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[莫旺纤维性舞蹈病还剩下什么?]

[What is left of Morvan's fibrillary chorea?].

作者信息

Serratrice G, Azulay J P

机构信息

Clinique des Maladies du Système Nerveux et de l'Appareil Locomoteur, CHU Timone, Marseille.

出版信息

Rev Neurol (Paris). 1994;150(4):257-65.

PMID:7863176
Abstract

In 1890, Morvan described a syndrome of myokimia associated with muscle pain, excessive sweating and sleep disorders. The course was severe and the patient died five weeks after the onset. Several cases were published after this first report in the French literature. The last for 10 years the disease seems to have disappeared. In fact, it seems that the progress in electromyography introduced clear definitions of spontaneous muscle discharges (myokimia, neuromyotonic discharges, fibrillations, fasciculations) allowing recognition of a disease characterized by a syndrome of spontaneous and continuous muscle fiber activity associated with cramping, slow relaxation (pseudomyotonia), excessive sweating and stiffness. Different terms have been used to describe this entity: neuromyotonia (Mertens and Zschocke, 1965) or continuous muscle fibre activity (Isaacs, 1961). Classification of these syndromes has to distinguish inherited from acquired forms, idiopathic cases from cases where neuromyotonia is associated with a peripheral neuropathy, a cancer or an immunological disease. Moreover, the frequent occurrence of this later association and recent reports of improvement after plasma exchanges suggest a possible autoimmune aetiology for this group. Finally, we suggest that the term of "Maladie de Morvan" must be used instead of chorée fibrillaire but only concerning patients who exhibit central disorders including insomnia, hallucinations and altered behaviour.

摘要

1890年,莫尔万描述了一种伴有肌肉疼痛、多汗和睡眠障碍的肌纤维颤搐综合征。病程严重,患者发病五周后死亡。在这篇首次报道之后,法国文献又发表了几例病例。在过去的10年里,这种疾病似乎已经消失。事实上,肌电图学的进展似乎对自发性肌肉放电(肌纤维颤搐、神经肌强直放电、纤颤、束颤)给出了明确的定义,从而得以识别一种以自发性和持续性肌纤维活动综合征为特征的疾病,该综合征伴有痉挛、缓慢松弛(假肌强直)、多汗和僵硬。人们用不同的术语来描述这个实体:神经肌强直(默滕斯和茨绍克,1965年)或持续性肌纤维活动(艾萨克斯,1961年)。这些综合征的分类必须区分遗传性和后天性形式,区分特发性病例与神经肌强直与周围神经病、癌症或免疫性疾病相关的病例。此外,这种后期关联的频繁出现以及血浆置换后病情改善的近期报道表明,这一组病例可能存在自身免疫病因。最后,我们建议必须使用“莫尔万病”这一术语来取代纤维性舞蹈病,但仅用于表现出包括失眠、幻觉和行为改变等中枢性障碍的患者。

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