【摘要】 目的 :总结发作性运动障碍中PKC的临床特征 ,并对其发病机制加以探讨。方法 :对 8例PKC患者临床表现及实验室资料进行分析。结果 :本组中 8例患者发病年龄 8～ 14岁 ,病程 1～ 10年 ,87%为男性。临床表现均由突发运动诱发 ,由久坐、久立后突然改变体位 ,表现为一侧或双侧不自主肢体舞动、躯干扭动和面部异常运动 ,持续数秒钟自行缓解 ,发作时意识清楚 ,2例EEG轻度异常 ,6例正常EEG中有 2例 2 4h动态脑电图示样放电 ,8例服用少量卡马西平完全控制发作。结论 :PKC是发作性运动障碍中一类疾病 ,以开始运动时出现发作性锥体外系症状为特点 ,发作范围局部或全身 ,是可以治愈的 ,抗癫药治疗效果良好更多还原

【Abstract】 Aim:To investigate the pathogenesis and clinical characteristics of paroxysmal dyskinesias.Methods:8 Patients with paroxysmal kinesigenic choreoathetosis(PKC) were analyzed including the clinical manifestation,the factors for inducing the attacks,duration of episode,therapeutic response etc.Etiologic studied included EEG,24 hours EEG monitoring,brain CT/MRI,serum T 3,T 4 level etc.Results:8 Patients with PKC,the age of onset ranged from 8 to 14 years,the history of PKC ranged from 1 to 10 years.87% of the cases were man.The episodes of all cases were induced by sudden movement or a startle or emotional stress.The duration of the attack is brief,usually lasting only seconds.The attacks in all cases were periodic involuntary movements of one side or both sides of the limbs,trunks,face and were often associated with dystonic,choreic,ballistic or athetoid movements without loss of consciousness.All laboratory tests showed no abnormality except for 4 cases had abnormal EEG findings,all patients responded well to low doses of carbamazepine.Conclusion:PKC is one kind of paroxysmal dyskinesias.PKC is characterized by short paroxysmal of focal or generalized involuntary moveement induced by sudden movement, PKC is known to be a treatable disorder.The treatment of antiepilepsy drugs can completely control the attacks.更多还原