Current Treatment Options in Neurology

, Volume 12, Issue 3, pp 231–243 | Cite as

Myasthenia Gravis

Neuroimmunology

Opinion statement

Treatment of patients with acquired (autoimmune) myasthenia gravis should rely on evidence-based therapeutic choices, taking into account the individual’s needs according to disease severity (mild to severe), extent (ocular or generalized), comorbidities (including other autoimmune diseases, infections, thymoma, and pregnancy), age, iatrogenic factors (the risks and benefits of therapy), patient autonomy and quality of life, financial burden to the patient, and associated health care costs. Therapy is aimed at managing symptoms by improving neuromuscular junction transmission (cholinesterase inhibitors) and/or modifying the underlying immunopathogenetic cause of acquired myasthenia gravis via immunosuppression or immunomodulation. Myasthenic patients with operable thymoma should be referred for surgery and closely followed up for tumor recurrence. A concerted international effort is addressing treatment recommendations for thymectomy in myasthenic patients with no radiologic evidence of thymoma who are positive for circulating acetylcholine receptor antibodies. There is a lack of evidence-based treatment guidelines for both acute and long-term management of ocular myasthenia. Acute management of myasthenic crisis requires intensive monitoring of the patient and institution of an efficient and safe treatment such as plasma exchange. Patient education is essential to a comprehensive long-term treatment plan.

