Focusing on the diagnosis and treatment of ocular myasthenia gravis —clinical challenges for ophthalmologists and strategies for early identification_Chinese Journal of Ocular Fundus Diseases

Authors：

Wu Songdi ¹ , Lin Xuemei ¹ ,  Wei Shihui ²

1. Department of Neuro-Ophthalmology, The First Hospital of Xi’an (The First Affiliated Hospital of Northwest University), Xi’an 710002, China;
2. Department of Neuro-Ophthalmology, Chinese PLA General Hospital, Beijing 100853, China;

Corresponding?author：

Wei Shihui, Email: weishihui@hotmail.com

Keywords：

Ocular myasthenia gravis; Early identification; Diagnosis and treatment; Editorial

DOI：

10.3760/cma.j.cn511434-20251208-00566

Video：

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Ocular myasthenia gravis (OMG) is the most common phenotype of myasthenia gravis (MG) in clinical practice. Patients often present to ophthalmology clinics with symptoms such as ptosis and/or diplopia. OMG exhibits high clinical heterogeneity and can mimic various types of extraocular muscle palsy, which easily leads to misdiagnosis or underdiagnosis. More than half of OMG patients may progress to generalized MG within two years of onset, with severe cases potentially being life-threatening. Therefore, early and accurate identification and diagnosis of OMG not only alleviate ocular symptoms and improve patients’ quality of life but also reduce the risk of systemic progression through appropriate treatment. In clinical practice, there is often a delay or lag in the diagnosis and management of OMG and need to standardize OMG clinical pathways and establish multidisciplinary collaboration models to improve the overall prognosis of OMG patients.

