发作性运动诱导性运动障碍的临床特征

目的:分析发作性运动诱导性运动障碍(PKC)的临床特征,并评定抗癫药物的疗效。方法:记录并分析31例PKC患者的一般资料、临床表现、脑电图和影像学改变及治疗情况。结果:本组患者平均起病年龄为16.8岁,10-20岁为起病高峰;最常见的发作诱因是突然运动起动(92%);发作时间均不超过1min;发作时表现为肌张力障碍(83.9%)、舞蹈样动作(16.1%)、口面部异常动作及其他(16.2%);71%发作频繁,超过1次/天;脑电图及影像学检查大多正常;小剂量抗癫药物治疗后50%-77.3%发作完全控制。结论:PKC是神经科常见的运动障碍性疾病,抗癫药物效果良好,应注意与癫鉴别,同时其与癫的相关性需要深入的临床随访研究。

Clinical Characteristics of Paroxysmal Kinesigenic Choreoathetosis

Objective:Clinical characteristics and efficacy of AEDs of paroxysmal kinesigenic choreoathetosis(PKC)have been analyzed.Methods:Thirty-one patients with PKC were recruited and clinically analyzed.Results:The average age of the first attack is 16.8 years old and the pinnacle is 10 to 20 years old.Sudden movement is the most common provocative causes(92%).The whole attack lasts less than 1 minute,manifested by muscle tension disturbances(83.9%),movement-like dancing(16.1%),abnormal movements of the mouth and face and other symptoms(16.2%).The attacks tend to be very frequent and 71% of patients experience more than one attack per day.EEG and neuroimaging study showed no abnormalities in most patients.Low dosage of anti-epilepsy drugs(AEDs)can control the attacks in 50 %-77.3 % of the patients.Conclusion:PKC is a common movement disorder in the neurological diseases.The AEDs is very effective.Differentiation between epilepsy and PKC needs our attention,and further clinical research in this disorder is necessary.