发作性运动诱发性运动障碍临床分析

Clinical analysis of paroxysmal kinesigenic dyskinesia

目的探讨发作性运动诱发性运动障碍的临床特点。方法依据Demirkiran等的诊断标准对19例患者进行确诊,并对其临床资料进行分析。结果本组患者发病年龄为7～16岁,随年龄增长病情逐渐缓解;通常由突然随意运动后而出现,表现肢体(最常受累)、躯干、颈部或面部的肌张力障碍,持续数秒至数十秒,意识清楚;发作间歇期运动功能正常;发作期及间歇期脑电图、神经影像学检查多正常;对抗癫痫药物反应良好。结论发作性运动诱发性运动障碍是临床上较为少见的疾病,容易误诊,但临床疗效及预后良好。

Objective To explore the clinical features of paroxysmal kinesigenic dyskinesia （PKD）. Methods A total of 19 cases were definitely diagnosed according to the diagnostic criteria of Demirkiran et al and the clinical data of these patients were analyzed. Results The onset age of PKD for subjects in this study was between 7 and 16 years old, and the disease became alleviated with the increasing of age; PKD in this study occurred frequently following sudden voluntary movement, showing dystonia involving extremities （most fi＇equently）, think, neck or face and lasting for several to tens of seconds, but patients still had clear consciousness; motor function was still normal during the intermittent period of onset; electroencephalogram （EEG） and neuroimaging examinations were mainly normal during the attack and intermittent periods; most patients responded well to antiepileptic drugs. Conclusion PKD is clinically uncommon and prone to be misdiagnosed, but it has good antiepileptic effects and prognosis.