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Myasthenia Gravis

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Neuromuscular Disease

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Abstract

Myasthenia gravis (MG) is an autoimmune disorder in which antibodies are either directed against the muscle nicotinic acetylcholine receptor (nAChR) itself or against other postsynaptic targets such as the muscle specific kinase (MuSK) that indirectly reduce nAchR numbers. The disease may be limited to the extra-ocular muscles and eyelids (ocular myasthenia) or may affect limb, bulbar and respiratory muscles (generalized myasthenia). The relative diagnostic utility of electrophysiological tests and the presence of anti-acetylcholine receptor antibodies vary depending on whether the disease is ocular or generalized. MG is the prototype neurological auto-immune disorder in which both antigen and antibody have been identified, removal of antibody has been shown to lead to clinical improvement, and the disease can be recreated in mice following passive transfer of the auto-antibody. Understandably, therefore, immunosuppressive therapy forms the mainstay of disease-modifying treatment, with acetylcholinesterase inhibitors being reserved for symptomatic management.

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References

  1. Padua L, Stalberg E, LoMonaco M, Evoli A, Batocchi A, Tonali P. SFEMG in ocular myasthenia gravis diagnosis. Clin Neurophysiol 2000;111:1203–1207.

    Article  CAS  PubMed  Google Scholar 

  2. Lefvert A, Bergstrom K, Matell G, Osterman P, Pirskanen R. Determination of acetylcholine receptor antibody in myasthenia gravis: clinical usefulness and pathogenetic implications. J Neurol Neurosurg Psychiatry 1978;41:394–403.

    Article  CAS  PubMed  Google Scholar 

  3. Oey PL, Wieneke GH, Hoogenraad TU, van Huffelen AC. Ocular myasthenia gravis: the diagnostic yield of repetitive nerve stimulation and stimulated single fiber EMG of orbicularis oculi muscle and infrared reflection oculography. Muscle Nerve 1993;16:142–149.

    Article  CAS  PubMed  Google Scholar 

  4. Limburg P, The T, Hummel-Tappel E, Oosterhuis H. Anti-acetylcholine receptor antibodies in myasthenia gravis. Part 1: relation to clinical parameters in 250 patients. J Neurol Sci 1983;58:357–370.

    Article  CAS  PubMed  Google Scholar 

  5. Rouseev R, Ashby P, Basinski A, Sharpe J. Single fiber EMG in the frontalis muscle in ocular myasthenia:specificity and sensitivity. Muscle Nerve 1992;15:399–403.

    Article  CAS  PubMed  Google Scholar 

  6. Milone M, Monaco M, Evoli A, Servidei S, Tonali P. Ocular myasthenia: diagnostic value of single fiber EMG in the orbicularis oculi muscle. J Neurol Neurosurg Psychiatry 1993;56:720–721.

    Article  CAS  PubMed  Google Scholar 

  7. Howard FM, Lennon VA, Finley J, Matsumoto J, Elveback LR. Clinical correlations of antibodies that bind, block or modulate human acetylcholine receptors in myasthenia gravis. Ann NY Acad Sci 1987;505:526–538.

    Article  PubMed  Google Scholar 

  8. Ozdemir C, Young RR. The results to be expected from electrical testing in the diagnosis of myasthenia gravis. Ann NY Acad Sci 1976;274:203–222.

    Article  CAS  PubMed  Google Scholar 

  9. Mercelis R. Abnormal single-fiber electromyography in patients not having myasthenia. Risk for diagnostic confusion. Ann NY Acad Sci 2003;998:509–511.

    Article  PubMed  Google Scholar 

  10. Hoch W, McConville J, Helms S, Newsom-Davis J, Melms A, Vincent A. Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med 2001;7:365–368.

    Article  CAS  PubMed  Google Scholar 

  11. Scuderi F, Marino M, Colonna L, et al. Anti-P110 autoantibodies identify a subtype of “seronegative” myasthenia gravis with prominent oculobulbar symptoms. Lab Invest 2002;82:1139–1146.

    CAS  PubMed  Google Scholar 

  12. Sanders D, El-Salem K, Massey J, McConville J, Vincent A. Clinical aspects of MuSK antibody positive seronegative MG. Neurology 2003;60:1978–1980.

