抗 MuSK 抗体阳性的重症肌无力患者的垂头综合征可能并发轴性肌病

Q4 Immunology and Microbiology Clinical and Experimental Neuroimmunology Pub Date : 2024-02-18 DOI:10.1111/cen3.12782

So Okubo, Mitsuhiro Kainaga, Shin-ichi Tokushige, Ayumi Uchibori, Chizuko Oishi, Teruyuki Hirano, Yaeko Ichikawa

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摘要

低头综合征（DHS）是一组导致颈部前屈的疾病。重症肌无力（MG）是导致 DHS 的常见原因。以前的报告表明，抗乙酰胆碱受体抗体阳性或胸腺肿瘤相关性 MG 患者的垂头综合征可能并发脊柱旁肌肉病变。神经系统检查发现双侧上睑下垂和颈后伸肌无力。电生理学检查提示神经肌肉接头受累。血清抗MuSK抗体呈阳性，诊断为全身性抗MuSK抗体阳性的重症肌无力（MuSK-MG）。脾盖肌和脊柱旁肌肉的针刺肌电图（nEMG）显示出急性和慢性肌源性改变。胸腺瘤阴性的MuSK-MG表现为难治性DHS，可能同时伴有轴性肌病。

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Dropped head syndrome in anti-MuSK antibody-positive myasthenia gravis with possible concurrent axial myopathy

Background

Dropped head syndrome (DHS) is a group of disorders that result in anterior neck flexion. Myasthenia gravis (MG) is a common cause of DHS. Previous reports have suggested that concurrent myopathy involving the paraspinal musculature may underlie DHS in patients with anti-acetylcholine receptor antibody-positive or thymoma-associated MG.

Case Presentation

A 64-year-old woman presented with a 3-month history of head drop and lordosis. Neurological examination revealed bilateral ptosis and weakness of the posterior neck extensors. Electrophysiology suggested neuromuscular junction involvement. Serum anti-MuSK antibodies were positive, and generalized anti-MuSK antibody-positive myasthenia gravis (MuSK-MG) was diagnosed. Needle electromyography (nEMG) of the splenius capitus and paraspinal muscles revealed acute and chronic myogenic changes. Imaging suggested paraspinal muscle atrophy. nEMG and magnetic resonance imaging (MRI) of both extremities were normal, and autoimmune myopathy-related antibody testing was negative.