癲癇持續(xù)狀態(tài)nicu課件

Statusepilepticus

SE

首都醫(yī)科大學宣武醫(yī)院神經(jīng)內(nèi)科N-ICU宿英英BackgroundStatusepilepticus(SE)isacommonlife-threateningneurologicaldisorder.Itisessentiallyanacute,prolongedepilepticcrisis.ThefirstdescriptionofSEinthemedicalliteraturewasaBabyloniantextfromthefirstmillenniumBC.

“Ifanepilepsydemonfallsmanytimesuponhimonagivenday,heseventimespunisheshimandpossesseshim,hislifewillbespared.Ifhefallsuponhimeighttimes,hislifemaynotbespared.“（WilsonandReynolds,1990）

Definition

AusefulworkingdefinitionwasformulatedbytheEpilepsyFoundationofAmerica'sworkinggrouponSE.

“Morethan30minutesofcontinuousseizureactivityortwoormoresequentialseizureswithoutfullrecoveryofconsciousnessbetweenseizures.”（Dodsonetal,1993

）Frequency

IntheUSExtrapolatingfromapopulation-basedstudyinRichmond,estimatedthat50,000-200,000ofstatusepilepticus

casesoccurannuallyintheUnitedStates.（DeLorenzoetal1996）

Mortality

Inthe1998VeteransAdministration（VA）study,theSECooperativeStudyGroupquantitatedmortalityrateat27%forovertgeneralizedconvulsiveSEand65%forsubtlegeneralizedconvulsiveSE.DeLorenzoetal（1995）reportedamortalityrateof21%inpatientswithgeneralizedSE,definingmortalityasdeathoccurringwithin30days.AicardiandChevrie（1970）studied239childrenwithgeneralizedconvulsiveSEthatlastedmorethananhour.11%diedand37%sufferedpermanentneurologicaldamage.MortalityThemortalityratesofSEhavedecreasedoverthepast60yearswhichprobablyisrelatedtofasterdiagnosisandmoreaggressivetreatment.deathcauseddirectlybySEoccursinnomorethan2%ofcases.BothseveresystemicdiseaseandanacuteCNSinsultassociatedwiththeSEpredictapooroutcome.（Hauser1990）

MortalityInprospectivepopulation-basedstudies（DeLorenzoetal）mortalityratefortheentirepopulationwas22%

inyoungadults,itwas13%

perinatalhypoxicinsultsormetabolicdisordersintheelderly,38%inthoseolderthan80years,greaterthan50%hypoxicorischemiccentralnervoussystem（CNS）insultsSystemicchanges

systemicarterialpressuredecreasestolevelsthatarebelowbaseline.

（1961）

MarkedacidosisoccurswithSE（1980

）33%patientshadapHoflessthan

7.0

（AminoffandSimon,1980）acidosisdoesnotcorrelatewiththedegreeofneuronalinjuryacidosisisknowntobeananticonvulsantsystemicchangesHyperthermiamotoractivity.

centralsympatheticdrive.83%patients

hadhyperthermiawithtemperaturesreaching42℃.（AminoffandSimon,1980）poorneurologicaloutcomeandshouldbetreatedaggressively.Marginatingleukocytesarecommon.withoutevidenceofinfectionhadWBCelevationsfrom12,700-28,800.Bandsshouldnotbeseen.Cerebrospinalfluid（CSF）pleocytosisiscommonbutthecellcountelevationsareusuallymodest.Only4of65patientshadgreaterthan30cellsintheCSF

（AminoffandSimon,1980）.

GCSE

ClinicalandEEG（２）

Iftheconditionisnottreatedoristreatedinadequately,theSEpersistsandthemotormanifestationsbecomelessandlessdramatic.Eventually,onlysubtlemovementssuchasnystagmoidjerksoftheeyesortwitchingoftheshouldermaybeseen（ie,subtlestatus）.IfSEcontinues,allmotoractivitymaystop,althoughelectrographicseizurespersist（ie,electricalgeneralizedconvulsiveSE）.GCSE

