Subscribe to RSS
DOI: 10.1055/s-2004-829586
Management of Myasthenia Gravis
Publication History
Publication Date:
01 July 2004 (online)
Although once a severe and often fatal illness, myasthenia gravis can now be well managed with several relatively safe and effective therapies. Management involves a graded approach, beginning with cholinesterase inhibitors for mild symptoms and advancing to immunomodulating medications for more severe weakness. There are several immunomodulating agents from which to choose; selection is based largely on time to clinical effect and adverse effects. This review will discuss the selection and use of therapies for patients with myasthenia gravis.
KEYWORDS
Myasthenia gravis - immunosuppressant - IVIg - plasmapheresis - thymectomy
REFERENCES
- 1 Berrouschot J, Baumann I, Kalischewski P, Sterker M, Schneider D. Therapy of myasthenic crisis. Crit Care Med. 1997; 25 1228-1235
- 2 Mayer S A, Thomas C E. Therapy of myasthenic crisis. Crit Care Med. 1998; 26 1136-1137
- 3 Tindall R SA. Humoral immunity in myasthenia gravis: effect of steroids and thymectomy. Neurology. 1980; 30 554-557
- 4 Warmolts J R, Engel W K. Benefit from alternate-day prednisone in myasthenia gravis. N Engl J Med. 1972; 286 17-20
- 5 Pascuzzi R M, Coslett H B, Johns T R. Long-term corticosteroid treatment of myasthenia gravis: report of 116 patients. Ann Neurol. 1984; 15 291-298
- 6 Johns T R. Long-term corticosteroid treatment of myasthenia gravis. Ann N Y Acad Sci. 1984; 505 568-583
- 7 Miller R G, Milner-Brown S, Mirka A. Prednisone-induced worsening of neuromuscular function in myasthenia gravis. Neurology. 1986; 36 729-732
- 8 Seybold M E, Drachman D B. Gradually increasing doses of prednisone in myasthenia gravis: reducing the hazards of treatment. N Engl J Med. 1974; 290 81-84
- 9 Saperstein D S, Amato A A, Katz J S, Barohn R J. Immune mediated polyneuropathies. In: Pourmand R Neuromuscular Diseases: Expert Clinicians' Views. Boston; Butterworth-Heinemann 2001: 135-198
- 10 Saag K G, Emkey R, Schnitzer T J et al.. Alendronate for the prevention and treatment of glucocorticoid-induced osteoporosis. Glucocorticoid-Induced Osteoporosis Intervention Study Group. N Engl J Med. 1998; 339 292-299
- 11 Mantegazza R, Antozzi C, Peluchitte D, Sghirlanzoni A, Cornelio F. Azathioprine as a single drug or in combination with steroids in the treatment of myasthenia gravis. J Neurol. 1988; 235 449-453
- 12 Myasthenia Gravis Clinical Study Group . A randomized clinical trial comparing prednisone and azathioprine in myasthenia gravis. Results of the second interim analysis. J Neurol Neurosurg Psychiatry. 1993; 53 1157-1163
- 13 Matell G. Immunosuppressive drugs: azathioprine in the treatment of myasthenia gravis. Ann N Y Acad Sci. 1987; 505 588-594
- 14 Hohlfeld R, Michels M, Heininger K, Besinger U, Toyka K V. Azathioprine toxicity during long-term immunosuppression of generalized myasthenia gravis. Neurology. 1988; 38 258-261
- 15 Mertens H G, Hertel P, Reuther P, Ricker K. Effect of immunosuppressive drugs (Azathioprine). Ann N Y Acad Sci. 1981; 337 691-699
- 16 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
- 17 Kissel J T, Levy R J, Mendell J R, Griggs R C. Azathioprine toxicity in neuromuscular disease. Neurology. 1986; 36 35-39
- 18 Herrlinger U, Weller M, Dichgans J, Melms A. Association of primary central nervous system lymphoma with long-term azathioprine therapy for myasthenia gravis. Ann Neurol. 2000; 47 682-683
- 19 Tindall R SA, Phillips J T, Rollins J A, Wells L, Hall K. A clinical therapeutic trial of cyclosporine in myasthenia gravis. Ann N Y Acad Sci. 1993; 681 539-551
- 20 Tindall R SA, Rollins J A, Phillips J T, Greenlee R G, 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
- 21 Ciafaloni E, Nikhar N K, Massey J M, Sanders D B. Retrospective analysis of the use of cyclosporine in myasthenia gravis. Neurology. 2000; 55 448-450
- 22 Ciafaloni E, Massey J M, Tucker-Lipscomb B, Sanders D B. Mycophenolate mofetil for myasthenia gravis: an open-label pilot study. Neurology. 2001; 56 97-99
