withalossofmuscletoneandsubsequentcollapse,followedbyarelativelyquickreturntobaseline.
Precedinglightheadednessiscommon.Brieftonicormyoclonicmovementscanalsooccur;thisisknown
asconvulsivesyncopeandisacommonandbenignsyncopalvariant(seepage165).Presyncopeshould
beevaluatedthesameastruesyncope,asthecauses—bothbenignandserious—arethesame.
Thereare,forourpurposes,threemajortypesofsyncope:
1. Reflex syncope is very common. It is caused by a sudden drop in blood pressure or heart rate due to alterations in autonomic
activation, either increasedparasympathetic or decreased sympathetic tone. Vasovagal syncope isthe most commontype of reflex
syncopeandclassicallypresentswithprodromallightheadedness,diaphoresis,pallor,palpitations,nausea,and/orblurringordarkeningof
visionpriortothelossofconsciousness.Ifyoususpectvasovagalsyncopebut,forwhateverreason,arenotcertain,tilttabletestingcan
behelpfultoconfirmthediagnosis.Situationalsyncopeisanothertypeofreflex syncopeand referstosyncopeduetoanidentifiable
triggersuchasurination(referredtoasmicturitionsyncope)orcoughing.
2. Syncope due to cardiopulmonary dise ase . Since this isa book about neurology,we won’t expoundonthis topic. Causes include
arrhythmias and structural cardiopulmonary diseases, such as aortic stenosis or hypertrophic cardiomyopathy with significant left
ventricular outflow obstruction. From a neurologic perspective, it is important to have a low threshold to refer to cardiology when
patientspresentwithotherwiseunexplainedsyncope.Concurrentneurologicandcardiacevaluationsareoftenideal.
3. OrthostaticSyncope.Orthostatichypotensionisdefinedasadecreaseinsystolicbloodpressureofatleast20mmHgoradecrease
indiastolicbloodpressureofatleast10mmHgwithin3minutesofstanding.Therearethreemaincauses:
Volum edeple tion.Signif icantvolum ede pletioncanbe duetovom iting,bleeding,orj ustdehy dration,am ongm a ny otherpotentialc auses.
Medic ations.Thislistisalongonea ndincludesm edic ationsthatcause vasodilation,volume depletion,anda utonomicdy sr egulation.
Autonomicfailure.Autonom icfa ilurec an bedue toneur odege nera tivedisea ses( sucha sPa rkinsondise ase or Multiple Sy stem Atrophy ;se eChapte r13) ora utonomic neur opathy (c ause dm ostoftenby diabete s,butdon’tforget
toconsiderthem a ny otherpote ntialetiologiesincludingamy loidosis,connec tivetissuediseases,vitam indef iciencie s,andva riousinfec tions;seeChapter11) .
Areyouwonderingwhytransientischemicattacks(TIAs)arenotonthislistofcausesofsyncope?It’s
acommonmisperceptionthatTIAsfrequentlypresentwithsyncope.VertebrobasilarTIAscancauseloss
ofconsciousnessifthalamicstructuresareinvolved,butforthistooccurinisolation,withoutanyother
neurologicdeficits,isveryrare.TheAmericanAcademyofNeurologyrecommendsagainstcarotidartery
imagingforsyncopewithoutthepresenceofotherneurologicsymptoms;occlusivecarotidarterydisease
resultsinischemiaofbraintissuesuppliedbytheophthalmic,anteriorandmiddlecerebralarteries,as
wediscussedinChapter2,andcausesfocaldeficitssuchasweakness,numbness,andvisualfieldcuts,
butitdoesnotcausesyncope.
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YourPatient’sFollow-up:AlthoughKyle’svertigoisimproving,youorderanMRIbecause
you’vefoundconvincingfocalityonhisexamination.Thenystagmusishardtosee—thisis
notunusual!—butyouthinkit’sthere,andyouareconfidentabouttheleftupperextremity
dysmetria.NowthatyouknowabouttheHINTStest,you’vealsonotedanegativehead
impulsetestandpositivetestofskew,bothconcerningforacentraletiology.Hissymptoms
areallbutresolvedbythetimetheMRIiscompleted,butthescanisnotableforasmall
acuteinfarctintheleftcerebellum.Youadmithimforastrokeevaluationand,onreviewing
histelemetrythefollowingmorning,noteabout30minutesofatrialfibrillation.He’ssoon
dischargedonanticoagulationandisgratefulforyourhelp.
