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Ocular myasthenia gravis

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Opinion statement

Myasthenia gravis (MG) is an autoimmune disorder characterized clinically by proximal weakness and bulbar symptoms and pathologically by damage to the post-synaptic membrane at the neuromuscular junction. Ocular myasthenia gravis (ocular MG) is a form of myasthenia gravis whereby the patients’ weakness is limited to the muscles of the eyes and eyelids (levator palpebrae superioris). Although not life-threatening, the limitations posed by ocular myasthenia gravis can prove disabling and distressing to patients. Acetylcholinesterase inhibitors such as pyridostigmine or neostigmine are the preferred first-line treatment for ocular myasthenia gravis, with mild cases requiring no additional intervention. However, in moderate or severe cases, treatment must be tailored to the needs and desires of the patient. Intravenous immunoglobulin, although costly, is safe and effective at treating MG. Corticosteroids are effective at reducing or eliminating symptoms and may modify the longterm course of the illness. Steroid-sparing agents such as azathioprine and mycophenolate mofetil are reasonably safe and well-tolerated alternatives to steroids. Surgical interventions such as strabismus surgery and eyelid suspension serve to correct impairments refractory to medical management. Thymectomy, although less frequently recommended, is a reasonable consideration, especially for young adults, given the potential for long-term benefit.

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References and Recommended Reading

  1. Robertson NP, Deans J, Compston DAS: Myasthenia gravis: a population based epidemiological study in Cambridgeshire, England. J Neurol Neurosurg Psychiatry 1998, 65:492–496.

    PubMed  CAS  Google Scholar 

  2. Grob D, Arsura E, Brunner NG, Namba T: The course of myasthenia gravis and therapies affecting outcome. Ann N Y Acad Sci 1987, 505:472–499.

    Article  PubMed  CAS  Google Scholar 

  3. Mantegazza R, Baggi F, Antozzi C, et al.: Myasthenia gravis: epidemiological data and prognostic factors. Ann N Y Acad Sci 2003, 998:413–423.

    Article  PubMed  Google Scholar 

  4. Padua L, Stalberg E, LoMonaco M, et al.: SFEMG in ocular myasthenia gravis diagnosis. Clin Neurophysiol 2000, 111:1203–1207.

    Article  PubMed  CAS  Google Scholar 

  5. Jaretzki A, Barohn R, Ernstoff R, et al.: Myasthenia gravis: Recommendations for clinical research standards: task force of the medical scientific advisory board of the myasthenia gravis foundation of America. Neurology 2000, 55:16–23.

    PubMed  Google Scholar 

  6. Bever CT, Aquino AV, Penn AS, et al.: Prognosis of ocular myasthenia. Ann Neurol 1983, 14:516–519.

    Article  PubMed  Google Scholar 

  7. Beekman R, Kuks JBM, Oosterhuis HJGH: Myasthenia gravis: diagnosis and follow-up of 100 consecutive patients. J Neurol 1997, 244:112–118.

    Article  PubMed  CAS  Google Scholar 

  8. Sanders DB: The electrodiagnosis of myasthenia gravis. Ann N.Y. Acad Sci 1987, 505:539–555.

    Article  PubMed  CAS  Google Scholar 

  9. Batocchi A, Majolini L, Evoli A, et al.: Course and treatment of myasthenia gravis during pregnancy. Neurology 1999, 52:447–452.

    PubMed  CAS  Google Scholar 

  10. Kupersmith MJ, Moster M, Bhuiyan S, et al.: Beneficial effects of corticosteroids on ocular myasthenia gravis. Arch Neurol 1996, 53:802–804.

    PubMed  CAS  Google Scholar 

  11. Kupersmith MJ, Latkany R, Homel P: Development of generalized disease at 2 years in patients with ocular myasthenia gravis. Arch Neurol 2003, 60:243–248.

    Article  PubMed  Google Scholar 

  12. Monsul NT, Patwa HS, Knorr AM, et al.: The effect of prednisone on the progression from ocular to generalized myasthenia gravis. J Neurol Sci 2004, 217:131–133.

    Article  PubMed  CAS  Google Scholar 

  13. Gajdos P, Chevret S, Clair B: Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Ann Neurol 1997, 41:789–796.

    Article  PubMed  CAS  Google Scholar 

  14. Palace J, Newson-Davis J, Lecky B: A randomized doubleblind trial of prednisolone alone or with azathioprine in myasthenia gravis. Neurology 1998, 50:1778–1783.

    PubMed  CAS  Google Scholar 

  15. Meriggioli MN, Ciafaloni E, Al-Hayk KA, et al.: Mycophenolate mofetil for myasthenia gravis: an analysis of efficacy, safety, and tolerability. Neurology 2003, 61:1438–1440.

    PubMed  CAS  Google Scholar 

  16. Chaudhry V, Conblath DR, Griffin JW, et al.: Mycophenolate mofetil: A safe and promising immunosuppressant in neuromuscular diseases. Neurology 2001, 56:94–96.

