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模仿肌萎缩侧索硬化症的抗肌肉特异性激酶抗体阳性重症肌无力

Anti-MuSK Antibody-positive Myasthenia Gravis Mimicking Amyotrophic Lateral Sclerosis.

作者信息

Furuta Natsumi, Ishizawa Kunihiko, Shibata Makoto, Tsukagoshi Setsuki, Nagamine Shun, Makioka Kouki, Fujita Yukio, Ikeda Masaki, Yoshimura Shunsuke, Motomura Masakatsu, Okamoto Koichi, Ikeda Yoshio

机构信息

Department of Neurology, Gunma University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2015;54(19):2497-501. doi: 10.2169/internalmedicine.54.4645. Epub 2015 Oct 1.

Abstract

We herein investigated the clinical features of three patients with anti-muscle-specific tyrosine kinase (MuSK) antibody-positive myasthenia gravis (MG), which was initially difficult to distinguish from amyotrophic lateral sclerosis (ALS). The patients exhibited dropped head syndrome or dysphagia as initial symptoms. Although their clinical findings were compatible with the revised El Escorial Criteria for ALS, their progression appeared to be more rapid than that of ALS. Both the edrophonium and repetitive nerve stimulation tests yielded negative results, and diurnal fluctuation was not confirmed. The patients were ultimately diagnosed with anti-MuSK antibody-positive MG. We therefore recommend the measurement of anti-MuSK antibodies when encountering such cases.

摘要

我们在此研究了3例抗肌肉特异性酪氨酸激酶(MuSK)抗体阳性重症肌无力(MG)患者的临床特征,这些患者最初难以与肌萎缩侧索硬化症(ALS)相区分。患者最初表现为低头综合征或吞咽困难。尽管他们的临床表现符合修订后的ALS埃斯科里亚尔标准,但病情进展似乎比ALS更快。依酚氯铵试验和重复神经电刺激试验结果均为阴性,且未证实有日间波动。这些患者最终被诊断为抗MuSK抗体阳性MG。因此,我们建议遇到此类病例时检测抗MuSK抗体。

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