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深入探究:重症肌无力非运动症状的潜在机制

Beyond the Surface: Investigating the Potential Mechanisms of Non-Motor Symptoms in Myasthenia Gravis.

作者信息

Wang Benqiao, Liu Dan, Yang Yingying, Zhu Ruixia

机构信息

Department of Neurology, The First Affiliated Hospital of China Medical University, Shenyang, China.

Department of Developmental Cell Biology, School of Life Sciences, China Medical University, Shenyang, China.

出版信息

Eur J Neurol. 2025 Aug;32(8):e70309. doi: 10.1111/ene.70309.

DOI:10.1111/ene.70309
PMID:40726281
Abstract

BACKGROUND

Myasthenia gravis (MG) is an autoimmune disorder affecting the neuromuscular junction (NMJ), driven by T cells, mediated by B cells, and dependent on autoantibodies. In addition to the typical motor symptoms of fluctuating weakness, the non-motor symptoms are also prevalent among MG patients. This review aims to present the non-motor symptoms of MG and their potential pathogenesis, hoping to contribute to personalized diagnosis and treatment.

METHODS

This review elaborates the non-motor symptoms of MG and systematically detail, for the first time, their potential pathogenic mechanisms, offering a new perspective for clinical evaluation.

RESULTS

The non-motor symptoms of MG include autonomic disorders (urinary, gastrointestinal, cardiovascular and ocular dysfunction), sensory disability (olfactory abnormalities, gustatory reduction and headaches), cognitive impairment, sleep disturbances, psychological problems (depression and anxiety), and TAMG-associated specific syndromes. Due to their insidious onset and lack of awareness, these symptoms are often overlooked. We review the non-motor symptoms of MG and first provide a systematic and detailed discussion on their potential mechanisms, including the influence of MG-specific antibodies (cross-reactivity of AChR-Ab, expression of MuSK-Ab, and striational antibodies at related functional sites), dysregulation of inflammatory factors and immune cells, collateral effects of motor symptoms, impacts of MG comorbidities, and paraneoplastic syndromes caused by thymoma.

CONCLUSION

Non-motor symptoms are common in MG patients. Given a series of potential mechanisms probably involved exploring these non-motor symptoms will not only enhance our understanding of MG but also aid in diagnosis and the development of precise, personalized treatments, ultimately improving the overall life quality of patients.

摘要

背景

重症肌无力(MG)是一种影响神经肌肉接头(NMJ)的自身免疫性疾病,由T细胞驱动,B细胞介导,并依赖自身抗体。除了典型的波动性肌无力运动症状外,非运动症状在MG患者中也很普遍。本综述旨在介绍MG的非运动症状及其潜在发病机制,希望有助于个性化诊断和治疗。

方法

本综述阐述了MG的非运动症状,并首次系统详细地阐述了其潜在致病机制,为临床评估提供了新的视角。

结果

MG的非运动症状包括自主神经功能障碍(泌尿、胃肠、心血管和眼部功能障碍)、感觉障碍(嗅觉异常、味觉减退和头痛)、认知障碍、睡眠障碍、心理问题(抑郁和焦虑)以及与TAMG相关的特定综合征。由于这些症状起病隐匿且易被忽视,常常被漏诊。我们回顾了MG的非运动症状,并首次对其潜在机制进行了系统而详细的讨论,包括MG特异性抗体的影响(AChR-Ab的交叉反应性、MuSK-Ab的表达以及相关功能位点的横纹肌抗体)、炎症因子和免疫细胞的失调、运动症状的附带效应、MG合并症的影响以及胸腺瘤引起的副肿瘤综合征。

结论

非运动症状在MG患者中很常见。鉴于可能涉及一系列潜在机制,探索这些非运动症状不仅会增进我们对MG的理解,还将有助于诊断以及精准、个性化治疗的发展,最终提高患者的整体生活质量。

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本文引用的文献

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Sleep in Myasthenia Gravis: A Questionnaire-Based Study.重症肌无力患者的睡眠问题:一项基于问卷的研究。
Neurol India. 2024 Jul 1;72(4):801-805. doi: 10.4103/neurol-india.NI_562_17. Epub 2024 Aug 31.
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Myasthenia gravis: the future is here.重症肌无力:未来已来。
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Frequency and severity of autonomic dysfunction assessed by objective hemodynamic responses and patient-reported symptoms in individuals with myasthenia gravis.通过客观血流动力学反应和重症肌无力患者报告的症状评估自主神经功能障碍的频率和严重程度。
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Treating myasthenia gravis beyond the eye clinic.眼肌型重症肌无力的治疗不应局限于眼科。
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Neurometabolic and Brain Functional Alterations Associated with Cognitive Impairment in Patients with Myasthenia Gravis: A Combined H-MRS and fMRI Study.神经代谢和脑功能改变与重症肌无力患者认知障碍的关系:一项 H-MRS 和 fMRI 的联合研究。
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Cervicogenic headache - How to recognize and treat.颈源性头痛 - 如何识别和治疗。
Best Pract Res Clin Rheumatol. 2024 Mar;38(1):101931. doi: 10.1016/j.berh.2024.101931. Epub 2024 Feb 22.
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Myasthenia gravis: the changing treatment landscape in the era of molecular therapies.重症肌无力:分子治疗时代不断变化的治疗前景。
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The Burden Patients with Myasthenia Gravis Experience in Terms of Breathing, Fatigue, Sleep, Mental Health, Discomfort and Usual Activities in Comparison to the General Population.重症肌无力患者在呼吸、疲劳、睡眠、心理健康、不适和日常活动方面与普通人群相比所承受的负担。
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