Wakamoto H, Nagao H, Manabe K, Kobayashi H, Hayashi M
Department of Pediatrics, Uwajima City Hospital, Ehime, Japan.
Neuropediatrics. 1999 Jun;30(3):149-50. doi: 10.1055/s-2007-973481.
A 10-year old girl with eyelid myoclonia with absences (EMA) in whom nonconvulsive status epilepticus developed shortly after awakening is described. A video-polygraphic recording during the status showed the characteristic eye-closure provocation of eyelid myoclonia with upward deviation of the eyeballs and brief absences. Ictal EEG showed generalized polyspikes concomitant with eyelid myoclonia, while absences were accompanied by 3.5 Hz polyspike-wave complexes on EEG. This condition occurred even in total darkness as well as even after seizures precipitated by bright sunlight had been eliminated by medication. The present case suggests that the eye closure mechanism could be a more potent precipitating factor than photosensitivity in the pathophysiology of EMA.
本文描述了一名10岁患有眼睑肌阵挛伴失神发作（EMA）的女孩，她在醒来后不久就发展为非惊厥性癫痫持续状态。癫痫持续状态期间的视频多导记录显示，典型的闭眼诱发眼睑肌阵挛，伴有眼球向上偏斜和短暂失神。发作期脑电图显示，眼睑肌阵挛伴有广泛性多棘波，而失神发作时脑电图伴有3.5Hz多棘慢波综合波。这种情况即使在完全黑暗中也会发生，甚至在药物消除了强光诱发的癫痫发作后依然出现。本病例表明，在EMA的病理生理学中，闭眼机制可能是比光敏性更强的诱发因素。
