Ocular myasthenia gravis — clinical diagnosis and treatment

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Abstract

The ocular myasthenia gravis has a variable clinical presentation and imitates many diseases with ocular region damaged. The diagnosis of ocular myasthenia gravis is complicated due to low sensitivity of the diagnostic approaches. The article introduces the features of the examination of patients with the ocular myasthenia gravis. The article describes the study methodology and interpretation of Simpson, Gorelick, Cogan`s lid twitch sign, rest and sleep tests, ice test. The article considered features of the treatment of the disease in order to achieve remission and prevent the generalization of the process. Timely diagnosis and optimal choice of treatment strategy can significantly change the quality of patient’s life and influence the subsequent prognosis of the disease.

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About the authors

Irina F. Khafizova

Kazan State Medical University

Author for correspondence.
Email: sclerdissem@mail.ru
ORCID iD: 0000-0003-2561-7456
SPIN-code: 6210-5860
Russian Federation, 420012, Kazan, Butlerov str., 49

Adelia R. Galiavieva

City clinical hospital №7

Email: gal-adelya@mail.ru
SPIN-code: 4337-2858
Russian Federation, 420103, Kazan, Marshal Chuikov str., 54

References

  1. Meriggioli M.N., Sanders D.B. Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol. 2009; 8: 475–490.
  2. Smith S.V., Lee A.G. Update on ocular myasthenia gravis. Neurol. Clin. 2017; 35: 115–123. doi: 10.1016/j.ncl.2016.08.008.
  3. Karni A., Asmail A., Drory V.E. et al. Characterization of patients with ocular myasthenia gravis — A case series. ENeurologicalSci. 2016; 4: 30–33. doi: 10.1016/j.ensci.2016.04.005.
  4. Kaminski H.J., Kusner L.L. Myasthenia gravis and related disorders. 3rd ed. Humana Press, Cham, Part of Springer Nature. 2018; 353 p. doi: 10.1007/978-3-319-73585-6.
  5. Koneczny I., Herbst R. Myasthenia gravis: Pathogenic effects of autoantibodies on neuromuscular architecture. Cells. 2019; 8: 671. doi: 10.3390/cells8070671.
  6. Okun M.S., Charriez C.M., Bhatti M.T. et al. Tensilon and the diagnosis of myasthenia gravis: are we using the tensilon test too much? Neurologist. 2001; 7: 295–299.
  7. Sanders D.B., Howard J.F. Single fiber EMG in myasthenia gravis. Muscle Nerve. 1986; 9: 809–819.
  8. Selvan V.A. Single-fiber EMG: A review. Ann. Indian Acad. Neurol. 2011; 14 (1): 64–67. doi: 10.4103/0972-2327.78058.
  9. Sanders D.B., Wolfe G.I., Benatar M. et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology. 2016; 87: 419–425.
  10. Nair A.G., Patil-Chhablani P., Venkatramani D.V. et al. Ocular myasthenia gravis: a review. Indian J. Ophthalmol. 2014; 62 (10): 985–991. doi: 10.4103/0301-4738.145987.
  11. Klemencic S.A., Condie J., Mei D. Ocular and generalized myasthenia gravis a teaching case series. Optometric Education. 2014; 39 (3): 129–137.
  12. Daroff R.B. The office Tensilon test for ocular myasthenia gravis. Arch. Neurol. 1986; 43: 843–844.
  13. Fortin E., Cestan D.M., Weinberg D.H. Ocular myasthenia gravis: an update on diagnosis and treatment. Curr. Opin. Ophthalmol. 2018; 29: 477–484.
  14. Apinyawasisuk S., Chongpison Y., Thitisaksakul C. et al. Factors affecting generalization of ocular myasthenia gravis in patients with positive acetylcholine receptor antibody. Am. J. Ophthalmol. 2020; 209: 10–17. doi: 10.1016/j.ajo.2019.09.019. 2019.
  15. Schmidt D. Signs in ocular myasthenia and pseudomyasthenia. Differential diagnostic criteria. A clinical review. Neuro-ophthalmology. 1995; 15 (1): 21–58.
  16. Sommer N., Melms A., Weller M. et al. Ocular myasthenia gravis. A critical review of clinical and pathophysiological aspects. Doc. Ophthalmol. 1993; 84: 309–333.