- 23 Chaudhry V, Cornblath D R, Griffin J W, O'Brien R, Drachman D B. Mycophenolate mofetil: a safe and promising immunosuppressant in neuromuscular diseases. Neurology. 2001; 56 94-96
- 24 Meriggioli M N, Ciafaloni E, Al-Hayk K A et al.. Mycophenolate mofetil for myasthenia gravis: an analysis of efficacy, safety, and tolerability. Neurology. 2003; 61 1438-1440
- 25 Meriggioli M N, Rowin J, Richman J G, Leurgans S. Mycophenolate mofetil for myasthenia gravis: a double-blind, placebo-controlled pilot study. Ann N Y Acad Sci. 2003; 998 494-499
- 26 Haberal M, Karakayali H, Emiroglu R, Basaran O, Moray G, Bilgin N. Malignant tumors after organ transplantation. Artif Organs. 2002; 26 778-781
- 27 Perez M C, Buot W L, Mercado-Danguilan C, Bagabaldo Z G, Renales L D. Stable remissions in myasthenia gravis. Neurology. 1981; 31 32-37
- 28 De Feo L G, Schottlender J, Martelli N A, Molfino N A. Use of intravenous pulsed cyclophosphamide in severe, generalized myasthenia gravis. Muscle Nerve. 2002; 26 31-36
- 29 Drachman D B, Jones R J, Brodsky R A. Treatment of refractory myasthenia: “rebooting” with high-dose cyclophosphamide. Ann Neurol. 2003; 53 29-34
- 30 Dau P C, Lindstrom J M, Cassel C K, Denys E H, Shev E E, Spitler L E. Plasmapheresis and immunosuppressive drug therapy in myasthenia gravis. N Engl J Med. 1977; 297 1134-1140
- 31 Arsura E. Experience with intravenous immunoglobulin in myasthenia gravis. Clin Immunol Immunopathol. 1989; 53 S170-S179
- 32 Fateh-Moghadam A, Wick M, Besinger U, Geursen R G. High-dose intravenous gammaglobulin for myasthenia gravis. Lancet. 1984; 1 848-849
- 33 Gajdos P, Outin H, Elkharrat D et al.. High-dose intravenous gammaglobulin for myasthenia gravis. Lancet. 1984; 1 406-407
- 34 Cosi V, Lombardi M, Piccolo G, Erbetta A. Treatment of myasthenia gravis with high-dose intravenous immunoglobulin. Acta Neurol Scand. 1991; 84 81-84
- 35 Dalakas M C. Experience with IVIg in the treatment of patients with myasthenia gravis. Neurology. 1997; 48(suppl 5) S64-S69
- 36 Achiron A, Barak Y, Miron S, Sarova-Pinhas I. Immunoglobulin treatment in refractory myasthenia gravis. Muscle Nerve. 2000; 23 551-555
- 37 Jongen J L, van Doorn P A, van der Meche F G. High-dose intravenous immunoglobulin therapy for myasthenia gravis. J Neurol. 1998; 245 26-31
- 38 Liblau R, Gajdos P, Bustarret F A et al.. Intravenous gamma-globulin in myasthenia gravis: interaction with anti-acetylcholine receptor autoantibodies. J Clin Immunol. 1991; 11 128-131
- 39 Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C. for the Myasthenia Gravis Clinical Study Group . Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Ann Neurol. 1997; 41 789-796
- 40 Qureshi A I, Choudhry M A, Akbar M S et al.. Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology. 1999; 52 629-632
- 41 Stricker R B, Kwiatkowska B J, Habis J A, Kiprov D D. Myasthenic crisis: response to plasmapheresis following failure of intravenous gamma-globulin. Arch Neurol. 1993; 50 837-840
- 42 Wolfe G I, Barohn R J, Foster B M et al.. Randomized, controlled trial of intravenous immunoglobulin in myasthenia gravis. Muscle Nerve. 2002; 26 549-552
- 43 Tan E, Hajinazarian M, Bay W, Neff J, Mendell J R. Acute renal failure resulting from intravenous immunoglobulin therapy. Arch Neurol. 1993; 50 137-139
- 44 Steg R E, Lefkowitz D M. Cerebral infarction following intravenous immunoglobulin therapy for myasthenia gravis. Neurology. 1994; 44 1180-1181
- 45 Caress J B, Cartwright M S, Donofrio P D, Peacock Jr J E. The clinical features of 16 cases of stroke associated with administration of IVIg. Neurology. 2003; 60 1822-1824
- 46 Blalock A, Mason M F, Morgan H J et al.. Myasthenia gravis and tumors of the thymic region. Report of a case in which the tumor was removed. Ann Surg. 1939; 110 544-561
- 47 Gronseth G S, Barohn R J. Practice parameter: thymectomy for autoimmune myasthenia gravis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2000; 55 7-15
- 48 Mantegazza R, Baggi F, Antozzi C et al.. Myasthenia gravis (MG): epidemiological data and prognostic factors. Ann N Y Acad Sci. 2003; 998 413-423
- 49 Perlo V P, Arnason B, Poskanzer D et al.. The role of thymectomy in the treatment of myasthenia gravis. Ann N Y Acad Sci. 1971; 183 308-315
