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[痛性肌束颤动综合征]

[Cramp-fasciculation syndrome].

作者信息

Lagueny A

机构信息

Service de Neurologie, USN Hôpital du Haut-Lévêque, Pessac.

出版信息

Rev Neurol (Paris). 2005 Dec;161(12 Pt 1):1260-6.

Abstract

The cramp-fasciculation syndrome is a rare clinical entity in comparison with the frequency of cramps and isolated fasciculations in the general population. It is recognized as a benign syndrome without weakness and atrophy, however a few reports suggest that it may precede the occurrence of a motor neuron disease. Most often, the cramp-fasciculation syndrome is idiopathic and may be a component of a hyperexcitable peripheral nerve syndrome including other activities such as myokymia and neuromyotonia where antibodies to voltage-gated potassium channels (VGKCs) appear to be one of the effector mechanisms. The most complete form of this hyperexcitable peripheral nerve syndrome is Isaacs' syndrome. The central nervous system is also concerned with anti-VGKC antibodies found in Morvan's disease and limbic encephalitis which is often a paraneoplastic condition. These findings extend the spectrum of the anti-VGKC syndrome that may be associated with other auto-immune diseases, chiefly myasthenia gravis with thymoma. Carbamazepine and phenytoin cause reduction of the clinical and electrophysiological signs of the nerve hyperexcitability, and plasmapheresis and (or) immunosuppressors are useful when an auto-immune origin is considered.

摘要

与普通人群中痉挛和孤立性肌束震颤的发生频率相比,痉挛-肌束震颤综合征是一种罕见的临床病症。它被认为是一种无肌无力和萎缩的良性综合征,然而有少数报告表明它可能先于运动神经元病出现。痉挛-肌束震颤综合征最常见为特发性,可能是包括肌纤维颤搐和神经性肌强直等其他活动在内的外周神经兴奋性增高综合征的一个组成部分,其中电压门控钾通道(VGKC)抗体似乎是一种效应机制。这种外周神经兴奋性增高综合征最完整的形式是艾萨克斯综合征。中枢神经系统也与莫旺病和边缘叶脑炎中发现的抗VGKC抗体有关,边缘叶脑炎通常是一种副肿瘤性疾病。这些发现扩展了抗VGKC综合征的范围,该综合征可能与其他自身免疫性疾病相关,主要是伴胸腺瘤的重症肌无力。卡马西平和苯妥英可减轻神经兴奋性增高的临床和电生理体征,当考虑自身免疫性病因时,血浆置换和(或)免疫抑制剂是有用的。

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