ClinicalandEEG（3）Theparadoxicalevolutionofapparentclinicalimprovementisimportanttounderstand.Theclinicianunfamiliarwiththisphenomenonmaystoptreatmentbecauseoftheapparentimprovement.Treatmentshouldbecontinueduntiltheelectrographicseizureactivityhasresolvedcompletely.Insomepatients,theunderlyingencephalopathicinsultissoseverethatonlyafew(orno)generalizedconvulsionsoccurbeforesubtleconvulsiveactivitydevelops.Finally,asthepatientevolvesfromgeneralizedtonic-clonicstatusintosubtleandthenelectricalgeneralizedtonic-clonicSE,themanifestationsbecomelessintermittentandmorecontinuous.PsychogenicseizuresPsychogenicseizuresmay,attimes,beindistinguishablefromGCSEbyappearancealone.unresponsivenesswithoutmovementwasthemostcommonpresentation.asynchronousextremitymovement,forwardpelvicthrusting,andgeotropiceyemovementstheeyesdeviatingtowardthegroundinanonphysiologicmannerwhethertheheadisturnedleftorright.MyoclonusRepetitivemyoclonusinacomatosepatientfollowingdiffusehypoxicbraininjurymaysimulategeneralizedseizures.Thephysiologicoriginofthemyoclonicjerksmaynotbecortical.Themyoclonususuallyislimitedindurationtoseveralhours.Causes（1groups

）

Inroughlyonethirdofcases,anexacerbationofanidiopathicseizuredisorderisthoughttobethecause.（thisisadiagnosisofexclusion）Inpeoplewithknownepilepsy,themostcommoncauseisachangeinmedicationthechangemaybeeitherunderthedirectionofaphysicianorduetononcompliance.NoncomplianceisthemostcommoncauseofSEinpatientswithknownepilepsy.Causes（3）

Amyriadofotherconditionsmayprecipitatestatusepilepticus,includingtoxicormetaboliccausesandanythingthatmightcausecorticalstructuraldamage.Stroke(remoteoracute)HypoxicinjuryTumorSubarachnoidhemorrhageTraumaToxicologic(eg,cocaine,theophylline,isoniazid,alcoholwithdrawal)Electrolyteabnormalities(eg,hyponatremia,hypernatremia,hypercalcemia,hepaticencephalopathy)Infectiousetiology(eg,meningitis,brainabscess,encephalitis)CausesInchildrenyoungerthan16years,themostcommoncausewasfever/infection（36%）;incontrast,thisaccountedforonly5%inadults

（DeLorenzoetal,1995）.Thesamestudyrevealedthatthemostcommonprecipitantforadultswascerebrovasculardisease（25%）,whereasthisfactorcausedonly3%ofpediatriccases.Inamorerefinedstudythatfocusedonchildrenfoundthatmorethan80%ofchildrenyoungerthan2yearshadSEoffebrileoracutesymptomaticorigin,whereascryptogenicandremotesymptomaticcausesweremorecommoninolderchildren（Shinnaretal，1997）

Laboratoryelectrolytestoxicologyscreeningglucoseanticonvulsantlevelscompletebloodcount(CBC)BloodculturesrenalfunctiontestsurinalysisarterialbloodgaseslumbarpunctureImaging

BrainCT/MRIisoftenhelpfultoevaluateforastructurallesionunderlyingSEbraintumor,infarction,abscess,hemorrhage.

Aneuroimagingstudyshouldnotbeallowedtoimpedetherapidandaggressivetreatmentofthedisorder.TreatmentPrehospitalCareSupportivecare,includingABCsHistoriccluesmaybeevidenttoEMSproviders.anticonvulsantsintravenously(IV)orperrectum(PR)diazepam(Valium)

TreatmentEmergencyDepartmentCarepromptterminationofelectricalseizureactivityarethegoals.nasopharyngealairwayplacementforsomepatientsendotrachealintubationforsomepatientsshort-actingneuromuscularparalysisEEGmonitoringInitiaterapidglucosedeterminationandcorrection.EstablishIVaccess,ideallyinalargeveinanticonvulsantmedicationEstablishcardiacandotherhemodynamicmonitoring.TreatmentNoprospective,double-blindstudiesontheofSEhavebeenpublishedrecently.Therefore,thechoiceofthebestinitialdrugtreatmentremainsuncertain.Treimanetal（1998）publishedatreatmentcomparisonforgeneralizedconvulsiveSE,investigatingtheuseofdiazepamfollowedbyphenytoin,lorazepam,phenobarbital.