- 50 Rodriguez M, Gomez M R, Howard Jr F M, Taylor W F. Myasthenia gravis in children: long-term follow-up. Ann Neurol. 1983; 13 504-510
- 51 Mulder D G, Graves M, Herrmann C. Thymectomy for myasthenia gravis: recent observation and comparisons with past experience. Ann Thorac Surg. 1989; 48 551-555
- 52 Lanska D J. Diagnosis of thymoma in myasthenics using anti-striated muscle antibodies: predictive value and gain in diagnostic certainty. Neurology. 1991; 41 520-524
- 53 Schumm F, Wiethölter H, Fateh-Moghadam A, Dichgans J. Thymectomy in myasthenia with pure ocular symptoms. J Neurol Neurosurg Psychiatry. 1985; 48 332-337
- 54 Lanska D J. Indications for thymectomy in myasthenia gravis. Neurology. 1990; 40 1828-1829
- 55 Grob D, Asura E L, Brunner N G, Namba T. The course of myasthenia gravis and therapies affecting outcome. Ann N Y Acad Sci. 1987; 505 472-499
- 56 McQuillen M P, Leone M G. A treatment carol: thymectomy revisited. Neurology. 1977; 27 1103-1106
- 57 Wolfe G I, Kaminski H J, Jaretzki III A, Swan A, Newsom-Davis J. Development of a thymectomy trial in nonthymomatous myasthenia gravis patients receiving immunosuppressive therapy. Ann N Y Acad Sci. 2003; 998 473-480
- 58 Monden Y, Nakahara K, Kagotani K et al.. Effects of preoperative duration of symptoms on patients with myasthenia gravis. Ann Thorac Surg. 1984; 38 287-291
- 59 DeFilippi V J, Richman D P, Ferguson M K. Transcervical thymectomy for myasthenia gravis. Ann Thorac Surg. 1994; 57 194-197
- 60 Younger D S, Jaretzki III A, Penn A S et al.. Maximum thymectomy for myasthenia gravis. Ann N Y Acad Sci. 1987; 505 832-835
- 61 Papatestas A E, Genkins G, Kornfeld P, Horowitz S, Kark A E. Transcervical thymectomy in myasthenia gravis. Surg Gynecol Obstet. 1975; 140 535-540
- 62 Cooper J D, Al-Jilaihawa A N, Pearson F G, Humphrey H E. An improved technique to facilitate transcervical thymectomy for myasthenia gravis. Ann Thorac Surg. 1988; 45 242-247
- 63 Yim A P, Kay R L, Ho J K. Video-assisted thoracoscopic thymectomy for myasthenia gravis. Chest. 1995; 108 1440-1443
- 64 Sabbagh M N, Garza J JS, Patten B. Thoracoscopic thymectomy in patients with myasthenia gravis. Muscle Nerve. 1995; 18 1475-1477
- 65 Mack M J, Landreneau R J, Yim A P, Hazelrigg S R, Scruggs G R. Results of video-assisted thymectomy in patients with myasthenia gravis. J Thorac Cardiovasc Surg. 1996; 112 1352-1359
- 66 Batra P, Herrmann Jr C, Mulder D. Mediastinal imaging in myasthenia gravis: correlation of chest radiography, CT, MR, and surgical findings. AJR Am J Roentgenol. 1987; 148 515-519
- 67 Nations S P, Wolfe G I, Amato A A, Jackson C, Bryan W W, Barohn R J. Myasthenia gravis developing after removal of thymoma. J Child Neurol. 1997; 12 133
- 68 Yoshikawa H, Mabuchi K, Yasukawa Y, Takamori M, Yamada M. Low-dose tacrolimus for intractable myasthenia gravis. J Clin Neurosci. 2002; 9 627-628
- 69 Evoli A, Di Schino C, Marsili F, Punzi C. Successful treatment of myasthenia gravis with tacrolimus. Muscle Nerve. 2002; 25 111-114
- 70 De Mattos A M, Olyaei A J, Bennett W M. Nephrotoxicity of immunosuppressive drugs: long-term consequences and challenges for the future. Am J Kidney Dis. 2000; 35 333-346
- 71 Wylam M E, Anderson P M, Kuntz N L, Rodriguez V. Successful treatment of refractory myasthenia gravis using rituximab: a pediatric case report. J Pediatr. 2003; 143 674-677
- 72 Zaja F, Russo D, Fuga G, Perella G, Baccarani M. Rituximab for myasthenia gravis developing after bone marrow transplant. Neurology. 2000; 55 1062-1063
- 73 Scuderi F, Marino M, Colonna L et al.. Anti-P110 autoantibodies identify a subtype of “seronegative” myasthenia gravis with prominent oculobulbar involvement. Lab Invest. 2002; 82 1139-1146
- 74 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
- 75 Sanders D B, El-Salem K, Massey J M, McConville J, Vincent A. Clinical aspects of MuSK antibody positive seronegative MG. Neurology. 2003; 60 1978-1980
David S SapersteinM.D.
Assistant Professor, Department of Neurology, University of Kansas Medical Center
3901 Rainbow Blvd., Mail-stop 2012
Kansas City, KS 66160