    Article  CAS  PubMed  Google Scholar 

  13. Papapetropoulos TH, Ellul J, Tsibri E. Development of generalized myasthenia gravis in patients with ocular myasthenia gravis. Arch Neurol 2003;60:1491–1492.

    Article  CAS  PubMed  Google Scholar 

  14. Mee J, Paine M, Byrne E, King J, Reardon K, O’Day J. Immunotherapy of ocular myasthenia gravis reduces conversion to generalized myasthenia gravis. J Neuroophthalmol 2003;23:251–255.

    Article  PubMed  Google Scholar 

  15. 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 

  16. Monsul NT, Patwa HS, Knorr AM, Lesser RL, Goldstein JM. The effect of prednisone on the progression from ocular to generalized myasthenia gravis. J Neurol Sci 2004;217:131–133.

    Article  CAS  PubMed  Google Scholar 

  17. Sommer N, Sigg B, Melms A, Weller M, Schepelmann K, Herzau V, Dichgans J. Ocular myasthenia gravis: response to long-term immunosuppressive treatment. J Neurol Neurosurg Psychiatry 1997;62:156–162.

    Article  CAS  PubMed  Google Scholar 

  18. Agius MA. Treatment of ocular myasthenia with corticosteroids: yes. Arch Neurol 2000;57(5):750–751.

    Article  CAS  PubMed  Google Scholar 

  19. Kaminski HJ, Daroff RB. Treatment of ocular myasthenia: steroids only when compelled. Arch Neurol 2000;57(5):752–753.

    Article  CAS  PubMed  Google Scholar 

  20. Hachinski V. Treatment of ocular myasthenia. Arch Neurol 2000;57(5):753.

    Article  CAS  PubMed  Google Scholar 

  21. Howard F, Duane D, Lambert, EH, Daube J. Alternate-day prednisone: preliminary report of a doubleblind controlled study. Ann NY Acad Sci 1976;274:596–607.

    Article  PubMed  Google Scholar 

  22. Sghirlanzoni A, Peluchetti D, Mantegazza R, Fiacchino F, Cornelio F. Myasthenia gravis: prolonged treatment with steroids. Neurology 1984;34:170–174.

    Article  CAS  PubMed  Google Scholar 

  23. Pascuzzi R, Coslett H, John TR. Long-term corticosteroid treatment of myasthenia gravis: report of 116 patients. Ann Neurol 1984;15:291–298.

    Article  CAS  PubMed  Google Scholar 

  24. Evoli A, Batocchi A, Palmisani M, Lon Monaco M, Tonali P. Long-term results of corticosteroid therapy in patients with myasthenia gravis. Eur Neurol 1992;32:37–43.

    Article  CAS  PubMed  Google Scholar 

  25. Evoli A, Tonali P, Bartoccioni E, Lo Monaco M. Ocuar myasthenia: diagnostic and therapeutic problems. Acta Neurol Scand 1988;77:31–35.

    Article  CAS  PubMed  Google Scholar 

  26. Seybold M, Drachman D. Gradually increasing doses of prednisone in myasthenia gravis. Reducing the hazards of treatment. N Engl J Med 1974;290:81–84.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  28. Lindberg C, Andersen O, Lefvert A. Treatment of myasthenia gravis with methylprednisolone pulse: a double-blind study. Acta Neurol Scand 1998;97:370–373.

    Article  CAS  PubMed  Google Scholar 

  29. A randomised clinical trial comparing prednisone and azathioprine in myasthenia gravis. Results of the second interim analysis. Myasthenia Gravis Clinical Study Group. J Neurol Neurosurg Psychiatry 1993;56:1157–1163.

    Google Scholar 

  30. Bromberg M, Wald J, Forshew D, Feldman E, Alberts J. Randomized trial of azathioprine or prednisone for initial immunosuppressive treatment of myasthenia gravis. J Neurol Sci 1997;150:59–62.

    Article  CAS  PubMed  Google Scholar 

  31. Palace J, Newsom-Davis J, Lecky B. A randomized double-blind trial of prednisolone alone or with azathioprine in myasthenia gravis. Myasthenia Gravis Study Group. Neurology 1998;50:1778–1783.

