进行性肌阵挛性癫痫(以下简称为PME),是一种少见的家族遗传性疾病。国内报导甚少,共有7例报告。现将我们在临床工作中遇到的2例报告如下: 例 1 男,21岁,1958年1月3日入院。14岁时每于做精细动作双手跳动。1～2年后加重,渐双下肢及头颈部也出现闪动性抽动,每日3～4次至30～40次不等,每次历时几秒钟。常因为精神紧张、噪音及亮光诱发。19岁以后曾有3次出现发作性意识障碍,伴四肢抽动,口吐白沫,双眼上翻,每次历时4～5分钟。发作前肌肉跳动增加。曾服鲁米那、苯妥英钠均不能满意控制肌肉跳动。 Progressive myoclonic epilepsy (hereinafter referred to as PME), is a rare family genetic disease. Very few domestic reports, a total of 7 cases report. Now we encountered in clinical work in 2 cases reported as follows: Example 1 male, 21 years old, January 3, 1958 admitted. At the age of 14, she beats both hands for her delicate movements. 1 to 2 years after the increase, gradually lower extremities and head and neck also flashing twitching, 3 to 4 times a day to 30 to 40 times, each time a few seconds. Often because of mental stress, noise and light induced. After 19 years of age there have been 3 episodes of episodic disturbance of consciousness, accompanied by extremities twitching, foaming at the mouth, his eyes upside down, each time 4 to 5 minutes. Before the onset of muscle beating increased. Once served luminal, phenytoin sodium are not satisfied with the control of muscle beating.