22/2/2016 Convulsivestatusepilepticusinadults:Classification,clinicalfeatures,anddiagnosis

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Convulsivestatusepilepticusinadults:Classification,clinicalfeatures,anddiagnosis

Author SectionEditor DeputyEditor

FrankWDrislane,MD TimothyAPedley,MD AprilFEichler,MD,MPH

Disclosures:FrankWDrislane,MDNothingtodisclose.TimothyAPedley,MDNothingtodisclose.AprilFEichler,MD,
MPHEquityOwnership/StockOptions:Johnson&Johnson[Dementia(galantamine),Epilepsy(topiramate)].
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedbyvetting
throughamultilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthecontent.
Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Jan2016.|Thistopiclastupdated:Nov20,2015.
INTRODUCTIONStatusepilepticusisarelativelycommonmedicalandneurologicemergencythatrequires
promptevaluationandtreatment.Therearemanydifferentstatusepilepticussyndromes,definedbyclinical
featuresandEEGfindings.Causes,prognoses,andtreatmentsdiffer,andoptimalevaluationandtreatment
requiresanunderstandingofboththetypeofstatusepilepticusandtheunderlyingcause.Someformsof
statusepilepticushaveanexcellentprognosis,whereasothershaveahighlikelihoodofmortality.

Theclinicalfeaturesanddiagnosisofconvulsivestatusepilepticusinadultsisdiscussedheretreatmentis
reviewedseparately.Nonconvulsivestatusepilepticusandthediagnosisandmanagementofstatus
epilepticusinchildrenarealsodiscussedseparately.(See"Convulsivestatusepilepticusinadults:Treatment
andprognosis"and"Nonconvulsivestatusepilepticus"and"Clinicalfeaturesandcomplicationsofstatus
epilepticusinchildren"and"Managementofconvulsivestatusepilepticusinchildren".)

DEFINITIONThedurationofcontinuousseizureactivityusedtodefinestatusepilepticushasvariedover
time.Historically,theInternationalLeagueAgainstEpilepsy(ILAE)andothersdefinedstatusepilepticusasa
singleepilepticseizureof>30minutesdurationoraseriesofepilepticseizuresduringwhichfunctionisnot
regainedbetweenictaleventsina30minuteperiod[1].

Becauseoftheclinicalurgencyintreatinggeneralizedconvulsivestatusepilepticus(GCSE),however,a30
minutedefinitionisneitherpracticalnorappropriateinclinicalpractice.Onceseizureshavecontinuedformore
thanafewminutes,treatmentshouldbeginwithoutfurtherdelay.

ConsideringtheneedforrapidevaluationandinterventioninGCSEtoavoidcardiovascularmorbidityand
refractorystatus,anacceptedoperationaldefinitionofGCSEconsistsofthefollowing[24]:

5minutesofcontinuousseizures,or
2discreteseizuresbetweenwhichthereisincompleterecoveryofconsciousness

In2015,theILAEpublishedarevisedconceptualdefinitionofstatusepilepticusthatincorporatestwo
operationaldimensions,t1andt2[5]:

Statusepilepticusisaconditionresultingfromeitherthefailureofthemechanismsresponsiblefor
seizureterminationorfromtheinitiationofmechanismsthatleadtoabnormallyprolongedseizures(after
timepointt1)

Statusepilepticusisaconditionthatcanhavelongtermconsequences(aftertimepointt2),including
neuronaldeath,neuronalinjury,andalterationofneuronalnetworks,dependingonthetypeanddurationof
seizures

ForGCSE,theILAEproposalspecifiesthatt1andt2are5and30minutes,respectively,basedonthebest
availabledatafromanimalandclinicalstudies[5].Forothertypesofstatusepilepticus,themostappropriate
timeintervalsfort1andt2havenotbeenwelldefinedandarefarmorespeculative,particularlyfor
nonconvulsivestatus.TheILAEsuggestsusingat1andt2of10and>60minutesforfocalstatusepilepticus
withimpairedconsciousness(oftendiagnosedascomplexpartialstatusepilepticus)andat1of10to15
minutesforabsencestatusepilepticus.

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EPIDEMIOLOGYThereportedyearlyincidenceofstatusepilepticusrangesfrom7to41casesper100,000
[6].Mostpopulationbasedstudieshavedefinedstatusepilepticususingadurationofatleast30minutes.

