Research progress on diagnostic strategies and clinical treatment of myasthenia gravis

doi:10.3760/cma.j.cn441417-20250424-17007

Abstract

Abstract:

Myasthenia gravis (MG), as a rare neuromuscular junction disorder, the advancements in its diagnosis and treatment have a profound impact on the prognosis and quality of life of patients. This article will systematically elaborate on the diagnostic strategies of MG (clinical diagnosis, serum antibody testing, electrophysiological examination, and pharmacological testing), and summarize its treatment plans (pharmacological treatment, surgical treatment, and immunotherapy). By analyzing the current medical treatment situation, the latest research progress and the future research directions, valuable references are provided for clinical practice.

Key words:

Myasthenia gravis, Diagnosis, Treatment, Progress

摘要：

重症肌无力（MG）作为一种罕见的神经肌肉接头疾病，其诊断与治疗的进展对于患者预后及生活质量的提升具有深远影响。本文将系统阐述MG的诊断策略（临床诊断、血清抗体检测、电生理检查及药物学检测），并归纳其治疗方案（药物治疗、手术治疗及免疫治疗）。通过分析当前诊疗现状、最新研究进展及未来研究方向，为临床实践提供有价值的参考。

关键词:

重症肌无力, 诊断, 治疗, 进展

Gao Yifan, Zhang Wei, Zhang Ziliang, Chen Jinbo.

Research progress on diagnostic strategies and clinical treatment of myasthenia gravis [J]. International Medicine and Health Guidance News, 2025, 31(17): 2851-2855.

高一凡张威张子良陈金波.

重症肌无力的诊断策略与临床治疗研究进展 [J]. 国际医药卫生导报, 2025, 31(17): 2851-2855.