  17. De Meel R.H.P., Raadsheer W.F., van Zwet E.W. et al. Ocular weakness in myasthenia gravis changes in affected muscles are a distinct clinical feature. J. Neuromusc. Dis. 2019; 6: 369–376. doi: 10.3233/JND-190407.
  18. Nagia L., Lemos J., Abusamra K. et al. Prognosis of ocular myasthenia gravis: retrospective multicenter analysis. Ophthalmology. 2015; 122 (7): 1517–1521. doi: 10.1016/j.ophtha.2015.03.010.
  19. Worley M.W., Gal O., Anderson R.L. et al. Eye dominance and Hering’s law effect on bilateral blepharotosis repair. Ophthal. Plast. Reconstr. Surg. 2013; 29: 437–439. doi: 10.1097/IOP.0b013e31829d02e8.
  20. Sethi K.D., Rivner M.H., Swift T.R. Ice pack test for myasthenia gravis. Neurology. 1987; 37: 1383–1385.
  21. Golnik K.C., Pena R., Lee A.G. et al. An ice test for the diagnosis of myasthenia gravis. Ophthalmology. 1999; 106: 1282.
  22. Ellis F.D., Hoyt C.S., Ellis F.J. et al. Extraocular muscle responses to orbital cooling (ice test) for ocular myasthenia gravis diagnosis. J. AAPOS. 2000; 4 (5): 271–281. doi: 10.1067/mpa.2000.106204.
  23. Odel J., Winterkorn J., Behrens M. The sleep test for myasthenia gravis — a safe alternative to Tensilon. J. Clin. Neuro-ophthalmol. 1991; 11: 288–292.
  24. Li Z.Y. China guidelines for the diagnosis and treatment of myasthenia gravis. Neuroimmunol. Neuroinflammation. 2016; 3: 1–9. doi: 10.20517/2347-8659.2015.60.
  25. Kaminski H.J., Maas E., Spiegel P. et al. Why are eye muscles frequently involved in myasthenia gravis? Neurology. 1990; 40: 1663–1669.
  26. Kupersmith M.J., Ying G. Ocular motor dysfunction and ptosis in ocular myasthenia gravis: effects of treatment. Br. J. Ophthalmol. 2005; 89 (10): 1330–1334. doi: 10.1136/bjo.2004.063404.
  27. Werner P., Kiechl S., Thaler C. et al. A relapsing-remitting type of ocular myasthenia gravis without typical muscle fatiguability. J. Neurol. Neurosurg. Psychiatry. 2002; 73 (2): 205. doi: 10.1136/jnnp.73.2.205.
  28. Kupersmith M.J., Latkany R., Homel P. Development of generalized disease at 2 years in patients with ocular myasthenia gravis. Arch. Neurol. 2003; 60 (2): 243–248.
  29. Li F., Hotter B., Swierzy M. et al. Generalization after ocular onset in myasthenia gravis: a case series in Germany. J. Neurol. 2018; 265 (12): 2773–2782.
  30. Li M., Ge F., Guo R. et al. Do early prednisolone and other immunosuppressant therapies prevent generalization in ocular myasthenia gravis in Western populations: a systematic review and meta-analysis. Ther. Adv. Neurol. Dis. 2019; 12: 1–8.
  31. Benatar M., McDermott M.P., Sanders D.B. et al. Efficacy of prednisone for the treatment of ocular myasthenia (EPITOME): a randomized controlled trial. Muscle Nerve. 2016; 53 (3): 363–359. doi: 10.1002/mus.24769.
  32. Melson A.T., McClelland C.M., Lee M.S. Ocular myasthenia gravis: updates on an elusive target. Curr. Opin. Neurol. 2020; 33 (1): 55–61. doi: 10.1097/WCO.0000000000000775.
  33. Haines S.R., Thurtell M.J. Treatment of ocular myasthenia gravis. Curr. Treat. Options Neurol. 2012; 14: 103–112. doi: 10.1007/s11940-011-0151-8.
  34. Kerty E., Elsais A., Argov Z. et al. EFNS/ENS Guidelines for the treatment of ocular myasthenia. Eur. J. Neurol. 2014; 21: 687–693.
  35. Morris O.C., O’Day J. Strabismus surgery in the management of diplopia caused by myasthenia gravis. Br. J. Ophthalmol. 2004; 88: 832.

Supplementary files

Supplementary Files
Action
1. JATS XML
2. Fig 1. Patient 63 years old, diagnosis "Myasthenia gravis, generalized form, class 3a". A. Rough blepharoptosis on the right, pseudoretraction of the upper eyelid on the left. B. Gorelik test: resolution of pseudoretraction of the upper eyelid

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Copyright (c) 2021 Khafizova I.F., Galiavieva A.R.

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