Thewiderangeofreportedincidenceratesislikelyduetodifferencesincaseascertainmentandthe
populationsbeingstudied.Inaddition,somestudieshaveincludedbothconvulsiveandnonconvulsivestatus
epilepticus(NCSE),whileothershaveexcludedNCSE.Inastudythatincludedonlygeneralizedconvulsive
statusepilepticus(GCSE),thereportedincidenceratewas7per100,000[7].

Thereisabimodalagedistribution,withpeakincidenceratesinchildrenlessthanoneyearofageandadults
overtheageof60years[6].Overalifetime,upto10percentofadultswithepilepsyand20percentofchildren
withepilepsywillgoontohavestatusepilepticus[8].

ETIOLOGYMostcasesofstatusepilepticusinadultsaresymptomaticofanunderlyingstructuralbrain
lesionoratoxicormetabolicdisturbance[9].Iftheunderlyingmedicalorstructuralcauseisofrecentorigin,
statusepilepticusisreferredtoasacutesymptomatic.Manyepisodescomefromacombinationofanearlier
lesion(ie,remotesymptomatic)andasuperimposednewmetabolic,infectious,orpharmacologicstressorsuch
asuremiaoramedicationchange.

Statusepilepticusalsocommonlyarisesinpatientswithanestablisheddiagnosisoffocalorgeneralized
idiopathicepilepsy.Statusepilepticusisoccasionallythepresentingmanifestationofepilepsy[10].

Commoncausesofconvulsivestatusepilepticusvarybyage.Inchildren,febrilestatusepilepticusisthemost
commonetiology,accountingforapproximatelyonethirdofcases.(See"Clinicalfeaturesandcomplicationsof
statusepilepticusinchildren",sectionon'Epidemiologyandetiology'.)

Inadults,themostcommonetiologiesareacutesymptomatic,accountingforapproximatelyhalfofallcases,
followedbyremotesymptomaticandlowantiseizuredruglevelsinapatientwithknownepilepsy[1012].
Examplesofsomeofthemorecommoncausesinadultsinclude:

Acutestructuralbraininjury(eg,stroke,headtrauma,subarachnoidhemorrhage,cerebralanoxiaor
hypoxia),infection(encephalitis,meningitis,abscess),orbraintumor.Strokeisthemostcommon,
especiallyinolderpatients.

Remoteorlongstandingstructuralbraininjury(eg,priorheadinjuryorneurosurgery,perinatalcerebral
ischemia,arteriovenousmalformations,andbenignbraintumors).

Antiseizuredrugnonadherenceordiscontinuationinpatientswithpriorepilepsy.

Withdrawalsyndromesassociatedwiththediscontinuationofalcohol,barbiturates,orbenzodiazepines.

Metabolicabnormalities(eg,hypoglycemia,hepaticencephalopathy,uremia,hyponatremia,
hyperglycemia,hypocalcemia,hypomagnesemia)orsepsis.(See"Evaluationofthefirstseizurein
adults",sectionon'Acutesymptomaticseizures'.)

Useof,oroverdosewith,drugsthatlowertheseizurethreshold(eg,theophylline,imipenem,highdose
penicillinG,cefepime,quinoloneantibiotics,metronidazole,isoniazid,tricyclicantidepressants,
bupropion,lithium,clozapine,flumazenil,cyclosporine,lidocaine,bupivacaine,metrizamide,
dalfampridine,and,toalesserextent,phenothiazines,especiallyathigherdoses).

Anincreasinglyrecognizedcauseofconvulsiveseizuresandstatusepilepticusisautoimmuneencephalitis,
whichsometimeshasanunderlyingparaneoplasticetiology.Antibodiestocomponentsofthevoltagegated
potassiumchannelcomplexandtheNmethylDaspartate(NMDA)receptorareamongthemorecommon
causes.Inonesinglecenterseriesof570consecutiveepisodesofstatusepilepticusinadults,2.5percentof
caseswerecategorizedasautoimmune[13].Specificetiologiesincludedmultiplesclerosis(fourcases),anti
NMDAreceptorencephalitis(twocases),adultonsetRasmussenencephalitis(onecase),Hashimoto
encephalitis(onecase),andcomplicationsoflupusvasculitiswithhemorrhagicstroke(onecase).Clinical
presentationscanrangefromconvulsivestatusepilepticustoconfusionaloramnesicstates.Autoimmune
casesareoftenmorerefractorytotreatmentcomparedwithothercausesofstatusepilepticusbutmayrespond
favorablytoimmunomodulatorytherapy[14].(See"Paraneoplasticandautoimmuneencephalitis"and
"Convulsivestatusepilepticusinadults:Treatmentandprognosis",sectionon'Immunomodulatorytherapy'.)
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Newonsetrefractorystatusepilepticus(NORSE)isasyndromedescribedinseveralreportsofpatientswho
presentwithseveregeneralizedseizuresandstatusepilepticusofunclearetiology,ofteninthesettingofa
prodromalfebrileillnesssuggestingaviralencephalitis[1519].Patientstypicallydonotrespondreadilyto
antiseizuredrugsandmortalityandmorbidityarehigh.