References

[1] Gilhus NE, Tzartos S, Evoli A, et al. Myasthenia gravis[J]. Nat Rev Dis Primers, 2019, 5(1):30. DOI: 10.1038/s41572-019-0079-y.
[2] 张华,闻洁曦. 重症肌无力诊治进展[J]. 中国神经免疫学和神经病学杂志,2020,27(1):6-8.DOI:10.3969/j.issn.1006-2963.2020.01.002.
[3] 王秀云,许贤豪,孙宏,等. 重症肌无力病人的临床绝对评分法和相对评分法[J]. 中华神经科杂志,1997,30(2):87-90.
[4] Barohn RJ, Mcintire D, Herbelin L, et al. Reliability testing of the quantitative myasthenia gravis score[J]. Ann N Y Acad Sci, 1998, 841:769-772. DOI: 10.1111/j.1749-6632.1998.tb11015.x.
[5] De Moura CM, Magno GM. Ice pack test in myasthenic ptosis as an important tool for clinical practice[J]. J Clin Neuromuscul Dis, 2021, 23(2):110-111. DOI: 10.1097/CND.0000000000000338.
[6] 常婷,李柱一. 中国重症肌无力研究进展近十年回顾与展望[J]. 中国神经免疫学和神经病学杂志,2022,29(5):349-355. DOI:10.3969/j.issn.1006-2963.2022.05.001.
[7] 邹玲莉,蔡定军,陈学波. 间接免疫荧光法检测AchR抗体、MuSK抗体和LRP4抗体在重症肌无力诊断中的价值[J]. 中国实验诊断学,2021,25(5):654-657. DOI:10.3969/j.issn.1007-4287.2021.05.006.
[8] Gastaldi M, Scaranzin S, Businaro P, et al. Improving laboratory diagnostics in myasthenia gravis[J]. Expert Rev Mol Diagn, 2021, 21(6):579-590. DOI: 10.1080/14737159.2021.1927715.
[9] Li Z, Zhang C, Chang T, et al. A multicentre, prospective, double-blind study comparing the accuracy of autoantibody diagnostic assays in myasthenia gravis: the SCREAM study[J]. Lancet Reg Health West Pac, 2023, 38:100846. DOI: 10.1016/j.lanwpc.2023.100846.
[10] Katzberg HD, Abraham A. Electrodiagnostic assessment of neuromuscular junction disorders[J]. Neurol Clin, 2021, 39(4):1051-1070. DOI: 10.1016/j.ncl.2021.06.013.
[11] 中国免疫学会神经免疫分会. 中国重症肌无力诊断和治疗指南(2020版)[J]. 中国神经免疫学和神经病学杂志,2021,28(1):1-12. DOI:10.3969/j.issn.1006-2963.2021.01.001.
[12] Juel VC. Single fiber electromyography[J]. Handb Clin Neurol, 2019, 160:303-310. DOI: 10.1016/B978-0-444-64032-1.00019-9.
[13] Yang XG, Guo HY, Peng Z, et al. Application of electrophysiological techniques in assessing of neuromuscular junction-related disorders[J]. World Neurosurg, 2024, 191:165-171. DOI: 10.1016/j.wneu.2024.08.076.
[14] 武卫周,员丽娜,路美,等. MuSK抗体阳性的重症肌无力诊断及治疗最新进展[J]. 武警医学,2020,31(12):1080-1083.DOI: 10.14010/j.cnki.wjyx.2020.12.019.
[15] Crisafulli S, Boccanegra B, Carollo M, et al. Myasthenia gravis treatment: from old drugs to innovative therapies with a glimpse into the future[J]. CNS Drugs, 2024, 38(1):15-32. DOI: 10.1007/s40263-023-01059-8.
[16] Dalakas MC. Immunotherapy in myasthenia gravis in the era of biologics[J]. Nat Rev Neurol, 2019, 15(2):113-124. DOI: 10.1038/s41582-018-0110-z.
[17] García ED, Pardo FJ. Myasthenia gravis. Update on diagnosis and therapy[J]. Med Clin (Barc), 2023, 161(3):119-127. DOI: 10.1016/j.medcli.2023.04.006.
[18] Zhen L, Zhao X, Li W, et al. Effectiveness of early glucocorticoids in myasthenia gravis: a retrospective cohort study[J]. Front Neurol, 2023, 14:1259484. DOI: 10.3389/fneur.2023.1259484.
[19] Lascano AM, Lalive PH. Update in immunosuppressive therapy of myasthenia gravis[J]. Autoimmun Rev, 2021, 20(1):102712. DOI: 10.1016/j.autrev.2020.102712.
[20] Bar J, Stahl B, Hod M, et al. Is immunosuppression therapy in renal allograft recipients teratogenic? A single-center experience[J]. Am J Med Genet A, 2003, 116A(1):31-36. DOI: 10.1002/ajmg.a.10817.
[21] Verschuuren J. New therapies for autoimmune myasthenia gravis[J]. Lancet Neurol, 2023, 22(5):368-369. DOI: 10.1016/S1474-4422(23)00125-4.
[22] Howard JF, Bril V, Vu T, et al. Safety, efficacy, and tolerability of efgartigimod in patients with generalised myasthenia gravis (ADAPT): a multicentre, randomised, placebo-controlled, phase 3 trial[J]. Lancet Neurol, 2021, 20(7):526-536. DOI: 10.1016/S1474-4422(21)00159-9.
[23] Howard JF, Vissing J, Gilhus NE, et al. Zilucoplan: an investigational complement C5 inhibitor for the treatment of acetylcholine receptor autoantibody-positive generalized myasthenia gravis[J]. Expert Opin Investig Drugs, 2021, 30(5):483-493. DOI: 10.1080/13543784.2021.1897567.
[24] Howard JF, Bresch S, Genge A, et al. Safety and efficacy of zilucoplan in patients with generalised myasthenia gravis (RAISE): a randomised, double-blind, placebo-controlled, phase 3 study[J]. Lancet Neurol, 2023, 22(5):395-406. DOI: 10.1016/S1474-4422(23)00080-7.
[25] 谭群友,陶绍霖,刘宝东,等. 重症肌无力外科治疗中国临床专家共识[J]. 中国胸心血管外科临床杂志,2022,29(5):529-541.DOI: 10.7507/1007-4848.202202043.
[26] Fernández-Fournier M, Kerguelen A, Rodríguez de Rivera FJ, et al. Therapeutic plasma exchange for myasthenia gravis, Guillain-Barre syndrome, and other immune-mediated neurological diseases, over a 40-year experience[J]. Expert Rev Neurother, 2022, 22(10):897-903. DOI: 10.1080/14737175.2022.2147827.
[27] Lazaridis K, Tzartos SJ. Autoantibody specificities in myasthenia gravis; implications for improved diagnostics and therapeutics[J]. Front Immunol, 2020, 11:212. DOI: 10.3389/fimmu.2020.00212.
[28] Dalakas MC. Update on intravenous immunoglobulin in neurology: modulating neuro-autoimmunity, evolving factors on efficacy and dosing and challenges on stopping chronic IVIg therapy[J]. Neurotherapeutics, 2021, 18(4):2397-2418. DOI: 10.1007/s13311-021-01108-4.
[29] 中国罕见病联盟神经罕见病专业委员会,中国罕见病联盟重症肌无力协作组,中华医学会神经病学分会神经肌肉病学组,等. 中国难治性全身型重症肌无力诊断和治疗专家共识(2024版)[J]. 中华神经科杂志,2024,57(8):840-847.DOI:10.3760/cma.j.cn113694-20240222-00106.
[30] Haghikia A, Hegelmaier T, Wolleschak D, et al. Anti-CD19 CAR T cells for refractory myasthenia gravis[J]. Lancet Neurol, 2023, 22(12):1104-1105. DOI: 10.1016/S1474-4422(23)00375-7.