Advertisement

Myasthenia Gravis: Classification and Outcome Measurements

  • Gil I. Wolfe
  • Richard J. Barohn
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

INTRODUCTION

Myasthenia gravis is the best characterized and understood autoimmune disease of the nervous system (1). It is the most common neuromuscular transmission disorder followed in neurology clinics. Nevertheless, standardized classification and grading systems and outcome measures for MG were not developed until the last decade and continue to be refined. In 1997 the Medical Scientific Advisory Board (MSAB) of the Myasthenia Gravis Foundation of America (MGFA) formed a task force to design classification and outcome measures for the disease (2). The task force’s primary charge was to create a system of uniformity in recording and reporting clinical data and outcomes research. Chairing the project was Dr. Alfred Jaretzki III, a cardiothoracic surgeon who had published extensively with colleagues at Columbia Presbyterian Medical Center on thymectomy for MG (3, 4). He expressed concern that published outcomes following thymectomy could not be reliably compared across studies due...

Keywords

Amyotrophic Lateral Sclerosis Mycophenolate Mofetil Manual Muscle Testing Score Static Fatigue Testing Complete Stable Remission 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

REFERENCES

  1. 1.
    Vincent A, Palace J, Hilton-Jones D. Myasthenia gravis. Lancet 2001;357:2122–2128.PubMedCrossRefGoogle Scholar
  2. 2.
    Barohn RJ. Standards of measurement in myasthenia gravis. Ann N Y Acad Sci 2003;998:432–439.PubMedCrossRefGoogle Scholar
  3. 3.
    Jaretzki III A. Thymectomy for myasthenia gravis: analysis of the controversies regarding technique and result. Neurology 1997;48(suppl 5):S52–S63.Google Scholar
  4. 4.
    Jaretzki III A, Penn AS, Younger DS, Wolff M, Olarte MR, Lovelace RE, et al. “Maximal” thymectomy for myasthenia gravis. Results. J Thorac Cardiovasc Surg 1988;95:747–757.PubMedGoogle Scholar
  5. 5.
    Jaretzki III A, Barohn RJ, Ernstoff RM, Kaminski HJ, Keesey JC, Penn AS, et al. Myasthenia gravis: recommendations for clinical research standards. Neurology 2000;55:16–23.PubMedGoogle Scholar
  6. 6.
    Osserman KE. Clinical aspects. In: Osserman KE, editor. Myasthenia Gravis. New York: Grune & Stratton; 1958. pp. 79–80.Google Scholar
  7. 7.
    Osserman KE, Kornfeld P, Cohen E, Genkins G. Studies in myasthenia gravis: review of two hundred eighty-two cases at the Mount Sinai Hospital, New York City. Arch Int Med 1958;102:72–81.CrossRefGoogle Scholar
  8. 8.
    Osserman KE, Genkins G. Studies in myasthenia gravis: review of a twenty-year experience in over 1200 patients. Mt Sinai J Med 1971;38:497–537.PubMedGoogle Scholar
  9. 9.
    Barohn RJ, Jackson CE. New classification system for myasthenia gravis (abstract). J Child Neurol 1994;9:205.Google Scholar
  10. 10.
    Besinger UA, Toyka KV, Heininger K, et al. Long-term correlation of clinical course and acetylcholine receptor antibody in patients with myasthenia gravis. Ann NY Acad Sci 1981;377:812–815.CrossRefGoogle Scholar
  11. 11.
    Olanow CW, Wechsler AS, Sirotkin-Roses M, Stajich J, Roses AD. Thymectomy as primary therapy in myasthenia gravis. Ann NY Acad Sci 1987;505:595–606.PubMedCrossRefGoogle Scholar
  12. 12.
    Wolfe GI, Barohn RJ. Neuromuscular junction disorders of childhood. In: Swaiman KF, Ashwal S, Ferriero DM, editors. Pediatric Neurology: Principles and Practice. 4th ed. Philadelphia: Mosby Elsevier; 2006. pp. 1941–1968.Google Scholar
  13. 13.
    Oosterhuis HJ. Observations of the natural history of myasthenia gravis and the effect of thymectomy. Ann N Y Acad Sci 1981;377:678–690.PubMedCrossRefGoogle Scholar
  14. 14.
    Wolfe GI, Kaminski HJ, Jaretzki III A, Swan A, Newsom-Davis J. Development of a thymectomy trial in nonthymomatous myasthenia gravis patients receiving immunosuppressive therapy. Ann NY Acad Sci 2003;998:473–480.PubMedCrossRefGoogle Scholar
  15. 15.
    Kawaguchi N, Kuwabara S, Nemoto Y, Fukutake T, Satomura Y, Arimura K, et al. Treatment and outcome of myasthenia gravis: retrospective multi-center analysis of 470 Japanese patients, 1999–2000. J Neurol Sci 2004;224:43–47.PubMedCrossRefGoogle Scholar
  16. 16.
    Besinger UA, Toyka KV, Homberg M, Heininger K, Hohlfeld R, Fateh-Moghadam A. Myasthenia gravis: long-term correlation of binding and bungarotoxin blocking antibodies against acetylcholine receptors with changes in disease severity. Neurology 1983;33:1316–1321.PubMedGoogle Scholar
  17. 17.
