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艾萨克斯与默滕斯综合征:1例报告(作者译)

[Isaacs and Mertens syndrome: report of one case (author's transl)].

作者信息

Cattaino G

出版信息

Riv Patol Nerv Ment. 1981 Mar-Apr;102(2):87-96.

PMID:7345555
Abstract

The author reports a clinical case of Isaacs-Mertens syndrome (syndrome of continuous muscle fibre activity, neuromyotonia, pseudomyotonia). A 55 years old man had increasing stiffness, painful spasms and myokymias of the peripheral muscle, associated with hyperhidrosis and absent tendon reflexes. Electromyography showed continuous electrical activity while at rest, general anaesthesia, after intravenous injection of diazepam and after peripheral nerve block. The activity disappeared after muscle infiltration of procaine and after intravenous injection of succinylcholine. After two years treatment with carbamazepine, 600 mg daily, the patient showed a normal clinical and neurophysiological picture. The etiology of the disease is unknown. The defect probably lies in the terminal motor network. A review of previous literature shows a typical uniformity of the electromyographic findings and of improvement on phenytoin and carbamazepine therapy, while there is a relative polymorphism of the clinical features.

摘要

作者报告了一例艾萨克斯 - 默滕斯综合征(持续性肌纤维活动综合征、神经性肌强直、假性肌强直)的临床病例。一名55岁男性出现外周肌肉僵硬加重、疼痛性痉挛和肌束震颤,伴有多汗和腱反射消失。肌电图显示在静息状态、全身麻醉时、静脉注射地西泮后以及外周神经阻滞后均有持续性电活动。在肌肉浸润普鲁卡因和静脉注射琥珀酰胆碱后,该活动消失。在用卡马西平每日600毫克治疗两年后,患者的临床和神经生理学表现恢复正常。该病的病因尚不清楚。缺陷可能存在于终末运动网络。对既往文献的回顾显示,肌电图表现具有典型的一致性,且苯妥英钠和卡马西平治疗有效,但临床特征存在相对多态性。

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