一个罕见的下肢多灶性运动神经病变(MMN)在上肢受累之前的表现。

IF 0.6 4区 医学 Q4 ORTHOPEDICS
J Adrian Wright, Grayson Catherwood, Christian Vanni, Julia Tanaka
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引用次数: 0

摘要

多灶性运动神经病变(MMN)是一种罕见的运动神经元疾病,典型表现为上肢远端不对称肌肉萎缩。作为一种自身免疫性疾病,据推测抗神经节苷-单唾液酸1(抗gm1)抗体的存在导致轴突脱髓鞘,传播MMN症状。静脉注射免疫球蛋白已被证明在恢复肌肉张力和功能,如果在疾病症状的早期发作。虽然不常见,MMN也可影响远端下肢。在这项研究中,我们提出了一名女性患者,在出现与左上肢相似的症状之前,她的右下肢出现肌肉萎缩和全身无力。由于这种非常罕见的表现,病理在疾病进展的后期才被发现。治疗的延迟导致右下肢肌肉张力和功能的永久性降低。据作者所知,这样的发现在目前的文献中尚未报道,这促使人们需要意识到一个已经不常见的疾病的真正不常见的表现。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Rare Lower-Extremity Presentation of Multifocal Motor Neuropathy (MMN) Prior to Upper-Extremity Involvement.

Multifocal motor neuropathy (MMN) is a rare motor neuron condition that typically presents as asymmetric muscular dystrophy to the distal upper extremities. As an autoimmune disorder, it is hypothesized that the presence of anti-ganglioside-monosialic acid 1 (anti-GM1) antibodies results in demyelination of axons, propagating symptoms of MMN. Intravenous immunoglobulin infusions have proven effective in restoring muscle tone and function if administered at early onset of the disease symptoms. Although uncommon, MMN can also affect the distal lower extremities. In this study, we present a female patient with muscle atrophy and general weakness to her right lower extremity prior to developing similar symptoms to her left upper extremity. Due to this very uncommon presentation, the pathology was identified late in the progression of the disease. The delay in treatment resulted in a permanent reduction in muscle tone and function in the right lower extremity. To the best of the authors' knowledge, such a finding has not been reported in the current literature, prompting the need for awareness of a truly uncommon presentation of an already uncommon condition.

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来源期刊
CiteScore
1.10
自引率
0.00%
发文量
128
审稿时长
6-12 weeks
期刊介绍: The Journal of the American Podiatric Medical Association, the official journal of the Association, is the oldest and most frequently cited peer-reviewed journal in the profession of foot and ankle medicine. Founded in 1907 and appearing 6 times per year, it publishes research studies, case reports, literature reviews, special communications, clinical correspondence, letters to the editor, book reviews, and various other types of submissions. The Journal is included in major indexing and abstracting services for biomedical literature.
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