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肌束震颤以及伴有持续运动单位活动的肌肉松弛受损。

Myokymia and impaired muscular relaxation with continuous motor unit activity.

作者信息

Lublin F D, Tsairis P, Streletz L J, Chambers R A, Riker W F, Van Poznak A, Duckett S W

出版信息

J Neurol Neurosurg Psychiatry. 1979 Jun;42(6):557-62. doi: 10.1136/jnnp.42.6.557.

Abstract

We have studied two cases of the syndrome of myokymia and impaired muscular relaxation with continuous motor unit activity. Both patients complained of muscle twitching, weakness, stiffness, and hyperhydrosis during their illness. Myokymia was present over the entire body in both. On repetitive testing of muscle strength each patient showed initial fatigue followed by increasing strength as he continued his efforts. Both patinets improved on phenytoin therapy at high blood levels. Nerve conduction velocities were decreased. Electromyograms showed continuous electrical activity at rest which persisted during sleep and spinal anaesthesia but was diminished by curare. Intravital staining with methylene blue in one case demonstrated sprouting and beading of motor nerve terminals with multiple innervation of muscle fibres. The neurophysiological and pathological findings in these two cases indicate an abnormality of peripheral nerve in this disorder.

摘要

我们研究了两例伴有持续运动单位活动的肌束震颤和肌肉松弛受损综合征。两名患者在患病期间均主诉有肌肉抽搐、无力、僵硬和多汗。两人全身均出现肌束震颤。对每位患者进行肌肉力量重复测试时,均显示出最初的疲劳,随后随着持续用力力量增强。两名患者在高血药浓度苯妥英治疗下均有改善。神经传导速度降低。肌电图显示静息时存在持续电活动,睡眠和脊髓麻醉期间持续存在,但箭毒可使其减弱。一例患者经亚甲蓝活体染色显示运动神经末梢有发芽和串珠样改变,肌纤维有多重神经支配。这两例患者的神经生理学和病理学发现表明该疾病存在周围神经异常。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9ad/490261/b5b92770504a/jnnpsyc00086-0074-a.jpg

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