    PubMed  CAS  Google Scholar 

  17. Ciafaloni E, Massey JM, Tucker-Lipscomb B, Sanders DB: Mycophenolate mofetil for myasthenia gravis: An open label pilot study. Neurology 2001, 56:97–99.

    PubMed  CAS  Google Scholar 

  18. Meriggioli MN, Rowin J, Richman JG, Leurgans S: Mycophenolate mofetil for myasthenia gravis: a doubleblind, placebo-controlled pilot study. Ann N Y Acad Sci 2003, 998:494–499.

    Article  PubMed  CAS  Google Scholar 

  19. Tinsdall R, Rollins J, Phillips JT, et al.: Preliminary results of a double-blind, randomized, placebocontrolled trial of cyclosporine in myasthenia gravis. N Engl J Med 1987, 316:719–724.

    Article  Google Scholar 

  20. Acheson JF, Elston JS, Lee JP, Fells P: Extraocular muscle surgery in myasthenia gravis. Br J Ophthalmol 1991, 75:232–235.

    PubMed  CAS  Google Scholar 

  21. Morris OC, O’Day J: Strabismus surgery in the management of diplopia caused by myasthenia gravis. Br J Ophthalmol 2004, 88:832.

    PubMed  CAS  Google Scholar 

  22. Roberts PF, Venuta F, Rendina E, et al.: Thymectomy in the treatment of ocular myasthenia gravis. J Thorac Cardiovasc Surg 2001, 122:562–568.

    Article  PubMed  CAS  Google Scholar 

  23. Nakamura H, Taniguchi Y, Suzuki Y, et al.: Delayed remission after thymectomy for myasthenia gravis of the purely ocular type. J Thorac Cardiovasc Surg 1996, 112:371–375.

    Article  PubMed  CAS  Google Scholar 

  24. Mantegazza R, Baggi F, Bernasconi P, et al.: Videoassisted thoracoscopic extended thymectomy and extended transsternal thymectomy (T-3b) in non-thymomatous myasthenia gravis patients: remission after 6 years of follow-up. J Neurol Sci 2003, 212:31–36. This prospective, nonrandomized study of a cohort of 206 patients with myasthenia gravis including a small number of patients with ocular MG, observed patients treated with thymectomy during 6 years. Patients who had thorascopic procedures seemed to do as well as those who had transsternal operations.

    Article  PubMed  Google Scholar 

  25. Evoli A, Minisci C, Di Schino C, et al.: Thymoma in patients with MG: characteristics and long-term outcome. Neurology 2002, 59:1844–1850.

    PubMed  CAS  Google Scholar 

  26. De Perrot M, Bril V, McRae K, Keshavjee S: Impact of minimally invasive trans-cervical thymectomy on outcome in patients with myasthenia gravis. Eur J Cardiothorac Surg 2003, 24:677–683.

    Article  PubMed  Google Scholar 

  27. Neuromuscular Homepage website: Accessed 09/01/04 http://www.neuro.wustl.edu/neuromuscular/antibody/solumedrolrx.htm * [Alan Pestronk’s Neuromuscular Homepage is undoubtedly the most concise and timely online resource for neuromuscular diseases.]

  28. Komiyama A, Arai H, Masanori K, Hirayama K: Extraocular muscle response to high dose intravenous methylprednisolone in myasthenia gravis. J Neurol Neurosurg Psychiatry 2000, 68:214–217. This small prospective series using intravenous methylprednisolone (1g daily for 3 days weekly for 2 to 3 weeks) produced notable improvement in symptoms of patients with ocular and generalized MG.

    Article  PubMed  CAS  Google Scholar 

  29. Arsura E, Brunner NG, Namba T, Grob D: High-dose intravenous methylprednisolone in myasthenia gravis. Arch Neurol 1985, 42:1149–1153.

    PubMed  CAS  Google Scholar 

  30. Lindberg C, Andersen O, Lefvert AK: Treatment of myasthenia gravis with methylprednisolone pulse: a double-blind study. Acta Neurol Scand 1998, 97:370–373. This small but well-done study compared the effect of a single treatment of intravenous methylprednisolone (4 g during 2 days) to placebo in 20 patients with MG. The authors found a statistically significant improvement in strength but no serious adverse reactions in the treatment group.

    Article  PubMed  CAS  Google Scholar 

  31. Kim J, Hwang J, Hwang YS, Kim KJ, Chae J: Childhood ocular myasthenia gravis. Ophthalmology 2003, 110:1458–1462.

    Article  PubMed  Google Scholar 

  32. Lindner A, Schalke B, Toyka KV: Outcome in juvenileonset myasthenia gravis: a retrospective study with long-term follow-up of 79 patients. J Neurol 1997, 244:515–520.

    Article  PubMed  CAS  Google Scholar 

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Porter, N.C., Salter, B.C. Ocular myasthenia gravis. Curr Treat Options Neurol 7, 79–88 (2005). https://doi.org/10.1007/s11940-005-0009-z

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