癫痫分类新概念杭州市儿童医院神经内科李光乾ILAE癫痫分类方案的发展癫痫发作和癫痫综合征的诊断建议方案2001修订的癫痫和癫痫综合征分类1989癫痫和癫痫综合征分类1985修订的癫痫发作临床和脑电图分类1981癫痫发作临床和脑电图分类19702010新报告癫痫分类方法:Eletroclinicalsyndromes电临床综合征Clinicoradiologicalsyndromes临床放射综合征Aetiology病因AetiologyClinicoradiologicalsyndromesEletroclinicalsyndromesILAE2010报告全面性发作1.强直阵挛性(以任何形式的组合)2.失神①典型失神②不典型的失神③伴特殊表现的失神:肌阵挛性失神发作/眼睑肌阵挛3.肌阵挛①肌阵挛②肌阵挛失张力③肌阵挛强直4.阵挛5.强直6.失张力局灶性发作不确定的发作癫痫性痉挛表1:Classificationofseizures根据发作时意识损伤程度描述局灶性发作无意识或知觉损伤伴有可见运动或自主神经成分,大致相当于“简单部分性发作”的概念“局灶性运动”和“自主神经”术语能够根据发作表现恰当的表达这个概念仅有主观的感觉或精神症状,相当于“先兆”,来自2001年词汇表有意识或知觉损伤大致相当于“复杂部分性发作”的概念演变为双侧的惊厥性发作包括强直、阵挛或强直和阵挛成分。代替“继发性全面性发作“一词描述:腹部先兆自动运动发作(AOC)全面强直-阵挛发作癫痫性痉挛(不能明确的发作)*癫痫性痉挛取代原来的痉挛;*其发作可出现在婴儿期,也可在婴儿期过后新发;*痉挛发作既有局灶性病变背景,也可有明显的全面性特征;ILAERevisedTerminologyforOrganizationofSeizuresandEpilepsies2010ElectroclinicalSyndromesandOtherEpilepsiesElectroclinicalsyndromesarrangedbyageatonset*Neonatalperiod-Benignfamilialneontalepilepsy(BFNE)-EarlyMyoclonicencephalopathy(EME)-OhtaharasyndromeInfancy-Epilepsyofinfancywithmigratingfocalseizures-Westsyndrome-Myoclonicepilepsyininfancy(MEI)-Benigninfantileepilepsy-Benignfamilialinfantileepilepsy-Dravetsydrome-MyoclonicencephalopathyinnonprogressivedisordersChildhood-Febrileseizuresplus(FS+)(canstartininfancy)-Earlyonsetchildhoodoccipitalepilepsy(Panayiotopoulossyndrome)-Epilepsywithmyoclonicatonic(previouslyastatic)seizures-Childhoodabsenceepilepsy(CAE)-Benignepilepsywithcentrotemporalspikes(BECTS)-Autosomaldominantnocturnalfrontallobeepilepsy(ADNFLE)-Lateonsetchildhoodoccipitalepilepsy(Gastauttype)-Epilepsywithmyoclonicabsences-Lennox-Gastautsyndrome-Epilepticencephalopahtywithcontinuousspike-and-waveduringsleep(CSWS)^-Landau-Kleffnersyndrome(LKS)Adolescence–Adult-Juvenileabsenceepilepsy(JAE)-Juvenilemyoclonicepilepsy(JME)-Epilepsywithgeneralizedtonic-clonicseizuresalone-Progressivemyoclonusepilepsies(PME)-Autosomaldominantepilepsywithauditoryfeatures(ADEAF)-OtherfamilialtemporallobeepilepsiesLessspecificagerelationship-Familialfocalepilepsywithvariablefoci(childhoodtoadult)-Reflexepilepsies*Thearrangementofeletroclinicalsyndromesdoesnotreflectetiology^SometimesreferredtoasElectricalStatusEpilepticusduringSlowSleep(ESES)ILAE2010报告表2:电-临床综合征和其他癫痫病根据起病年龄排列的电临床综合征(不反映病因)新生儿良性家族性新生儿惊厥(BFNE)早期肌阵挛脑病(EME)大田原综合征婴儿期伴游走性局灶性发作的婴儿癫痫West综合征婴儿肌阵挛性癫痫(MEI)良性婴儿癫痫良性家族性婴儿癫痫Dravet综合征非进行性疾病中肌阵挛脑病儿童期热性惊厥附加症(FS+)(可以起病于婴儿期)Panayiotopoulos综合征肌阵挛失张(以前称站立不能性)癫痫伴中央颞区棘波的良性癫痫(BECT)常染色体显性遗传夜间额叶癫痫(ADNFLE)晚发性儿童枕叶癫痫(Gastaut型)肌阵挛失神癫痫Lennox-Gastaut综合征伴睡眠期持续棘慢波的癫痫性脑病(CSWS)*Landau-Kleffner综合征儿童失神癫痫(CAE)青少年—成年期:青少年失神癫痫(JAE)青少年肌阵挛癫痫(JME)仅有全...