    Tindall RSA, Phillips JT, Rollins JA, Wells L, Hall K. A clinical therapeutic trial of cyclosporine in myasthenia gravis. Ann NY Acad Sci 1993;681:539–551.PubMedCrossRefGoogle Scholar
  18. 18.
    Tindall RSA, Rollins JA, Phillips JT, Greenlee RG, Wells L, Belendiuk G. Preliminary results of a double-blind, randomized, placebo-controlled trial of cyclosporine in myasthenia gravis. N Engl J Med 1987;316:719–724.PubMedCrossRefGoogle Scholar
  19. 19.
    Barohn RJ, McIntire D, Herbelin L, Wolfe GI, Nations S, Bryan WW. Reliability testing of the Quantitative Myasthenia Gravis Score. Ann NY Acad Sci 1998;841:769–772.PubMedCrossRefGoogle Scholar
  20. 20.
    Wolfe GI, Barohn RJ, Foster BM, Jackson CE, Kissel JT, Day JW, et al. Randomized, controlled trial of intravenous immunoglobulin in myasthenia gravis. Muscle & Nerve 2002;26:549–552.CrossRefGoogle Scholar
  21. 21.
    Sharshar T, Chevret S, Mazighi M, Chillet P, Huberfeld G, Berreotta C, et al. Validity and reliability of two muscle strength scores commonly used as endpoints in assessing treatment of myasthenia gravis. J Neurol 2000;246:286–289.CrossRefGoogle Scholar
  22. 22.
    Bedlack RS, Simel DL, Bosworth H, Samsa G, Tucker-Lipscomb B, Sanders DB. Quantitative myasthenia gravis score: assessment of responsiveness and longitudinal validity. Neurology 2005;64:1968–1970.PubMedCrossRefGoogle Scholar
  23. 23.
    Meriggioli MN, Ciafaloni E, Al-Hayk KA, Rowin J, Tucker-Lipscomb B, Massey JM, et al. Mycophenolate mofetil for myasthenia gravis: an analysis of efficacy, safety, and toleratibility. Neurology 2003;61:1438–40.PubMedGoogle Scholar
  24. 24.
    Nagane Y, Utsugisawa K, Obara D, Kondoh R, Terayama Y. Eur Neurol 2005;53:146–150.PubMedCrossRefGoogle Scholar
  25. 25.
    Ponseti JM, Azem J, Fort JM, Codina A, Montoro JB, Armengol M. Benefits of FK506 (tacrolimus) for residual, cyclosporin- and prednisone-resistant myasthenia gravis: one-year follow-up of an open-label study. Clin Neurol Neurosurg 2005;107:187–190.PubMedCrossRefGoogle Scholar
  26. 26.
    Rowin J, Meriggioli MN, Tüzün E, Leurgans S, Christadoss P. Etanercept treatment in corticosteroid-dependent myasthenia gravis. Neurology 2004;63:2390–2392.PubMedGoogle Scholar
  27. 27.
    Barohn RJ. Video: how to administer the quantitative myasthenia test. Myasthenia Gravis Foundation of America, Inc. 1821 University Ave.W. Suite S256,St. Paul, MN 55104; 1996.Google Scholar
  28. 28.
    Sanders DB, Tucker-Lipscomb B, Massey JM. A simple manual muscle test for myasthenia gravis. Ann N Y Acad Sci 2003;998:440–444.PubMedCrossRefGoogle Scholar
  29. 29.
    Ciafaloni E, Massey JM, Tucker-Lipscomb B, Sanders DB. Mycophenolate mofetil for myasthenia gravis: an open-label pilot study. Neurology 2001;56:97–99.PubMedGoogle Scholar
  30. 30.
    Gajdos P, Simon N, de Rohan Chabot P, Goulon M. Effets a long terme des echanges plasmatiques au cours de la myasthenie. Resultats d'une etude randomisee. Presse Med 1983;12:939–942.PubMedGoogle Scholar
  31. 31.
    Gajdos P, Sharshar T, Chevret S. Standards of measurement in myasthenia gravis. Ann N Y Acad Sci 2003;998:445–452.PubMedCrossRefGoogle Scholar
  32. 32.
    Wolfe GI, Herbelin L, Nations SP, Foster B, Bryan WW, Barohn RJ. Myasthenia gravis activities of daily living profile. Neurology 1999;52:1487–1489.PubMedGoogle Scholar
  33. 33.
    Takamori M, Motomura M, Kawaguchi N, Nemoto Y, Hattori T, Yoshikawa Y, et al. Anti-ryanodine receptor antibodies and FK506 in myasthenia gravis. Neurology 2004;62:1894–1896.PubMedGoogle Scholar
  34. 34.
    Kawaguchi N, Yoshiyama Y, Nemoto Y, Munakata S, Fukutake T, Hattori T. Low-dose tacrolimus treatment in thymectomised and steroid-dependent myasthenia gravis. Curr Med Res Opinions 2004;20:1269–1273.CrossRefGoogle Scholar
  35. 35.
    Herbelin LL, Nations SP, Wolfe GI, Foster BM, Bryan WW, Barohn RJ. The correlation between static fatigue testing and the quantitative myasthenia gravis score and activities of daily living profile (abstract). Neurology 2001;56:A63.Google Scholar
  36. 36.
    Ponseti JM, Azem J, Fort JM, López-Cano M, R V, Buera M, et al. Long-term results of tacrolimus in cyclosporine- and prednisone-dependent myasthenia gravis. Neurology 2005;64:1641–1643.PubMedCrossRefGoogle Scholar

Copyright information

© Humana Press, a part of Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Gil I. Wolfe
    • 1
  • Richard J. Barohn
    • 2
  1. 1.Department of NeurologyUniversity of Texas Southwestern Medical SchoolDallas
  2. 2.Department of NeurologyUniversity of Kansas Medical CenterKansas City

Personalised recommendations