CLASSIFICATIONStatusepilepticus,likeseizures,iscategorizedelectroclinicallyaccordingtowhether
seizureactivityisfocalorgeneralized.Inmanycases,however,generalizedconvulsivestatusepilepticus
(GCSE)cannotbeseparatedeasilyintocaseswithaprimarilygeneralizedonsetversusthosewithfocalonset
andsecondarygeneralization.MostGCSEhassomeevidenceofafocalonsetorfocallesion[11].

Thedistinctionbetweenfocalandgeneralizedonsethasclinicalimplications:incaseswithfocalonset,a
causativelesionmustbesoughtinprimarygeneralizedepilepsies,certainantiseizuredrugs(eg,phenytoin,
carbamazepine,andoxcarbazepine)shouldbeavoided.(See"Convulsivestatusepilepticusinadults:
Treatmentandprognosis",sectionon'Otherseconddrugs'and"Convulsivestatusepilepticusinadults:
Treatmentandprognosis",sectionon'Myoclonicstatusepilepticus'.)

Bothfocalandgeneralizedstatusepilepticuscanbefurtherclassifiedaccordingtowhetherclinicalseizure
activityisconvulsiveornonconvulsive(table1).Convulsiveformsofstatusepilepticusarethefocusofthis
topicnonconvulsivestatusepilepticusisdiscussedseparately.(See"Nonconvulsivestatusepilepticus".)

Classifyingthetypeofstatusepilepticusisimportantbecauseitisamajorfactorindeterminingmorbidityand,
therefore,theaggressivenessoftreatmentthatisrequired.Forthepurposesofthistopic,wedistinguish
amongseveraldifferentformsofconvulsivestatusepilepticus:generalized,focalmotor,myoclonic,andtonic.

CLINICALFEATURESPatientswithconvulsivestatusepilepticuspresentwithcharacteristicmotor
manifestationsthatvaryaccordingtotheseizuretype.Whilepatientswithgeneralizedconvulsivestatus
epilepticus(GCSE)haveobviousbilateraltonicandclonicmotoractivityandlossofconsciousness,patients
withfocalmotorstatusepilepticusmayhavejerkingmovementsrestrictedtooneareaofthebody,usually
withpreservedconsciousness.Myoclonicstatusepilepticustypicallyinvolvesmuchmorerapid,butlower
amplitude,jerkingmuscleactivity,butwithmarkedvariability.Tonicstatusepilepticusincludesslower,more
sustainedmaintenanceofaposture,orslowmovement.

GeneralizedconvulsivestatusepilepticusGCSEisthemostdramaticformofstatusepilepticus,withthe
potentialforseriouscomplications,morbidityandevenmortality.GCSEincludesbothprimarygeneralizedand
secondarilygeneralizedconvulsiveseizures.Thereisalwaysimpairedconsciousnessandbilateraltonic
stiffening,followedbyrhythmicjerkingofthelimbs(clonus)thatisusuallysymmetric.

FocalmotorstatusepilepticusFocalstatusepilepticushasmanyclinicalmanifestations,largely
dependingonthelocationoftheepileptogenicbrainarea.Focalmotorstatusepilepticusisthemosteasily
recognized.Itmayhaveprogressionoffocaljerkingactivityofalimb(aJacksonianmarch)orwidespreadbut
unilateraljerkingmuscleactivity,withorwithoutimpairedconsciousness.

Inalmostallcases,thereisanassociatedfocallesion,althoughthelesionisnotalwaysevidentonimaging.
Examplesofcausativelesionsincludeheterotopias,vascularorinfectiouslesions,andtumors.Occasionally,
benignidiopathicfocalepilepsiesleadtostatusepilepticusofthesametype.(See"Localizationrelated(focal)
epilepsy:Causesandclinicalfeatures",sectionon'Benignfocalepilepsiesofchildhood'.)