Notes

Disclosure

No potential conflicts of interest relevant to this article were reported.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Keesey JC: Myasthenia Gravis: An Illustrated History. Roseville: Publishers Design Group; 2002.Google Scholar
  2. 2.
    Ohry A: Dr. Lazar Remen (1907-74): a forgotten pioneer in the treatment of myasthenia gravis. J Med Biogr 17(2):73–74.Google Scholar
  3. 3. •
    Jaretzki A, Sonett JR: Thymectomy for non-thymomatous myasthenia gravis. In Myasthenia Gravis and Related Disorders, edn 2. Edited by Kaminski HJ. New York: Humana Press; 2008:185–208. This chapter presents a detailed analysis of current expert opinion and controversies regarding thymectomy.Google Scholar
  4. 4.
    Agius MA, Richman DP, Vincent A: Autoantibody testing in the diagnosis and management of autoimmune disorders of neuromuscular transmission and related disorders. In Myasthenia Gravis and Related Disorders. By Kaminski HJ. Totowa: Humana; 2003:143–156.Google Scholar
  5. 5. •
    Leite MI, Jacob S, Viegas S, et al.: IgG1 antibodies to acetylcholine receptors in “seronegative” myasthenia gravis. Brain 2008, 131(7):1940–1952. This paper proves the presence of low-affinity antibodies in “seronegative” MG and their role in complement activation at the neuromuscular junction.Google Scholar
  6. 6. •
    Kaminski HJ: Treatment of myasthenia gravis. In Myasthenia Gravis and Related Disorders, edn 2. Edited by Kaminski HJ. New York: Humana Press; 2008:157–173. This chapter presents a concise overview of treatment options in autoimmune MG.Google Scholar
  7. 7.
    Pasnoor M, Wolfe GI, Nations S, et al.: Clinical findings in MuSK-antibody positive myasthenia gravis: A U.S. experience. Muscle Nerve 2010, 41(3):370–374.CrossRefPubMedGoogle Scholar
  8. 8.
    Stickler DE, Massey JM, Sanders DB: MuSK-antibody positive myasthenia gravis: clinical and electrodiagnostic patterns. Clin Neurophysiol 2005, 116(9):2065–2068.CrossRefPubMedGoogle Scholar
  9. 9.
    Romi F, Skeie GO, Gilhus NE: Striational antibodies in myasthenia gravis: reactivity and possible clinical significance. Arch Neurol 2005, 62(3):442–446.CrossRefPubMedGoogle Scholar
  10. 10.
    Lisak RP: Myasthenia gravis. Curr Treat Options Neurol 1999, 1:239–249.CrossRefPubMedGoogle Scholar
  11. 11.
    Meriggioli MN: Myasthenia gravis: immunopathogenesis, diagnosis, and management. Continuum 2009, 15(1):34–62.Google Scholar
  12. 12.
    Hart IK, Sathasivam S, Sharshar T: Immunosuppressive agents for myasthenia gravis. Cochrane Database Syst Rev 2007, 4:CD005224.PubMedGoogle Scholar
  13. 13.
    Lisak RP: Myasthenia gravis. In Current Therapy in Neurological Disease. By Johnson RT, Griffin JW, McArthur JC. St. Louis: Mosby; 2002:407–409.Google Scholar
  14. 14. •
    Jani-Acsadi A, Lisak RP: Myasthenic crisis: guidelines for prevention and treatment. J Neurol Sci 2007, 261(1–2):127–133. This recent review of expert opinion on pharmacologic and interventional treatment options for myasthenic crisis emphasizes the role of plasma pheresis.Google Scholar
  15. 15.
    Skeie GO, Apostolski S, Evoli A, et al.: Guidelines for the treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol 2006, 13(7):691–699.CrossRefPubMedGoogle Scholar
  16. 16. •
    Punga AR, Stalberg E: Acetylcholinesterase inhibitors in myasthenia gravis: to be or not to be? Muscle Nerve 2009, 39:724–728. This is a concise review of the mechanisms, therapeutic effects, and contraindications of newer and older acetylcholinesterase inhibitors in MG.Google Scholar
  17. 17.
    Kulaksizoglu IB: Mood and anxiety disorders in patients with myasthenia gravis: etiology, diagnosis and treatment. CNS Drugs 2007, 21:473–481.CrossRefPubMedGoogle Scholar
  18. 18.
    Hoff JM, Daltveit AK, Gilhus NE: Myasthenia gravis in pregnancy and birth: identifying risk factors, optimizing care. Eur J Neurol 2007, 14:38–43.CrossRefPubMedGoogle Scholar
  19. 19.
    Ciafaloni E, Massey JM: The management of myasthenia gravis in pregnancy. Semin Neurol 2004, 24:95–100.CrossRefPubMedGoogle Scholar
  20. 20.
    Mehndiratta MM, Kuntzer T, Pandey S: Anticholinesterase treatment for myasthenia gravis. Cochrane Database Syst Rev 2008, 1:CD006986.Google Scholar
  21. 21.
    Schneider-Gold C, Gajdos P, Toyka KV, Hohlfeld RR: Corticosteroids for myasthenia gravis. Cochrane Database Syst Rev 2005, 2:CD002828.PubMedGoogle Scholar
  22. 22.
    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(6):1778–1783.PubMedGoogle Scholar
  23. 23.
    Conti-Fine B: Myasthenia Gravis: The Immunobiology of an Autoimmune Disease. Austin: Landes Bioscience; 1997.Google Scholar
  24. 24.
    Ciafaloni E, Nikhar NK, Massey JM, Sanders DB: Retrospective analysis of the use of cyclosporine in the treatment of myasthenia gravis. Neurology 2000, 55:448–450.PubMedGoogle Scholar
  25. 25.
    Sanders DB, Hart IK, Mantegazza R, et al.: An international, phase III, randomized trial of mycophenolate mofetil in myasthenia gravis. Neurology 2008, 71(6):400–406.CrossRefPubMedGoogle Scholar
  26. 26.
    Benatar M, Kaminski HJ: Quality Standards Subcommittee of the American Academy of Neurology: Evidence report: the medical treatment of ocular myasthenia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2007, 68(24):2144–2149.CrossRefPubMedGoogle Scholar
  27. 27.
    Ponseti JM, Azem J, Fort JM, et al.: Long-term results of tacrolimus in cyclosporine—and prednisone-dependent myasthenia gravis. Neurology 2005, 64:1641–1643.CrossRefPubMedGoogle Scholar
  28. 28.
    Ponseti JM, Gamez J, Azem J, et al.: Tacrolimus for myasthenia gravis: a clinical study of 212 patients. Ann NY Acad Sci 2008, 1132:254–263.CrossRefPubMedGoogle Scholar
  29. 29.
    The utility of therapeutic plasmapheresis for neurological disorders. NIH Consens Statement 1986, 6(4):1–7.Google Scholar
  30. 30.
    Gajdos P, Chevret S, Toyka K: Plasma exchange for myasthenia gravis. Cochrane Database Syst Rev 2002, 4:CD002275.PubMedGoogle Scholar
  31. 31.
    Gajdos P, Chevret S, Toyka K: Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev 2008, 1:CD002277.PubMedGoogle Scholar
  32. 32.
    Qureshi AI, Choudhry MA, Akbar MS, et al.: Plasma exchange versus intravenous immunoglobuline treatment in myasthenic crisis. Neurology 1999, 52:629–632.PubMedGoogle Scholar
  33. 33.
    Evoli A, Minisci C, Di Schino C, et al.: Thymoma in patients with MG: characteristics and long-term outcome. Neurology 2002, 59:1844–1850.PubMedGoogle Scholar
  34. 34.
    Cea G, Benatar M, Verdugo RJ, Salinas RA: Thymectomy for non-thymomatous myasthenia gravis. Cochrane Database Syst Rev 2009, 4:CD008111.Google Scholar
  35. 35.
    Wagner AJ, Cortes RA, Strober J, et al.: Long-term follow-up after thymectomy for myasthenia gravis: thoracoscopic vs open. J Pediatr Surg 2006, 41(1):50–54.CrossRefPubMedGoogle Scholar
  36. 36.
    Rückert JA, Ismail M, Swierzy M, et al.: Thoracoscopic thymectomy with the da Vinci robotic system for myasthenia gravis. Ann NY Acad Sci 2008, 1132:329–335.CrossRefPubMedGoogle Scholar
  37. 37.
    Essaa M, El-Medanya Y, Hajjara W, et al.: Maximal thymectomy in children with myasthenia gravis. Eur J Cardiothorac Surg 2003, 24(2):187–191.CrossRefGoogle Scholar
  38. 38.
    Sonett JR, Jaretzki A: Thymectomy for nonthymomatous myasthenia gravis: a critical analysis. Ann N Y Acad Sci 2008, 1132:315–328.CrossRefPubMedGoogle Scholar
  39. 39.
    Prokakis C, Koletsis E, Salakou S, et al.: Modified maximal thymectomy for myasthenia gravis: effect of maximal resection on late neurologic outcome and predictors of disease remission. Ann Thorac Surg 2009, 88(5):1638–1645.CrossRefPubMedGoogle Scholar
  40. 40.
    Toker A, Tanju S, Ziyade S, et al.: Early outcomes of video-assisted thoracoscopic resection of thymus in 181 patients with myasthenia gravis: Who are the candidates for the next morning discharge? Interact Cardiovasc Thorac Surg 2009, 9(6):995–998.CrossRefPubMedGoogle Scholar
  41. 41.
    Ponseti JM, Gamez J, Vilallonga R, et al.: Influence of ectopic thymic tissue on clinical outcome following extended thymectomy in generalized seropositive nonthymomatous myasthenia gravis. Eur J Cardiothorac Surg 2008, 34(5):1062–1067.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Department of Neurology, Neuromuscular DivisionWayne State University, School of MedicineDetroitUSA
  2. 2.Department of NeurologyWayne State University, School of MedicineDetroitUSA

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