Younowknow:
1. Thatacutedizzinesscanmeaneitherlightheadednessorvertigo.Lightheadednessismostoftennotaprimarilyneurologiccomplaint;it
is moreoftenthe resultofdehydrationor,lesscommonlybutimportantly,underlyingcardiacdisease.Vertigoisaneurologicsymptom
andrequiresacarefulhistoryandexaminationtoappropriatelymanageandtreat.Disequilibriumisanothertypeofvertigo—chronic,as
opposedtoacute—that’smostoftenseenintheelderly.
2. Vertigo doesnotalwaysmeanspinning!Vertigoisdefined as the false sensationofmovement,orthesensationofmovement when
nothingisactuallymoving.
3. BPPV, Meniere disease, and vestibular neuritis are common causes of peripheral vertigo. If the history is straightforward and the
examinationconsistent,anMRI is notneeded.Butifyouare unsure,orifyourexaminationisconcerningfor central etiology,getan
MRI.
4. Centralvertigoismostoftencausedbyischemicorhemorrhagicstrokewithinthebrainstemorcerebellum.Multiplesclerosislesionsin
thesameregionscanalsopresentwithvertigo.Suspectcentralvertigowhen“neighborhood”signsorsymptomsarepresent.
5. Syncope can be divided into reflex syncope, syncope due to cardiopulmonary disease, and orthostatic syncope. TIAs rarely cause
syncope.Iftheydo,itistheposteriorcirculation(i.e.,vertebrobasilarsystem)thatisresponsibleandnottheanteriorcirculation.Carotid
dopplersarenotrecommendedforpatientswhopresentwithsimplesyncopewithoutotherassociatedneurologicfindings.
1
Menierediseasewaspreviouslybelievedtobetheresultofexcessendolymphwithinthesemicircularcanals,butthecurrentconsensusis
thatso-calledendolymphatichydropsismorelikelyamarkerfordiseaseinsomepatientsratherthanthecause;itisn’tevidentinallpatients,
andmanypatientswithhydropsdonothaveclinicalevidenceofMenieredisease.
2
Itisthoughtthatmigrainousvertigoisatypeofcentralvertigopredominantlybecauseevaluationforperipheralcauses(i.e.,earornerve
dysfunction)comesupnegative.
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6Seizures
Inthischapter,youwilllearn:
1. Howtoclassifyanddistinguishamongdifferenttypesofseizures
2. Howtomanageafirst-timeseizureinanurgentsetting
3. Howtosortthroughandmakesenseoftheantiepilepticmedications
4. Howtodefine,classify,andtreatstatusepilepticus
5. Howtodiagnoseandmanagepsychogenicnonepilepticseizures(PNES)
CASE6
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YourPatient:Carlton,a58-year-oldaccountantwithnoknownmedicalhistory,isbrought
intotheemergencydepartment(ED)byemergencymedicalservices(EMS)afterhewas
foundonthegroundinasupermarketparkinglot.EMStellsyouthatawitnessatthescene
sawseveralminutesoffull-bodyshaking,butthishadresolvedbythetimeEMSarrived.The
patient’svitalsarestableandhisfingerstickglucoseiswithinnormallimits.Heappears
sleepy,butwhenheopenshiseyes,younoticethathe’slookingpreferentiallytotheleft.
Youdon’tnoticeanyabnormalshakingmovements.Hewon’tfollowcommandsbutseemsto
withdrawhisrightarmandleglessbrisklythanhislefttonoxiousstimuli.Whatisthefirst
stepinyourmanagement?
Seizuresaresuddenburstsofabnormalelectricalactivityoccurringwithinthecerebralcortex.Because
theycanhappeninanyareaofthecortex,seizurescancausealmostanyneurologicmanifestationyoucan
imagine,fromdramaticmotoreventswithlossofconsciousnesstosubtlesensoryorbehavioral
abnormalities.Sometimesyoucanevenfindelectricalactivityonanelectroencephalogram(EEG)
indicativeofaseizurewithoutanyassociatedclinicalmanifestationsatall.Seizuresareamongthemost
commonandtreatableconditionsinallofneurology.