Aparticularlyrefractoryfocalmotorstatusepilepticuswithveryprolongedandveryregularjerkingactivityis
calledepilepsiapartialiscontinua(EPC).EPCcanberemarkablypersistent,withepilepticfocalrepetitive
jerkingactivitylastingdays,weeks,orevendecades[20,21].Causesincludeheterotopias,inflammatoryor
infectiouslesions(eg,tuberculosis,syphilis,andtoxoplasmosis),vascularlesions,neoplasms,congenital
malformations,andRasmussenencephalitis.Thejerkingisoftenrestrictedtoonepartofthebody,doesnot
spread,andisoftenslowerthaninmostotherformsoffocalmotorstatusepilepticus.

Rasmussenencephalitis,althoughrare,isarelativelycommoncauseofEPCthatoccursinchildrenand
adolescents.Itisusuallymanifestedbyrefractory,unremittingfocalseizures,progressivecerebral
hemiatrophywithcontralateralhemiparesis,andprogressivecognitivedysfunction.Itisoftenassociatedwith

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periodicepileptiformdischargesonEEG.(See"Localizationrelated(focal)epilepsy:Causesandclinical
features",sectionon'Hemisphericsyndromes'.)

HyperosmolarnonketotichyperglycemiacanalsocauseEPC.Althoughhyperglycemiaandfluidandelectrolyte
disturbancesprovideaprecipitantforseizures,thereisusuallyanunderlyingfocalcerebrallesionaswell[22].
Bloodglucoselevelscanbeextremelyhigh,oftenabove1000mg/dL.TheEPCassociatedwithnonketotic
hyperglycemiaisusuallyreadilytreatablebycorrectingthemetabolicabnormality[23].(See"Diabetic
ketoacidosisandhyperosmolarhyperglycemicstateinadults:Clinicalfeatures,evaluation,anddiagnosis".)

Otherformsoffocalstatusepilepticusthatdonotinvolvemotormanifestations,includingcomplexpartial
statusepilepticus,arediscussedseparately.(See"Nonconvulsivestatusepilepticus",sectionon
'Electroclinicalclassification'.)

MyoclonicstatusepilepticusMyoclonicstatusepilepticus(MSE)ischaracterizedbyfrequentmyoclonic
jerksthatcanberhythmicorarrhythmic.Myoclonicseizuresareoftengeneralized,butsomearefocal.The
EEGdemonstratesrapidepileptiformdischargestimelockedtothemovementsthatarepersuasiveforan
epilepticoriginofthemyoclonus.MSEhasaremarkablevarietyofcauses,oftendividedintoepilepsy
syndromerelatedcausesandsymptomaticcauses.Clinicalpresentationscanbeverysimilarfromone
syndrometothenext.Someoccurinbenignforms,andothershaveapoorprognosis.

PrimaryformsofepilepsysyndromerelatedMSEarethoseinwhichmyoclonusisacharacteristicfinding,
suchasinjuvenilemyoclonicepilepsy(JME)[24].SecondaryepilepsysyndromescausingMSEareusually
moresevere,butmyoclonusisnotsoprominentatbaseline.Thesesyndromesincludesuchconditionsas
LennoxGastautsyndrome,whereMSEmaybemixedwithotherformsofSE,suchasmyoclonicastatic
seizures.(See"Juvenilemyoclonicepilepsy"and"Epilepsysyndromesinchildren".)

Inothercases,MSEissymptomaticofmorewidespreadneurologicdysfunctionoranacuteencephalopathy.
ThemyoclonusmaybemoreirregularthaninotherMSE.Themostominouscauseisanoxia,butMSEmay
alsobecausedbyametabolicdisturbancesuchasuremicorhepaticencephalopathy,oranencephalopathy
causedbysepsisormultiplemedicalproblems,suchasacombinationofuremiaandsepsis(atypicalcause)
[25].EEGinsuchcasesshowsthe(oftensevere)encephalopathyandfrequent,brief,sometimesregular,
epileptiformdischarges.MSEduetotheseencephalopathiestypicallyhasapoorprognosis,determinedalmost
entirelybytheunderlyingmedicalillness.TheclinicalappearanceofallthesetypesofMSEmaybevery
similar,andEEGfeaturesareoftencrucialindistinguishingthedifferentetiologiesandtypesofMSE.(See
'Electroencephalography'below.)