    Article  CAS  PubMed  Google Scholar 

  32. Tindall R, Rollins J, Phillips J, Greenlee R, Wells L, Belendiuk G. Preliminary results of a doubleblind, randomized, placebo-controlled trial of cyclosporine in myasthenia gravis. N Engl J Med 1987;316:719–724.

    Article  CAS  PubMed  Google Scholar 

  33. Tindall R, Phillips J, Rollins J, Wells L, Hall K. A clinical therapeutic trial of cyclosporine in myasthenia gravis. Ann NY Acad Sci 1993;681:539–551.

    Article  CAS  PubMed  Google Scholar 

  34. Cos L, Mankodi AK, Tawil R, Thornton CA. Mycophenolate mofetil (MyM) is safe and well-tolerated in myasthenia gravis (MG). Neurology 2000;54:A137.

    Google Scholar 

  35. Ciafaloni E, Massey J, Tucker-Lipscomb B, Sanders D. Mycophenolate mofetil for myasthenia gravis: an open-label pilot study. Neurology 2001;56:97–99.

    Article  CAS  PubMed  Google Scholar 

  36. Chaudhry V, Cornblath D, Griffin J, O’Brien R, Drachman D. Mycophenolate mofetil: A safe and promising immunosuppressant in neuromuscular diseases. Neurology 2001;56:94–96.

    Article  CAS  PubMed  Google Scholar 

  37. Meriggioloi M, Ciafaloni E, Al-Hayk K, et al. Mycophenolate mofetil for myasthenia gravis. An analysis of efficacy, safety and tolerability. Neurology 2003;61:1438–1440.

    Article  Google Scholar 

  38. Meriggioloi MN, Rowin J, Richman JG, Leurgans S. Mycophenolate mofetil for myasthenia gravis. A double-blind, placebo-controlled pilot study. Ann NY Acad Sci 2003;998:494–499.

    Article  Google Scholar 

  39. Gajdos P, Simon N, de Rohan-Chabot P, Raphael JC, Goulon M. Long-term effects of plasma exchanges in myasthenia. Results of a randomized study. Presse Med 1983;12:939–942.

    CAS  PubMed  Google Scholar 

  40. Gajdos P, Chevret S, Toyka K. Plasma exchange for myasthenia gravis. Cochrane Database Syst Rev 2002;4:CD002275.

    Google Scholar 

  41. Kornfeld P, Ambinder E, Mittag T, et al. Plasmapheresis in refractory generalized myasthenia gravis. Arch Neurol 1981;38:478–481.

    Article  CAS  PubMed  Google Scholar 

  42. The utility of therapeutic plasmapheresis for neurological disorders. NIH Consensus Development. JAMA 1986;256:1333–1337.

    Google Scholar 

  43. Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C. Clinical trial of plasma exchange and high dose immunoglobulin in myasthenia gravis. Myasthenia Gravis Clinical Study Group. Ann Neurol 1997;41:789–796.

    Article  CAS  PubMed  Google Scholar 

  44. Ronager J, Ravnborg M, Hermansen I, Vorstrup S. Immunoglobulin treatment versus plasma exchange in patients with chronic moderate to severe myasthenia gravis. Artif Organs 2001;25:967–973.

    Article  CAS  PubMed  Google Scholar 

  45. Wolfe GI, Barohn RJ, Foster BM, et al. Randomized, controlled trial of intravenous immunogloublin in myasthenia gravis. Muscle Nerve 2002;26:549–552.

    Article  CAS  PubMed  Google Scholar 

  46. Gronseth G, Baroh R. Practice parameter: thymectomy for autoimmune myasthenia gravis (an evidencebased review). Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2000;55:636–643.

    Article  PubMed  Google Scholar 

  47. Lanska D. Indications for thymectomy in myasthenia gravis. Neurology 1990;40:1828–1829.

    Article  CAS  PubMed  Google Scholar 

  48. Oosterhuis HJ. The natural course of myasthenia gravis: a long term follow-up study. J Neurol Neurosurg Psychiatry 1989;52:1121–1127.

    Article  CAS  PubMed  Google Scholar 

  49. Grob D, Brunner NG, Namba T. The natural course of myasthenia gravis and effect of therapeutic measures. Ann NY Acad Sci 1981;377:652–669.

