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产后神经性肌萎缩

Postpartum neuralgic amyotrophy.

作者信息

Lederman R J, Wilbourn A J

机构信息

Department of Neurology, Cleveland Clinic Foundation, OH, USA.

出版信息

Neurology. 1996 Nov;47(5):1213-9. doi: 10.1212/wnl.47.5.1213.

DOI:10.1212/wnl.47.5.1213
PMID:8909432
Abstract

We report 11 women with at least one episode of neuralgic amyotrophy occurring postpartum. One woman had three episodes-two postpartum and a third occurring after minor foot surgery. Two others had a second episode, one following a first trimester spontaneous abortion and one after a viral syndrome. Pain followed delivery from as little as 1 to 2 hours to up to 2 weeks and usually lasted from a few weeks to several months. Weakness, if delayed, followed onset of pain by 2 or 3 days up to 5 weeks. Four of the episodes were bilateral. Clinical weakness and electrodiagnostic findings varied widely, from involvement of a single peripheral nerve (e.g., long thoracic, anterior interosseous) to multiple bilateral proximal and distal nerves. Functional recovery was excellent (90 to 100%) in 8 of the 10 with adequate follow-up, requiring as little as 2 weeks or up to 3 years. Two women have moderate persisting weakness (both bilateral) at 3 years and 2 years. Five patients went on to subsequent deliveries without recurrence of neuralgic amyotrophy. Only one of the 11 women had a history suggesting familial neuralgic amyotrophy, a disorder generally thought to be associated particularly with postpartum episodes. Although the etiology of postpartum neuralgic amyotrophy remains unknown, an immunologic mechanism is suspected. Focal demyelination may play a role in some, but clearly axonal degeneration is predominant in the majority.

摘要

我们报告了11例产后至少发作过一次神经性肌萎缩的女性。其中一名女性发作过三次,两次在产后,第三次在足部小手术后发作。另外两名女性有第二次发作,一名在孕早期自然流产后发作,另一名在病毒感染综合征后发作。疼痛在分娩后1至2小时至2周内出现,通常持续数周至数月。如果出现延迟性肌无力,则在疼痛发作后2至3天至5周内出现。其中4次发作是双侧的。临床肌无力和电诊断结果差异很大,从单一周围神经(如胸长神经、骨间前神经)受累到多根双侧近端和远端神经受累。在10例有充分随访的患者中,8例功能恢复良好(90%至100%),恢复时间最短为2周,最长为3年。两名女性在3年和2年时仍有中度持续性肌无力(均为双侧)。5名患者随后再次分娩,神经性肌萎缩未复发。11名女性中只有1名有提示家族性神经性肌萎缩的病史,这种疾病通常被认为特别与产后发作有关。尽管产后神经性肌萎缩的病因仍不清楚,但怀疑有免疫机制。局灶性脱髓鞘可能在某些病例中起作用,但显然大多数病例中轴索变性占主导。

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