Notallepisodesofpersistentmyoclonusareepilepticinoriginmanyarebetterlabeledasstatus
myoclonicusratherthantrulyepileptic.Theyincludeprolonged,continuous,butfrequentlynonrhythmic,
myoclonicjerking,usuallyoflargeamplitude,ofteninvolvingtheface,trunk,andlimbs,butsometimes
multifocalorasynchronous.Thecauseisusuallyanacute,severeencephalopathy,particularlyanoxia[25,26],
althoughmetabolicdisturbancesarealsocommon[25].Mostpatientsarecomatose[26].TheEEGtypically
showswidespreadslowingindicativeofanencephalopathy,andthemyoclonicjerksdonotcorrelatewith
spikesorsharpwaves.Theprognosisdependsonthatoftheunderlyingetiology[25].Followinganoxia,itis
usuallyfatalwithmetabolicencephalopathiesitisoftenreversible.

TonicstatusepilepticusTonicstatusepilepticus(TSE)israreinadults.Itconsistsofmaintenanceofa
tonicposture,particularlyofaxialmusculature,ratherthanfrankconvulsions[27].Itusuallyoccursinchildren
withmanydifferentseizuretypes,particularlythosewhohavemajorneurologicandcognitivedeficitsfrombirth
orearlychildhood,suchasLennoxGastautsyndrome.TheEEGmayshowwidespreadfastactivityorvery
rapidspikes,butmayalsoincludeperiodsofbackgroundsuppressionorattenuation.TSEcanbedifficultto
interruptwithantiseizuredrugs[28].BenzodiazepinesmayexacerbateTSEattimes.(See"Epilepsy
syndromesinchildren",sectionon'LennoxGastautsyndrome'.)

ELECTROENCEPHALOGRAPHYDuringgeneralizedconvulsivestatusepilepticus(GCSE),theEEGis
oftenobscuredbymuscleandmovementartifacts,butitmayshowcontinuousspikeandwaveactivity
indicativeofgeneralizedseizureactivity.InsomecasesofGCSE,afocalonsetisevidentonEEG,especially
earlyintheepisode,andthiscanhelptofocustheevaluationonanunderlyingfocalcause.Onceconvulsions
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haveceased,EEGiscrucialindeterminingwhetherstatusepilepticushastrulyended,orwhetherthereis
continuingseizureactivitywithoutconvulsions.

Inmanycasesoffocalmotorstatusepilepticus,EEGevidenceofseizureactivityissubtleorabsent[29].This
isgenerallythoughtduetoadeeperseizurefocusororientationoftheseizuredischargessuchthattheyare
notevidentonsurface,scalpEEGs.Theseizuresoffocalstatusepilepticuscanalsobeintermittentand
therebyabsentonashortEEGrecording.

CertainEEGpatternsinunresponsivepatientsarediagnosticofstatusepilepticus,includingpatternsthat
showtemporalevolution.Themeaningofotherpatterns,suchaslateralizedperiodicdischarges(LPDs
previouslyreferredtoasperiodiclateralizedepileptiformdischargesorPLEDS)(waveform1),remains
controversial,althoughaggressivepharmacologictreatmentinapatientwhoseEEGshowsonlyLPDswithout
evolutionandwhoseexaminationrevealsnoclinicalseizuresshouldgenerallybeavoided[30].Continuous
EEGrecordingsmaybehelpfuliftheinitialstudyisnotdiagnostic.(See"Nonconvulsivestatusepilepticus",
sectionon'Electroencephalography'.)

Inmyoclonicstatusepilepticus(MSE),EEGfeaturescanhelptodistinguishbetweenthedifferentetiologic
groups,inturnhelpingtodirectmanagement.Thegeneticgeneralizedmyoclonicepilepsysyndromesof
childhood,especiallythebenignsyndromes,oftenshowgeneralizedpolyspikesonarelativelynormal
background.Inthesecondarymyoclonicepilepsysyndromes,theremaybebackgroundslowingandless
rhythmicandbroaderspikes,sometimeswithperiodicdischarges.Caseswithacuteorremotesymptomatic
causesmayshowfocalslowingmanyshowmorewidespreadbackgroundslowing,indicativeofan
encephalopathy.

