癫痫脑病—医学课件

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1、癫痫性脑病癫痫性脑病IntroductionDefinitionExplanationExamplesConcludingRemarks癫痫性脑病EpilepticEncephalopathy脑病：Encephalo-pathy癫痫性脑病：癫痫Epileptic+脑病癫痫所致（相关）的脑病X脑病所致（相关）的癫痫ILAE2001ILAE2001EpilepticEncephalopathyAconditioninwhichtheepileptiformabnormalitiesthemselvesarebelievedtocontributetotheprogr

2、essivedisturbanceincerebralfunction.脑功能障碍，进展性癫痫性异常（放电本身）所致ILAE2001SummaryofkeyrecommendationsfromtheCommissiononClassificationandTerminology–ForDiscussionattheICEinBudapestILAE2009Embodiesthenotionthatepilepticactivityleadtoseverecognitiveandbehavioralimpairmentaboveandbeyondwhatfrom

3、theunderlyingpathology(e.g.corticalmalformation)alone（癫痫样活动本身的意义，而不是基础病因）seizuresthemselvescontributetothecognitiveimpairments（发作本身的意义）Althoughmayoccuratanyage,mostcommonandsevereinthedevelopingbrain（发育期）EpilepticEncephalopathyInherentinthisconceptistheideathatbysuppressingorpreventi

4、ngtheepilepticactivity,onemayimprovethecognitiveandbehavioraloutlookofthedisorder.（干预的意义）Inthedevelopingbrain,rapideffectiveinterventionshouldandcanbeusedbeforetheabnormalepilepticactivityinterferesirrevocablywithnormalprocessesofbraindevelopment.（快速有效的干预）Thebestknownandmostcommonoft

5、heseareWest,LGS,Dravet,LKS-CSWS,andDoosesyndromes.（包括多个综合征-有待更多研究）EpilepticEncephalopathy癫痫性脑病DefinitionExplanation癫痫性脑病具有相对独特而复杂的临床脑电特征精神行为认知损伤癫痫样活动本身所致独立于基础脑疾病包括一组预后不良的综合征West,LGS,Dravet,LKS-CSWS,andDoose,etc.及时干预可能改善认知的转归多见于发育时期癫痫性脑病IntroductiontoEpilepticEncephalopathyExamples:Ep

6、ilepticEncephalopathiesOhtaharaSyndromeEarlymyoclonicencephalopathy（EME）DravetSyndromeLennox-GastautSyndromeConcludingRemarks癫痫和癫痫综合征分类（简化，ILAE2001）将各种癫痫综合征分成8组婴儿和儿童特发性局灶性癫痫(4)家族性（常染色体显性遗传）局灶性癫痫(5)症状性（或可能为症状性）局灶性癫痫(5+)特发性全面性癫痫(8+)反射性癫痫(3+)癫痫性脑病(8)进行性肌阵挛癫痫可不诊断为癫痫的发作(8)癫痫性脑病早期肌阵挛脑病(Ear

7、lymyoclonicencephalopathy,EME)大田原（Ohtahara）综合征West综合征Dravet综合征(severemyoclonicepilepsyininfancy,SMEI)非进行性脑病中的肌阵挛持续状态（Myoclonicstatusinnonprogressiveencephalopathies)Lennox-Gastaut综合征(LGS)Landau-Kleffner综合征(LKS)慢波睡眠中持续棘慢波的癫痫（Epilepsywithcontinuousspike–wavesduringslow-wavesleep）ILAE20

8、01Ohtahara综合