Epilepsy
Noteveryonewhohasaseizurehasepilepsy.Asmanyas1in20peoplewillhaveasingleseizurein
theirlifetime,butonlyapproximately1in50willbediagnosedwithepilepsy.Thediagnosisofepilepsy
requires:
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Atleasttwounprovokedseizuresoccurringmorethan24hoursapart
OR
One unprovoked seizure, plus the probability of further seizures must be similar to the risk of
recurrenceaftertwounprovokedseizures(atleast60%)—don’tworry,wewillexplainthisinjusta
moment
OR
Thediagnosisofaspecificepilepsysyndrome(seepage170)
ProvokedVersusUnprovokedSeizures
AccordingtotheInternationalLeagueAgainstEpilepsy(ILAE)guidelines,a“provoked”seizureisa
seizurethatisdirectlycausedbyanacute,symptomatic1condition.Sepsis,hypo-orhyperglycemia,and
alcoholwithdrawalarecommoncausesofprovokedseizures.Otherconditions—stroke,traumaticbrain
injury,intracranialsurgery,andpostanoxicencephalopathy—areconsideredacute,symptomatic
conditionsonlyiftheseizureoccurswithinthefirst7daysfollowingtheinsult.
An“unprovoked”seizureisaseizureofunknownetiologyoraseizurerelatedtoapreexistingbrain
lesionorpreexistingdisorder(i.e.,a“remote,symptomatic”condition2).Suchlesions—anoldstroke,
forinstance,orabraintumor—canactasseizure“niduses”inthattheycancreateirritablebraintissue
thatispronetoseize.Butwhy,forexample,doesapatientwithastrokethatoccurredmanyyearsago
seizeforthefirsttimetoday?Theanswerisoftenelusive;thus,intheabsenceofanacute,symptomatic
condition,thatseizureisconsideredunprovoked.
WhatConditionsIncreasetheRiskofRecurrenceAfteraSingleSeizure?
Let’sgobacktothatseconddefinitionofepilepsyforamoment:oneunprovokedseizure,plusthe
probabilityoffurtherseizuressimilartothegeneralrecurrenceriskaftertwounprovokedseizures.
Thechanceofseizurerecurrenceislowfollowingasingleseizurebutincreasessignificantlyaftera
secondseizure.So,whatfactorsincreasethisriskafterjustasingleseizuresuchthattheriskishigh
enoughtopotentiallywarrantthediagnosisofepilepsy?Thereareonlyahandfulofthings:
AnabnormalEEG.AnEEGthatis“irritable”or“epileptiform”showsabrainthatisnotactively
seizingbuthasthepotentialtoseize.Itisthereforenotsurprisingthatspecific“irritable”featureson
anEEG(the details of this are beyond thescope of this book)are associatedwith a significantly
increasedriskoffurtherseizures.
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AnabnormalEEGshowinghigh-amplitudegeneralizedfast4-to-6Hzandslowwavesinateenagerwith
myoclonicandgeneralizedtonic-clonicseizuresuponawakening,characteristicofjuvenilemyoclonic
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epilepsy(seepage173).(ReprintedfromWyllieE,CascinoGD,GidalBE,GoodkinHP.Wyllie’sTreatment
ofEpilepsy.5thed.WoltersKluwer;2012.)
Remote,symptomaticseizures.Althoughanoldstrokeorknownbraintumordoesnot“count”asan
acute, provokingfactor, theselesionscanrepresenta locus ofirritable braintissue. Patients with
theselesionshaveanincreasedriskforfurtherseizures.
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Achronicleftmiddlecerebralartery(MCA)distributioninfarctonCTthatcouldserveasthesourceof
seizureactivity.(ReprintedfromWeinerWJ,GoetzCG,ShinRK,LewisSL.NeurologyfortheNon-
Neurologist.6thed.WoltersKluwer;2010.)
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