    Article  CAS  PubMed  Google Scholar 

  50. Bever CT, Aquino AV, Penn AS, Lovelace RE, Rowland LP. Prognosis of ocular myasthenia. Ann Neurol 1983;14:516–519.

    Article  PubMed  Google Scholar 

  51. Cosi V, Romani A, Lombardi M, et al. Prognosis of myasthenia gravis: a retrospective study of 380 patients. J Neurol 1997;244:548–555.

    Article  CAS  PubMed  Google Scholar 

  52. Mantegazza R, Beghi E, Pareyson D, et al. A multicenter follow-up study of 1152 patients with myasthenia gravis in Italy. J Neurol 1990;237:339–344.

    Article  CAS  PubMed  Google Scholar 

  53. Beghi E, Antozzi C, Batocchi AP, et al. Prognosis of myasthenia gravis: a multicenter follow-up study of 844 patients. J Neurol Sci 1991;106:213–220.

    Article  CAS  PubMed  Google Scholar 

  54. Mantegazza R, Baggi F, Antozzi C, et al. Myasthenia gravis (MG): epidemiological data and prognostic factors. Ann NY Acad Sci 2003;998:413–423.

    Article  PubMed  Google Scholar 

  55. Christensen P, Jensen T, Tsiropoulos I, et al. Mortality and survival in myasthenia gravis: a Danish population based study. J Neurol Neurosurg Psychiatry 1998;64:78–83.

    Article  CAS  PubMed  Google Scholar 

  56. Behan P, Shakir R, Simpson J, Burnett A, Allan T, Haase G. Plasma-exchange combined with immunosuppressive therapy in myasthenia gravis. Lancet 1979;2:438–440.

    Article  CAS  PubMed  Google Scholar 

  57. Dau PC. Response to plasmapheresis and immunosuppressive drug therapy in sixty myasthenia gravis patients. Ann NY Acad Sci 1981;377:700–708.

    Article  CAS  PubMed  Google Scholar 

  58. Perlo V, Shahani B, Huggins C, Hunt J, Kosinski K, Potts F. Effect of plasmapheresis in myasthenia gravis. Ann NY Acad Sci 1981;377:709–724.

    Article  CAS  PubMed  Google Scholar 

  59. Olarte MR, Schoenfeldt RS, Penn AS, Lovelace RE, Rowland LP. Effect of plasmapheresis in myasthenia gravis 1978–1980. Ann NY Acad Sci 1981;377:725–728.

    Article  CAS  PubMed  Google Scholar 

  60. Antozzi C, Gemma M, Regi B, et al. A short plasma exchange protocol is effective in severe myasthenia gravis. J Neurol 1991;238:103–107.

    Article  CAS  PubMed  Google Scholar 

  61. Chiu HC, Chen WH, Yeh JH. The six year experience of plasmapheresis in patients with myasthenia gravis. Ther Apher 2000;4:291–295.

    Article  CAS  PubMed  Google Scholar 

  62. Yeh J, Chiu H. Double filtration plasmapheresis in myasthenia gravis-analysis of clinical efficacy and prognostic parameters. Acta Neurol Scand 1999;100:305–309.

    Article  CAS  PubMed  Google Scholar 

  63. Kornfeld P, Ambinder E, Papatestas A, Bender A, Genkins G. Plasmapheresis in myasthenia gravis: controlled study. Lancet 1979;2:629.

    Article  CAS  PubMed  Google Scholar 

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  1. Department of Neurology, Emory University School of Medicine, Atlanta, GA

    Michael Benatar MBChB, MS, DPhil

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  1. Michael Benatar MBChB, MS, DPhil
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  1. Department of Neurology, Emory University School of Medicine, Atlanta, GA

    Michael Benatar MBChB, MS, DPhil

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© 2006 Humana Press Inc., Totowa, NJ

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Benatar, M. (2006). Myasthenia Gravis. In: Benatar, M. (eds) Neuromuscular Disease. Humana Press. https://doi.org/10.1007/978-1-59745-106-2_17

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  • DOI: https://doi.org/10.1007/978-1-59745-106-2_17

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