CaseswiththeparticularlyominousMSEduetoanoxiamayshowaverydisturbed,nearlyflatbackground
EEG,indicativeofanextremelysevereencephalopathyandpredictiveofapoorprognosis.InMSE,the
backgroundEEGrhythmgenerallycorrelatesbetterwithprognosisthandotheclinicalmanifestations.

NEUROIMAGINGAnoncontrastheadCTobtainedoncethepatientisstabilizedmayshowhyperdensity
consistentwithacutehemorrhageorfocalhypodensityindicativeofischemiaoranunderlyingmasslesion.
Magneticresonanceimaging(MRI)isthebesttesttoshowthestructurallesionsthatmaycauseorprecipitate
statusepilepticus.MRIisnotnecessarytodiagnosestatusepilepticus,however,andcannotbeperformed
untilapatientisstabilizedandseizureshavebeencontrolled.

Asidefromunderlyingstructurallesionsthatmayidentifytheunderlyingcauseofseizures,MRIcanshow
reversibleabnormalitiesrelatedtotheseizuresthemselves.Examplesincludeareasofincreasedsignal
intensityonfluidattenuatedinversionrecovery(FLAIR),T2,ordiffusionweightedimages,patchycontrast
enhancement,andincreasedbloodflowonperfusionweightedimaging(table2).Thesefindingsarethoughtto
representseizureinducedcellularedema.Theyaremostoftenseenincorticalandlimbicstructures,
particularlythehippocampusorotherdeepstructures.Manyarereversiblebutmaylastforweeksorlonger
[15],especiallyifseizuresareprolongedtheyultimatelyresolveorevolveintofocalatrophyandsclerosis.Itis
notalwayseasytotellthedifferencebetweenseizurerelatedfindingsandanunderlyingcause,andrepeat
imagingissometimesnecessary[31].(See"Magneticresonanceimagingchangesrelatedtoacuteseizure
activity",sectionon'Localperiictalmrifindings'.)

DIAGNOSISGeneralizedconvulsivestatusepilepticus(GCSE)isaclinicaldiagnosis,confirmedinmost
casesbythepresenceonexaminationofsustainedandrhythmicgeneralizedtonicandclonicmotoractivity
lastingforlongerthanfiveminutesorrepetitiveconvulsiveseizureswithoutareturntobaselineconsciousness
betweenseizures.AlthoughthediagnosisofGCSEisusuallyobvious,adetailedneurologicexaminationis
importantinmakingthediagnosisofmoresubtleorfocalformsofstatusepilepticus.(See'Clinicalfeatures'
above.)

Particularlyimportantareassessmentofthelevelofconsciousness,observationforautomaticmovementsor
myoclonus,andanyasymmetricfeaturesonexaminationthatmayindicateafocalstructurallesion.(See"The
detailedneurologicexaminationinadults"and"Stuporandcomainadults",sectionon'Neurologic
examination'.)

ClinicallyobviousstatusepilepticusshouldbetreatedimmediatelythereisnoneedtowaitforanEEG.An

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EEGiscriticalinthediagnosisofmoresubtleformsofstatusepilepticus,fordistinguishingmyoclonicstatus
epilepticusfromnonepilepticmyoclonus,andintheaftermathofgeneralizedconvulsivestatusepilepticusto
excludeongoingnonconvulsiveseizures.(See'Electroencephalography'aboveand"Convulsivestatus
epilepticusinadults:Treatmentandprognosis".)

DIFFERENTIALDIAGNOSISFewotherconditionsappearsimilartogeneralizedconvulsivestatus
epilepticus(GCSE).Somemovementdisordersoccasionallymimicstatusepilepticus.AnEEGatthetimeof
themovementsandexaminationbyanexperiencedclinicianwilldifferentiatethemfromstatusepilepticus.
Manyotherconditions,mostlyvariouscausesofencephalopathies,maybehardtodistinguishfrom
nonconvulsivestatusepilepticus.AnEEGwillusuallymakethediagnosis,butsomepatternsare
controversial.(See"Nonconvulsivestatusepilepticus",sectionon'Electroencephalography'.)

