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阵发性舞蹈手足徐动症

Paroxysmal choreoathetosis.

作者信息

Lüders H O

机构信息

Cleveland Clinic Foundation, Department of Neurology/S90, Ohio 44195, USA.

出版信息

Eur Neurol. 1996;36 Suppl 1:20-3. doi: 10.1159/000118879.

Abstract

Paroxysmal choreoathetosis is the essential symptomatology in the following 4 syndromes: (a) paroxysmal kinesogenic choreoathetosis; (b) paroxysmal nonkinesogenic choreoathetosis; (c) supplementary sensorimotor seizures, and (d) paroxysmal nocturnal dystonia. All these syndromes are characterized by similar clinical symptomatology of the paroxysmal events. However, the pathophysiology of the choreoathetotic episodes differs considerably. In the first two syndromes the paroxysms are most probably generated by nonepileptogenic abnormal discharges in the basal ganglia whereas in the third syndrome (supplementary sensorimotor seizures) there is convincing evidence indicating that the pathogenesis is an epileptic process in the cortex. Finally, in the last syndrome (paroxysmal nocturnal dystonia) there is still controversy whether the paroxysms are of an epileptic or nonepileptic nature.

摘要

发作性舞蹈手足徐动症是以下4种综合征的主要症状表现:(a)发作性运动诱发性舞蹈手足徐动症;(b)发作性非运动诱发性舞蹈手足徐动症;(c)辅助性感觉运动性癫痫发作,以及(d)发作性夜间肌张力障碍。所有这些综合征都以发作性事件的相似临床症状为特征。然而,舞蹈手足徐动症发作的病理生理学有很大差异。在前两种综合征中,发作很可能是由基底神经节的非癫痫性异常放电引起的,而在第三种综合征(辅助性感觉运动性癫痫发作)中,有令人信服的证据表明其发病机制是皮质的癫痫过程。最后,在最后一种综合征(发作性夜间肌张力障碍)中,发作是癫痫性还是非癫痫性性质仍存在争议。

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