de Saint-Martin A, Badinand N, Picard F, Fischbach M, Challamel M J, Marescaux C, Hirsch E
Service de Pédiatrie I, Hôpitaux Universitaires de Strasbourg.
Rev Neurol (Paris). 1997 May;153(4):262-7.
Paroxysmal dyskinesias are intermittent attacks of involuntary hyperkinetics abnormal movements. Among paroxysmal dyskinesias were individualized three entities: paroxysmal kinesigenic choreoathetosis, paroxysmal choreoathetosis of Mount and Reback, hypnogenic paroxysmal dystonia. New classifications are based upon the circumstances of occurrence, the duration of attacks and their etiology. We report here two observations of idiopathic non familial paroxysmal dyskinesias in three-year-old children. Both were seen first in consultation for falls and nocturnal motor agitation. The attacks were paroxysmal jerky "puppet-like" movements lasting from 20 seconds to 15 minutes. They could occur during non REM sleep, during the day, at rest, after a sudden movement, or during prolonged exercise. Carbamazepine was inefficient. These cases were not classifiable according to the classical criteria and could constitute a new entity. Moreover, some sleep-EEG showed abnormal patterns (frontal rapid rhythms, central spikes in one case) and led us to discuss the pathophysiology of this episodic movements disorder, and its relation with frontal partial epilepsy.
发作性运动障碍是一种非自愿性运动亢进异常的间歇性发作。在发作性运动障碍中，可分为三种类型：发作性运动诱发性舞蹈手足徐动症、Mount和Reback型发作性舞蹈手足徐动症、睡眠性发作性肌张力障碍。新的分类基于发作情况、发作持续时间及其病因。我们在此报告两例3岁儿童特发性非家族性发作性运动障碍的病例。两人最初都是因跌倒和夜间运动性激惹前来就诊。发作表现为阵发性急促的“木偶样”运动，持续20秒至15分钟。发作可发生在非快速眼动睡眠期、白天、休息时、突然运动后或长时间运动期间。卡马西平治疗无效。这些病例不符合经典标准，可能构成一种新的类型。此外，一些睡眠脑电图显示异常模式（一例为额部快速节律，另一例为中央棘波），这促使我们讨论这种发作性运动障碍的病理生理学及其与额叶部分性癫痫的关系。