Althoughrelativelyuncommon,psychogenicstatusepilepticusshouldbeconsideredinsituationswherethere
arebilateralmotormovementswithpreservedconsciousness.Althoughsomefrontalseizurescanappearthis
way,GCSEdoesnot.VideoandEEGmonitoringarethebestwaytoestablishthecorrectdiagnosis.Notall
seizuresshowuponanEEG,butprolongedepisodesofimpairedawarenessorbehaviorwithoutanychanges
onEEGareunlikelytorepresenttruestatusepilepticus(rememberingthattheEEGoftenremainsnormal
duringsimplepartialstatusepilepticusaswell).Psychogenicstatusepilepticusisimportanttorecognize,
becauseiatrogenicinjurycanoccurthroughovertreatmentwithbenzodiazepines.Psychogenicnonepileptic
seizuresandpsychogenicstatusepilepticusarediscussedseparately.(See"Psychogenicnonepileptic
seizures".)

INFORMATIONFORPATIENTSUpToDateofferstwotypesofpatienteducationmaterials,"TheBasics"
and"BeyondtheBasics."TheBasicspatienteducationpiecesarewritteninplainlanguage,atthe5thto6th
gradereadinglevel,andtheyanswerthefourorfivekeyquestionsapatientmighthaveaboutagiven
condition.Thesearticlesarebestforpatientswhowantageneraloverviewandwhoprefershort,easytoread
materials.BeyondtheBasicspatienteducationpiecesarelonger,moresophisticated,andmoredetailed.
Thesearticlesarewrittenatthe10thto12thgradereadinglevelandarebestforpatientswhowantindepth
informationandarecomfortablewithsomemedicaljargon.

Herearethepatienteducationarticlesthatarerelevanttothistopic.Weencourageyoutoprintoremailthese
topicstoyourpatients.(Youcanalsolocatepatienteducationarticlesonavarietyofsubjectsbysearchingon
"patientinfo"andthekeyword(s)ofinterest.)

Basicstopic(see"Patientinformation:Epilepsyinadults(TheBasics)")

SUMMARYANDRECOMMENDATIONS

Generalizedconvulsivestatusepilepticus(GCSE)isoperationallydefinedas5minutesofcontinuous
seizureactivity,ormorethanoneseizurewithoutrecoveryinbetween.Thisestablishesthecriticalpoint
atwhichtreatmentmustbeinitiatedinordertoavoidseriousmorbidity.(See'Definition'above.)

Theincidenceofstatusepilepticusfollowsabimodalagedistribution,withpeakratesinchildrenless
than1yearofageandadultsovertheageof60years.(See'Epidemiology'above.)

Etiologiesincludeacutebraininjuryorinfection,noncompliancewithantiseizuredrugtreatment,drugor
alcoholwithdrawalsyndromes,andmetabolicdisturbances,amongothers.(See'Etiology'above.)

Classifyingthetypeofstatusepilepticusisimportantbecauseitisamajorfactorindeterminingmorbidity
and,therefore,theaggressivenessoftreatmentrequired.Thefourmajorformsofconvulsivestatus
epilepticusaregeneralizedconvulsive,focalmotor,myoclonic,andtonic.Variousformsofnonconvulsive
statusepilepticusarediscussedseparately(See'Classification'aboveand"Nonconvulsivestatus
epilepticus".)

Patientswithconvulsivestatusepilepticuspresentwithcharacteristicmotormanifestationsthatvary
accordingtotheseizuretype.Whilepatientswithgeneralizedconvulsivestatushaveobviousbilateral
tonicfollowedbyclonicmotoractivityandlossofconsciousness,patientswithfocalmotorstatusmay
havemotorjerkinglimitedtoonelimbandretainedawareness.(See'Clinicalfeatures'above.)

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GCSEisaclinicaldiagnosis,confirmedinmostcasesbythepresenceonexamofsustainedand
rhythmicgeneralizedorfocaltonicandclonicmotoractivitylastingfor5minutes.
Electroencephalography(EEG)isnecessaryintheaftermathofGCSEtoexcludeongoingnonconvulsive
seizuresandismandatoryformanagingprolongedandrefractorystatusepilepticus.(See'Diagnosis'
aboveand'Electroencephalography'above.)

ACKNOWLEDGMENTTheeditorialteamatUpToDate,Inc.wouldliketoacknowledgeDr.MarkStecker,
whocontributedtoanearlierversionofthistopicreview.

UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.

REFERENCES

1.Guidelinesforepidemiologicstudiesonepilepsy.CommissiononEpidemiologyandPrognosis,
InternationalLeagueAgainstEpilepsy.Epilepsia199334:592.
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