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1. Iorio R. Myasthenia gravis: the changing treatment landscape in the era of molecular therapies[J]. Nat Rev Neurol, 2024, 20(2): 84-98. DOI: 10.1038/s41582-023-00916-w.
2. 中國免疫學會神經免疫分會. 中國重癥肌無力診斷和治療指南(2020版)[J]. 中國神經免疫學和神經病學雜志, 2021, 28(1): 1-12. DOI: 10.3969/j.issn.1006-2963.2021.01.001.Neuroimmunology Branch of the Chinese Society for Immunology. Chinese guidelines for the diagnosis and treatment of myasthenia gravis (2020 Edition)[J]. Chin J Neuroimmunol & Neurol, 2021, 28(1): 1-12. DOI: 10.3969/j.issn.1006-2963.2021.01.001.
3. Hendricks TM, Bhatti MT, Hodge DO, et al. Incidence, epidemiology, and transformation of ocular myasthenia gravis: a population-based study[J]. Am J Ophthalmol, 2019, 205: 99-105. DOI: 10.1016/j.ajo.2019.04.017.
4. Nagia L, Lemos J, Abusamra K, et al. Prognosis of ocular myasthenia gravis: retrospective multicenter analysis[J]. Ophthalmology, 2015, 122(7): 1517-1521. DOI: 10.1016/j.ophtha.2015.03.010.
5. Zhu Y, Wang B, Hao Y, et al. Clinical features of myasthenia gravis with neurological and systemic autoimmune diseases[J/OL]. Front Immunol, 2023, 14: 1223322[2023-09-14]. https://pubmed.ncbi.nlm.nih.gov/37781409/. DOI: 10.3389/fimmu.2023.1223322.
6. Chen J, Tian DC, Zhang C, et al. Incidence, mortality, and economic burden of myasthenia gravis in China: a nationwide population-based study[J/OL]. Lancet Reg Health West Pac, 2020, 5: 100063[2020-11-27]. https://pubmed.ncbi.nlm.nih.gov/34327399/. DOI: 10.1016/j.lanwpc.2020.100063.
7. Grob D, Brunner N, Namba T, et al. Lifetime course of myasthenia gravis[J]. Muscle Nerve, 2008, 37(2): 141-149. DOI: 10.1002/mus.20950.
8. Behbehani R. Ocular myasthenia gravis: a current overview[J]. Eye Brain, 2023, 15: 1-13. DOI: 10.2147/EB.S389629.
9. Fang CEH, Bokre D, Wong SH. Clinical characteristics associated with secondary generalization in patients with ocular myasthenia gravis: a systematic review and meta-analysis[J/OL]. Neurology, 2023, 101(16): e1594-e605[2023-10-17]. https://pubmed.ncbi.nlm.nih.gov/37643888/. DOI: 10.1212/WNL.0000000000207642.
10. Menon D, Alharbi M, Katzberg HD, et al. Effect of immunosuppression in risk of developing generalized symptoms in ocular myasthenia gravis: a retrospective cohort study[J/OL]. Neurology, 2024, 103(4): e209722[2024-08-27]. https://pubmed.ncbi.nlm.nih.gov/39079067/. DOI: 10.1212/WNL.0000000000209722.
11. Evoli A, Iorio R. Controversies in ocular myasthenia gravis[J/OL]. Front Neurol, 2020, 11: 605902[2020-11-30]. https://pubmed.ncbi.nlm.nih.gov/33329368/. DOI: 10.3389/fneur.2020.605902.
12. Djordjevic IS, Peric S, Djordjevic SA, et al. Risk factors for generalization of ocular myasthenia gravis[J]. Indian J Ophthalmol, 2025, 73(10): 1460-1465. DOI: 10.4103/IJO.IJO_650_25.
13. Kee HJ, Yang HK, Hwang JM, et al. Evaluation and validation of sustained upgaze combined with the ice-pack test for ocular myasthenia gravis in Asians[J]. Neuromuscul Disord, 2019, 29(4): 296-301. DOI: 10.1016/j.nmd.2018.12.011.
14. Pietris J, Madike R, Lam A, et al. Cogan's lid twitch for myasthenia gravis: a systematic review[J]. Semin Ophthalmol, 2023, 38(8): 727-736. DOI: 10.1080/08820538.2023.2211134.
15. Gay AJ, Salmon ML, Windsor CE. Hering's law, the levators, and their relationship in disease states[J]. Arch Ophthalmol, 1967, 77(2): 157-160. DOI: 10.1001/archopht.1967.00980020159002.
16. Gorelick PB, Rosenberg M, Pagano RJ. Enhanced ptosis in myasthenia gravis[J]. Arch Neurol, 1981, 38(8): 531. DOI: 10.1001/archneur.1981.00510080093017.
17. Al-Haidar M, Benatar M, Kaminski HJ. Ocular myasthenia[J]. Neurol Clin, 2018, 36(2): 241-251. DOI: 10.1016/j.ncl.2018.01.003.
18. Elrod RD, Weinberg DA. Ocular myasthenia gravis[J]. Ophthalmol Clin North Am, 2004, 17(3): 275-309. DOI: 10.1016/j.ohc.2004.05.014.
19. Cheo SW, Low QJ, Mow WC, et al. Ice pack test-an useful bedside test to diagnose myasthenia gravis[J]. QJM, 2019, 112(5): 381-382. DOI: 10.1093/qjmed/hcy284.
20. Proudman W, Kleinig O, Lam L, et al. The icepack test in the diagnosis of myasthenia gravis with ocular features: a systematic review of diagnostic accuracy, technique, and economic utility[J]. Semin Ophthalmol, 2023, 38(7): 679-685. DOI: 10.1080/08820538.2023.2194984.
21. 彭丹濤, 許賢豪, 佘子瑜. 新斯的明試驗改良結果判定法研究[J]. 中國神經免疫學和神經病學雜志, 2007, 14(1): 1-3. DOI: 10.3969/j.issn.1006-2963.2007.01.001.Peng DT, Xu XH, She ZY. Study on revive criteria for neostigmine test[J]. Chin J Neuroimmunol& Neurol, 2007, 14(1): 1-3. DOI: 10.3969/j.issn.1006-2963.2007.01.001.
22. Benatar M. A systematic review of diagnostic studies in myasthenia gravis[J]. Neuromuscul Disord, 2006, 16(7): 459-467. DOI: 10.1016/j.nmd.2006.05.006.
23. Kupersmith MJ, Wolfe GI, Kaminski H. Ocular myasthenia gravis management[J/OL]. Front Neurol, 2025, 16: 1678151[2025-11-03]. https://pubmed.ncbi.nlm.nih.gov/41255784/. DOI: 10.3389/fneur.2025.1678151.
24. Li J, Zhu C, Zhao M, et al. OMGMed: advanced system for ocular myasthenia gravis diagnosis via eye image segmentation[J/OL]. Bioengineering (Basel), 2024, 11(6): 595[2024-06-11]. https://pubmed.ncbi.nlm.nih.gov/38927831/. DOI: 10.3390/bioengineering11060595.

Chinese Journal of Ocular Fundus Diseases

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