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以远端肌无力为主要起病症状的胸腺瘤相关性重症肌无力:一例报告及文献复习

Distal muscle weakness as the main onset symptom in thymoma-associated myasthenia gravis: a case report and literature review.

作者信息

Wu Xuan, Xu Xiao-Tian, Zhou Lin, Qiao Kai, Zhao Chong-Bo, Luo Su-Shan

机构信息

Department of Neurology, Affiliated Hospital of Yangzhou University, Yangzhou, China.

Department of Neurology, Huashan Hospital, Fudan University, Shanghai, China.

出版信息

Front Immunol. 2025 Jan 24;16:1498847. doi: 10.3389/fimmu.2025.1498847. eCollection 2025.

Abstract

Myasthenia gravis (MG) is an autoimmune disorder within the spectrum of neuromuscular rare diseases, characterized by fluctuating muscle weakness. This report presents a case of a middle-aged woman with a chronic onset of asymmetric upper limb weakness accompanied by difficulty in finger extension, without ptosis or fluctuation for 4 years. The patient was finally diagnosed with MG by a significant decrement of Compound Muscle Action Potential in repetitive nerve stimuli, positive anti-acetylcholine receptor antibodies as well as the presence of a mass located in the anterior mediastinum. With subsequent immunotherapies for one month, the patient exhibited marked enhancement in muscle strength, followed by an uneventful thymectomy. After two months, the patient's symptoms were fully alleviated, as evidenced by the reduction in Quantitative MG Score from 9 to 4 points, Myasthenia Gravis Composite Score from 6 to 1 points, Myasthenia Gravis Activities of Daily Living Score from 4 to 1 points, and Myasthenia Gravis Quality of Life-15 score from 14 to 8 points respectively. This case highlights the importance of differentiating autoimmune disorders from hereditary neuromuscular diseases and initiating timely treatment.

摘要

重症肌无力(MG)是一种神经肌肉罕见病范围内的自身免疫性疾病,其特征为肌肉无力波动。本报告介绍了一例中年女性病例,该患者慢性起病,上肢不对称无力,伴有手指伸展困难,4年来无眼睑下垂或症状波动。通过重复神经刺激时复合肌肉动作电位显著递减、抗乙酰胆碱受体抗体阳性以及前纵隔有肿块,该患者最终被诊断为重症肌无力。经过为期一个月的后续免疫治疗,患者肌肉力量显著增强,随后顺利进行了胸腺切除术。两个月后,患者症状完全缓解,定量MG评分从9分降至4分、重症肌无力综合评分从6分降至1分、重症肌无力日常生活活动评分从4分降至1分、重症肌无力生活质量-15评分从14分降至8分,均证明了这一点。该病例强调了区分自身免疫性疾病与遗传性神经肌肉疾病并及时开展治疗的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcc4/11802489/83e5c43f7f0d/fimmu-16-1498847-g001.jpg

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