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目的 总结各类阵发性运动障碍性疾病的临床特征与治疗反应 ,并对其发病机理、分类加以探讨。方法 对 12例阵发性运动障碍性疾病患儿进行了临床观察与随访 ,及录像脑电图(VEEG)、头颅CT和 (或 )MRI、铜蓝蛋白、角膜K F环和肌电图 (EMG)检查。结果 本组中 6例为阵发性运动性舞蹈手足徐动 (PKC) ,6例为阵发性肌张力不全舞蹈手足徐动 (PDC) ,无阵发性运动诱发的肌张力不全 (PED)。CT和 (或 )MRI、角膜K F环及EMG无特殊 ,3例PKC患儿EEG有样放电。PKC对抗癫药反应良好 ,PDC无有效治疗。结论 阵发性运动障碍包含了一组少见的反复发作的运动异常性疾病 ,可以分为PKC、PDC、PED、阵发性睡眠诱发运动障碍 (PHD)和其他。PKC由突发运动所诱发 ,发作频繁 ,持续时间短暂 ,抗癫药治疗效果显著 ,其发病机理可能与癫有关。PDC与运动无关 ,持续时间长短不一 ,尚无有效的治疗方法
Objective To summarize the clinical characteristics and treatment response of various types of paroxysmal nociceptive disorders, and to explore its pathogenesis and classification. Methods Twelve patients with paroxysmal nocturnal dyskinesia were observed and followed up. VEEG, CT and MRI, ceruloplasmin, corneal KF ring and EMG )an examination. Results Six cases in this group were patients with paroxysmal sports dance hand-foot-and-knee hyperplasia (PKC), 6 cases with paroxysmal atypical choreosis (PDC), no paroxysmal motor-induced dystonia (PED) . CT and (or) MRI, corneal KF ring and EMG no special, 3 cases of PKC children with EEG-like discharge. PKC responds well to antiepileptic drugs and PDC has no effective treatment. CONCLUSIONS: Paroxysmal dyskinesia includes a rare group of recurrent anomalous disorders that can be classified as PKC, PDC, PED, paroxysmal sleep-induced dyskinesia (PHD), and others. PKC is triggered by sudden movement, frequent seizures, short duration, anti-epileptic drug treatment effect is remarkable, the pathogenesis may be related to epilepsy. PDC has nothing to do with exercise, duration varies, there is no effective treatment