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1、分析癫痫伴觉醒期大发作的临床表现ClinicalmanifestationsofanalysisofepilepsywithgrandmalAwakeningGeneralizedtonic-clonicseizures(GTCS),alsoknownasgrandmal(GM),canbeseeninavarietyofepilepsysyndromecausedbyavarietyofcauses,bothidiopathicgeneralizedseizures,partialseizuresmayalsobesecondaryGTCSmain
2、lytootherseizuretypes,GTCSoftendoesnothaveaspec讦ic,notthemainbasisforthediagnosisofepilepsysyndrometype・CurrentlyonlyoneintheonsetofadolescencetoattackmainlyappearsoonafterawakeningGTCShasbeenidentifiedasanidiopathicgeneralizedepilepsysyndrome,epilepsywithgrandmalAwakening(EGMA
3、).全身强直一阵挛性发作(GTCS)又称大发作(GM),可见于由各种病因引起的多种癫痫综合征,既可为特发性全身性发作,也可由局部性发作继发GTCS,因此当以其他发作类型为主时,GTCS往往不具有特异性,不是诊断癫痫综合征类型的主要依据。目前只有一种在青少年期发病,发作主要出现在觉醒后不久的GTCS被确定为一种特发性的全身性癫痫综合征,即癫痫伴觉醒期大发作(EGMA)oEGMAidiopathicepilepsysyndrome・Occurredduringsleep,wakestateandtheawakeningoftheGTCSresearchs
4、howsthatthemajorityoftheAwakeningGTCSetiologycanbefound.EGMApatientswithfamilialfebrileseizuresorepilepsyoccursinsleepandawakebigattackpatientswassigrdficantlyhigherwiththechildrenandjuvenileabseneeepilepsy,juvenilemyoclonicepilepsyandotheridiopathicgeneralizedepilepsy,suggesti
5、ngthattheincidenceofthisdiseasewithgeneticsusceptibilitytoconvulsions.EGMA为特发性癫痫综合征。对发生在睡眠中、醒觉状态和觉醒期的GTCS的研究显示,觉醒期的GTCS大多数无病因可寻。EGMA患者家族屮有热性惊厥或癫痫者比发牛在睡眠中和清醒状态的大发作患者明显增高,而与儿童及少年失神性癫痫、少年肌阵挛性癫痫等其他特发性全身性癫痫相似,提示本症的发病与遗传性惊厥易感性有关。Inadditiontotheinherentgeneticpredisposition,someextern
6、alfactorsintheonsetofEGMAinducedorfacilitated・Themostimportantpredisposingfactorforthelackofsleep,suchasdeprivationofsleeporchroniclackofsleep.Polysomnographystudieshaveshownthat,thepatientssleepEGMAespeciallyunstableandvulnerabletoexternalfactors.Inaddition,lightstimulation,fa
7、tigue,alcoholismisalsolikelytocauseseizures・除内在的遗传易感性外,一些外在的因素对EGMA的发作具有诱发或易化作用。最重要的诱发因素为睡眠缺乏,如剥夺睡眠或慢性睡眠不足。多导睡眠图研究显示,EGMA患者的睡眠特别不稳定,易受外界因素的影响。此外,光刺激、疲劳、酗酒等也容易诱发发作。Clinicalmanifestations【临床表现】Theboymorecommon(62.2%to65.6%).Onsetagerangeof6to35yearsold,themajorityofonsetat10to20y
8、earsold,withanaverageof17.1yearsold.Idiopathicagesever