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Published in: Current Treatment Options in Neurology 3/2010

01-05-2010 | Neuroimmunology

Myasthenia Gravis

Authors: Agnes Jani-Acsadi, MD, Robert P. Lisak, MD

Published in: Current Treatment Options in Neurology | Issue 3/2010

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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.
Literature
1.
go back to reference Keesey JC: Myasthenia Gravis: An Illustrated History. Roseville: Publishers Design Group; 2002. Keesey JC: Myasthenia Gravis: An Illustrated History. Roseville: Publishers Design Group; 2002.
2.
go back to reference Ohry A: Dr. Lazar Remen (1907-74): a forgotten pioneer in the treatment of myasthenia gravis. J Med Biogr 17(2):73–74. Ohry A: Dr. Lazar Remen (1907-74): a forgotten pioneer in the treatment of myasthenia gravis. J Med Biogr 17(2):73–74.
3. •
go back to reference 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. 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.
4.
go back to reference 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. 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.
5. •
go back to reference 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. 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.
6. •
go back to reference 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. 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.
7.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
8.
go back to reference Stickler DE, Massey JM, Sanders DB: MuSK-antibody positive myasthenia gravis: clinical and electrodiagnostic patterns. Clin Neurophysiol 2005, 116(9):2065–2068.CrossRefPubMed Stickler DE, Massey JM, Sanders DB: MuSK-antibody positive myasthenia gravis: clinical and electrodiagnostic patterns. Clin Neurophysiol 2005, 116(9):2065–2068.CrossRefPubMed
9.
go back to reference Romi F, Skeie GO, Gilhus NE: Striational antibodies in myasthenia gravis: reactivity and possible clinical significance. Arch Neurol 2005, 62(3):442–446.CrossRefPubMed Romi F, Skeie GO, Gilhus NE: Striational antibodies in myasthenia gravis: reactivity and possible clinical significance. Arch Neurol 2005, 62(3):442–446.CrossRefPubMed
11.
go back to reference Meriggioli MN: Myasthenia gravis: immunopathogenesis, diagnosis, and management. Continuum 2009, 15(1):34–62. Meriggioli MN: Myasthenia gravis: immunopathogenesis, diagnosis, and management. Continuum 2009, 15(1):34–62.
12.
go back to reference Hart IK, Sathasivam S, Sharshar T: Immunosuppressive agents for myasthenia gravis. Cochrane Database Syst Rev 2007, 4:CD005224.PubMed Hart IK, Sathasivam S, Sharshar T: Immunosuppressive agents for myasthenia gravis. Cochrane Database Syst Rev 2007, 4:CD005224.PubMed
13.
go back to reference Lisak RP: Myasthenia gravis. In Current Therapy in Neurological Disease. By Johnson RT, Griffin JW, McArthur JC. St. Louis: Mosby; 2002:407–409. Lisak RP: Myasthenia gravis. In Current Therapy in Neurological Disease. By Johnson RT, Griffin JW, McArthur JC. St. Louis: Mosby; 2002:407–409.
14. •
go back to reference 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. 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.
15.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
16. •
go back to reference 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. 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.
17.
go back to reference Kulaksizoglu IB: Mood and anxiety disorders in patients with myasthenia gravis: etiology, diagnosis and treatment. CNS Drugs 2007, 21:473–481.CrossRefPubMed Kulaksizoglu IB: Mood and anxiety disorders in patients with myasthenia gravis: etiology, diagnosis and treatment. CNS Drugs 2007, 21:473–481.CrossRefPubMed
18.
go back to reference Hoff JM, Daltveit AK, Gilhus NE: Myasthenia gravis in pregnancy and birth: identifying risk factors, optimizing care. Eur J Neurol 2007, 14:38–43.CrossRefPubMed Hoff JM, Daltveit AK, Gilhus NE: Myasthenia gravis in pregnancy and birth: identifying risk factors, optimizing care. Eur J Neurol 2007, 14:38–43.CrossRefPubMed
19.
