41例发作性运动诱发性运动障碍临床与神经电生理分析

Clinical and electroneurophysiological study on 41 cases of paroxysmal kinesigenic movement disorders

目的 观察发作性运动诱发性运动障碍 (PKMD)的临床特征及与癫的关系。方法 详细观察 4 1例PKMD的临床特征 ,影像学和脑电图改变。结果 4 1例均由运动诱发 ,呈发作性运动诱发性舞蹈手足徐动症 30例 ,发作性运动诱发性肌张力障碍 11例 ,发作时意识清楚 ,影像学有异常者 6例 ,脑电图有样放电者 12例 ,其中 2例发作时有样放电 ,脑体感诱发电位半数以上病例有定位侧半球改变。对抗药有良效。结论 本症障碍的部位可能在感觉刺激的传入通路与发作症状的传出通路之间的反射中枢。具有某些癫性质 。

Objective To observe the clinical features of paroxysmal kinesigenic movement disorders(PKMD) and to explore their relationship with epilepsy. Methods Clinical data, imaging and electroencephalograms(EEG) changes in 41 patients with PKMD were investigated in detail. Results All the cases were paroxysmal kinesigenic, among which 30 presented as paroxysmal kinesigenic choreoathetosis(PKC) and 11 complained of paroxysmal kinesigenic dystonia(PKD). All patients were alert throughout the ictuses. Abnormal imaging changes occurred in 6 cases, epileptiform discharges in EEG examination did in 12 cases , 2 during attacks. Somatosensory evoked potential examination revealed unilateral hemisphere abnormality in more than half cases . Antiepileptic drugs took effect on all patients. Conclusions Dysfunction of the reflex center between sensory afferent pathway and motor efferent pathway might contribute to dyskinesia which presented some characteristics similar to epilepsy. It was suggcsted that PKMD share similar biological changes with epilepsy.