进行性肌阵挛性癫痫(PME)是一种少见病。本院1986年发现1例经脑、肌活检证实为本病,兹报道如下: 患者 女,16岁。因阵发性头昏、手足抽搐伴进行性智力下降1年半,发作性全身抽搐、意识不清1年入院。1年半前起阵发性头昏,眼前发黑,手足抽动。每次持续约数秒至1分钟,10余天发作1次,发作时神清。以后逐渐出现无故发笑,行走不稳。学习成绩下降。半年后出现发作性意识丧失,全身强直性抽搐、口吐白沫、双眼上翻及小便失禁。发作次数逐渐增多并间歇性出现手足不自主抽动,说话吐词欠清。上述发作常因情绪激动及光线刺激而诱发。入院前1 Progressive myoclonic epilepsy (PME) is a rare disease. Our hospital in 1986 found that 1 case of brain, muscle biopsy confirmed the disease, it is reported as follows: Female patient, 16 years old. Due to paroxysmal dizziness, hand, foot and twitch with progressive intelligence decreased by 1.5 years, episodes of generalized convulsions, unconsciousness 1 year admission. One and a half years ago, paroxysmal dizziness, black eyes, hands and feet twitching. Each lasted for a few seconds to 1 minute, more than 10 days attack 1, clear seizures. After gradual emergence of no reason to laugh, walking unstable. declining grade. Six months after onset of episodic loss of consciousness, tonic twitching, foaming at the mouth, eyes upturned and incontinence. The number of seizures gradually increased and intermittent hand-foot involuntary twitch, voicing words. The seizures often induced by emotional and light stimulation. Before admission