阵发性运动源性舞蹈手足徐动症4例报道

Paroxysmal Kinesigenic Choreoathetosis a Report of 4 Cases

阵发性运动源性舞蹈手足徐动症(PKCA)是一种罕见的非进展性不自主运动疾病。发作时常表现为痉挛样、肌紧张异常样或舞蹈手足徐动样,而且常由突然运动或惊跳所诱发。发病年龄以儿童期为多见。本文报道4例PKCA,2例有家族史而另2例为散发者,4例均由突然运动如由坐位起立、跑步等所诱发。发作间歇期神经系统检查,实验室检查,不同时期脑电图(包括诱发试验)和脑CT扫描等均未发现异常。经苯妥英钠治疗后2例发作被完全控制,另2例则无效。

Paroxysmal kinesigenic choreoathetosis (PKCA) is a rare nonprogressive involuntary movement disorder induced by movement or startle.The movements are tonic,dystonic or choreoathetotic. Onset occurs most often during childhood Four cases of the PKCA (2 familial form and two sporadic form) were reported All of them had attacks after the initiation of movements, such as rising from chair running, etc. Results of physical examination, laboratory studies, EEGs and CT scans of the head were normal in all patients.The attacks ceased in two of the four patients after treatment with phenytoin.