Anticonvulsantagents

StartanIVline,administer50ccof50%dextroseIVpush,thenstarttheanticonvulsant.Administerdiazepamorlorazepam0.15mg/kgIVover5minutes,followedbyfosphenytoin15-20mgphenytoinequivalents(PE)/kgatamaximumrateof150mg/minorless.IntubateifnecessaryandcontrolhyperthermiaIfseizurescontinueafter20minutes,giveanadditional10mgPE/kgoffosphenytoin.Ifseizurescontinueafter20minutes,giveanadditional10mgPE/kgoffosphenytoin.Ifseizurescontinue,administergeneralanesthesia.GCSE

系統(tǒng)性病理生理兒茶酚胺大量釋放，血壓增高、心率加快、心律失常、血糖升高。GCSE持續(xù)發(fā)作不止，最終血壓下降，心率下降，心功能失代償機械性呼吸運動停止、急性肺水腫、抗癲癇藥物的呼吸中樞抑制作用，呼吸功能障礙，呼吸性酸中毒（有人認為酸中毒可結(jié)束癲癇發(fā)作，因此，當PH值下降時，無需加以糾正）肌肉強烈收縮，乳酸鹽增多，代謝性酸中毒，體溫過高體溫中樞受累，體溫＞41℃，神經(jīng)細胞繼發(fā)損傷多數(shù)癲癇發(fā)作自行終止，終止原因不清；少數(shù)自行終止失敗，原因也不清。Generalizedconvulsivestatusepilepticus陣發(fā)性或持續(xù)性肌肉節(jié)律性強直、陣攣、強直-陣攣意識障礙，發(fā)作間期意識障礙不恢復GCSE發(fā)作3個階段顯而易見的GCSE，肌肉節(jié)律性強直、陣攣、強直-陣攣微小而不意識別的GCSE，肌肉運動越來越小，甚至變得十分微細而不易被發(fā)現(xiàn)，如眼皮的眨動或肩的肌肉顫動（twitch）肌肉運動停止的電GCSE，異常電活動仍然繼續(xù)伴隨外傷，包括舌咬傷，肩關節(jié)脫位，頭顱外傷和面部創(chuàng)傷GCSE未予治療或治療不充分，癲癇發(fā)作繼續(xù)AbsencestatusepilepticusEEG雙側(cè)同步節(jié)律性3Hz棘慢復合波或彌散棘波臨床表現(xiàn)與EEG變化不同步，持續(xù)時間數(shù)min~數(shù)d突發(fā)意識障礙程度輕，嗜睡或意識混濁自主運動減少，語言緩慢伴或不伴其他臨床征象，如定向力障礙，記憶力障礙（癲癇性失記憶），復雜的自動癥或精神癥狀Complexpartialstatusepilepticus意識障礙程度和EEG異常電活動多樣性、周期性、長久性意識障礙從朦朧狀態(tài)到完全無反應EEG從一側(cè)性癲癇樣發(fā)放（PLED）到彌漫性棘慢復合波節(jié)律異常電發(fā)放的起源通常在顳葉伴隨癥狀與ASE相似，但更豐富Simplepartialstatusepilepticus意識基本正常感覺異常發(fā)作：軀體感覺的、視覺的、聽覺的、嗅覺的、味覺的運動異常發(fā)作：軀體運動、眼球陣攣、軟腭震顫、語言障礙或失語發(fā)作自主神經(jīng)異常發(fā)作和其它奇異發(fā)作少見

EEG異常電活動（如3Hz棘慢復合波）具有周期性、局限性、不擴展性，異常電活動的部位與臨床感覺或運動異常一致，有時大腦皮層病變非常局限而EEG無異常發(fā)現(xiàn)診斷與鑒別診斷診斷內(nèi)容癲癇的識別癲癇持續(xù)狀態(tài)的確認癲癇持續(xù)狀態(tài)類型的區(qū)分癲癇持續(xù)狀態(tài)病因的確定實驗室、影像學、或其它輔助檢查幫助尋找病因和判斷繼發(fā)損害治療原則積極支持性醫(yī)療與護理無論是容易判斷的SE，如肢體肌肉連續(xù)大幅度抽動；還是難以識別的SE，如非驚厥性或微小的肌肉顫動；迅速終止癲癇發(fā)作“Seizuresbegetseizures”已經(jīng)成為公認的警句。癲癇發(fā)作4min~5min不停止，應迅速給予antiepilepticdrug（AED）終止發(fā)作。支持性醫(yī)療與護理呼吸支持開放通氣道，鼻咽通氣道、氣管插管、機械通氣建立大靜脈通道矯正異常生理生化指標降溫昏迷與氣道護理用藥原則首