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与“特发性震颤”相关且运动传导速度正常和/或轻度减慢的夏科-马里-图思病。7例报告。

Charcot-Marie-Tooth disease associated with 'essential tremor' and normal and/or slightly diminished motor conduction velocity. Report of 7 cases.

作者信息

Salisachs P, Codina A, Gimenez-Roldan S, Zarranz J J

出版信息

Eur Neurol. 1979;18(1):49-58. doi: 10.1159/000115053.

Abstract

We present a study of 7 cases of Charcot-Marie-Tooth disease, associated with a dyskinesia clinically identical with essential tremor, in which motor conduction velocity in the upper limbs was normal or slightly diminished. An analysis of age of onset, sex distribution and clinical signs from cases in the literature is compared with the present series. A family with Charcot-Marie-Tooth disease in which affected members have widely different motor conduction velocity values is reported. Stress is laid upon the fact that categorization of this disease on electrophysiological studies in the upper limbs may present considerable difficulties.

摘要

我们报告了7例夏科-马里-图思病(Charcot-Marie-Tooth disease)患者的研究情况,这些患者伴有一种临床上与特发性震颤相同的运动障碍,其上肢运动传导速度正常或略有降低。将文献中病例的发病年龄、性别分布及临床体征分析与本系列病例进行了比较。报告了一个夏科-马里-图思病家族,其中受影响成员的运动传导速度值差异很大。着重指出,根据上肢电生理研究对这种疾病进行分类可能存在相当大的困难。

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