go back to reference Ciafaloni E, Massey JM: The management of myasthenia gravis in pregnancy. Semin Neurol 2004, 24:95–100.CrossRefPubMed Ciafaloni E, Massey JM: The management of myasthenia gravis in pregnancy. Semin Neurol 2004, 24:95–100.CrossRefPubMed
20.
go back to reference Mehndiratta MM, Kuntzer T, Pandey S: Anticholinesterase treatment for myasthenia gravis. Cochrane Database Syst Rev 2008, 1:CD006986. Mehndiratta MM, Kuntzer T, Pandey S: Anticholinesterase treatment for myasthenia gravis. Cochrane Database Syst Rev 2008, 1:CD006986.
21.
go back to reference Schneider-Gold C, Gajdos P, Toyka KV, Hohlfeld RR: Corticosteroids for myasthenia gravis. Cochrane Database Syst Rev 2005, 2:CD002828.PubMed Schneider-Gold C, Gajdos P, Toyka KV, Hohlfeld RR: Corticosteroids for myasthenia gravis. Cochrane Database Syst Rev 2005, 2:CD002828.PubMed
22.
go back to reference 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.PubMed 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.PubMed
23.
go back to reference Conti-Fine B: Myasthenia Gravis: The Immunobiology of an Autoimmune Disease. Austin: Landes Bioscience; 1997. Conti-Fine B: Myasthenia Gravis: The Immunobiology of an Autoimmune Disease. Austin: Landes Bioscience; 1997.
24.
go back to reference 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.PubMed 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.PubMed
25.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
26.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
27.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
28.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
29.
go back to reference The utility of therapeutic plasmapheresis for neurological disorders. NIH Consens Statement 1986, 6(4):1–7. The utility of therapeutic plasmapheresis for neurological disorders. NIH Consens Statement 1986, 6(4):1–7.
30.
go back to reference Gajdos P, Chevret S, Toyka K: Plasma exchange for myasthenia gravis. Cochrane Database Syst Rev 2002, 4:CD002275.PubMed Gajdos P, Chevret S, Toyka K: Plasma exchange for myasthenia gravis. Cochrane Database Syst Rev 2002, 4:CD002275.PubMed
31.
go back to reference Gajdos P, Chevret S, Toyka K: Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev 2008, 1:CD002277.PubMed Gajdos P, Chevret S, Toyka K: Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev 2008, 1:CD002277.PubMed
32.
go back to reference Qureshi AI, Choudhry MA, Akbar MS, et al.: Plasma exchange versus intravenous immunoglobuline treatment in myasthenic crisis. Neurology 1999, 52:629–632.PubMed Qureshi AI, Choudhry MA, Akbar MS, et al.: Plasma exchange versus intravenous immunoglobuline treatment in myasthenic crisis. Neurology 1999, 52:629–632.PubMed
33.
go back to reference 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 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
34.
go back to reference Cea G, Benatar M, Verdugo RJ, Salinas RA: Thymectomy for non-thymomatous myasthenia gravis. Cochrane Database Syst Rev 2009, 4:CD008111. Cea G, Benatar M, Verdugo RJ, Salinas RA: Thymectomy for non-thymomatous myasthenia gravis. Cochrane Database Syst Rev 2009, 4:CD008111.
35.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
36.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
37.
go back to reference 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.CrossRef 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.CrossRef
38.
go back to reference Sonett JR, Jaretzki A: Thymectomy for nonthymomatous myasthenia gravis: a critical analysis. Ann N Y Acad Sci 2008, 1132:315–328.CrossRefPubMed Sonett JR, Jaretzki A: Thymectomy for nonthymomatous myasthenia gravis: a critical analysis. Ann N Y Acad Sci 2008, 1132:315–328.CrossRefPubMed
39.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
40.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
41.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
Metadata
Title
Myasthenia Gravis
Authors
Agnes Jani-Acsadi, MD
Robert P. Lisak, MD
Publication date
01-05-2010
Publisher
Current Science Inc.
Published in
Current Treatment Options in Neurology / Issue 3/2010
Print ISSN: 1092-8480
Electronic ISSN: 1534-3138
DOI
https://doi.org/10.1007/s11940-010-0070-0

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