Miastenia grave: tratamento com timectomia, corticóide e plasmaferese

Dias-Tosta, Elza; Morato-Fernandes, Rubens Nelson

doi:10.1590/S0004-282X1989000100006

Resumos

Após introdução sobre a etiopatogenia da miastenia grave e divergências quanto às várias modalidades terapêuticas, os autores mostram os resultados favoráveis obtidos com a timectomia isoladamente (13 casos): 15,3% com remissão completa (2 de 13), 46,15% com melhora importante (6 casos), 30,7% com melhora de apenas um grau na escala de atividade (4 casos). A associação de corticosteróide e/ou plasmaferese elevou para 14,8% a remissão (4 do total de 27 casos); 74% apresentaram melhora (20 casos), 7,4%, piora (dois casos) e um caso não apresentou resposta às diferentes terapêuticas. Na sua casuística mostram ainda que a corticoterapia deve entrar como terapêutica de segunda linha, devido a seus efeitos colaterais e à dificuldade na restirada; apontam a plasmaferese como tratamento da emergência miastênica. Fazem ainda revisão da literatura quanto ao uso de outros imunossupressores não esteróides.

This paper reviews a 12 year experience with myasthenia gravis management (surgical and drug therapy). A total of 27 patients were submitted to thymectomy, and 12 out of 13 showed fairly good results with this only form of therapy (15.3% with complete remission, 46.15% with marked improvement and 30.7% with moderate improvement). The other 14 need either a combination of surgery and plasmapheresis or corticosteroids with the cummulative results of: 14.8% of remission (4 out of 27), 74% of improvement (20 out of 27), 7.4% of worsening (2 out of 27) and 3.7% without change (1 out of 27). Two other patients not submitted to surgery showed either a stable state ot their symptoms or a mildly worsening. Another eight patients not submitted to surgery could not be hollowed up. The authors also conclude by the validity of the use of plasmapheresis in myasthenic crises leading to a transient relief of the symptoms and suggest the use of corticosteroids as a second choice, due to their undesirable side effects and difficulty in their reduction and elimination without worsening the symptoms. Other immunosuppressive drugs could be used in cases in which those above cited therapies showed unsuitable results.

Miastenia grave: tratamento com timectomia, corticóide e plasmaferese

Myasthenia gravis: thymectomy, corticosteroid and plasmapheresis therapy

Elza Dias-Tosta^I; Rubens Nelson Morato-Fernandes^II

^IUnidade de Neurologia do Hospital de Base do Distrito Federal (HBDF): Médica Neurologista, atualmente no Hospital de Doenças do Aparelho Locomotor (HDAL)/SARAH

^IIUnidade de Neurologia do Hospital de Base do Distrito Federal (HBDF): Médico Neurologista

RESUMO

Após introdução sobre a etiopatogenia da miastenia grave e divergências quanto às várias modalidades terapêuticas, os autores mostram os resultados favoráveis obtidos com a timectomia isoladamente (13 casos): 15,3% com remissão completa (2 de 13), 46,15% com melhora importante (6 casos), 30,7% com melhora de apenas um grau na escala de atividade (4 casos). A associação de corticosteróide e/ou plasmaferese elevou para 14,8% a remissão (4 do total de 27 casos); 74% apresentaram melhora (20 casos), 7,4%, piora (dois casos) e um caso não apresentou resposta às diferentes terapêuticas. Na sua casuística mostram ainda que a corticoterapia deve entrar como terapêutica de segunda linha, devido a seus efeitos colaterais e à dificuldade na restirada; apontam a plasmaferese como tratamento da emergência miastênica. Fazem ainda revisão da literatura quanto ao uso de outros imunossupressores não esteróides.

SUMMARY

This paper reviews a 12 year experience with myasthenia gravis management (surgical and drug therapy). A total of 27 patients were submitted to thymectomy, and 12 out of 13 showed fairly good results with this only form of therapy (15.3% with complete remission, 46.15% with marked improvement and 30.7% with moderate improvement). The other 14 need either a combination of surgery and plasmapheresis or corticosteroids with the cummulative results of: 14.8% of remission (4 out of 27), 74% of improvement (20 out of 27), 7.4% of worsening (2 out of 27) and 3.7% without change (1 out of 27). Two other patients not submitted to surgery showed either a stable state ot their symptoms or a mildly worsening. Another eight patients not submitted to surgery could not be hollowed up. The authors also conclude by the validity of the use of plasmapheresis in myasthenic crises leading to a transient relief of the symptoms and suggest the use of corticosteroids as a second choice, due to their undesirable side effects and difficulty in their reduction and elimination without worsening the symptoms. Other immunosuppressive drugs could be used in cases in which those above cited therapies showed unsuitable results.

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Full text available only in PDF format.

Agradecimentos - Os autores desejam expressar seus agradecimentos a toda a equipe de Neurologia do HBDF e de Neurofisiologia do HDAL/SARAH e HBDF, que permitiu a realização deste estudo, aos responsáveis pela Cirurgia sob a orientação do Dr. Manoel Ximenes Neto e de Plasmaferese (Dr. Cid L. Vale e Dra. Jussara S. Oliveira).

SQS 306, Bloco C, Apto. 506 - 7035.3 Brasília DF - Brasil

1. Assis JL, Marchiori PE, Scaff M, Zambon AA - Tratamento conservador e timectomia na miastenia grave. Arq Neuro-Psiquiat (São Paulo) 45:119, 1987.
2. Bever CT, Aquino AV, Penn AS, Lovelace RE, Rowland LP - Prognosis of ocular myasthenia. Ann Neurol 14:516, 1983.
3. Buckingham JN, Howard FM Jr, Bernatz PE, Spencer Payne W, Harrison EG Jr, O'Brien PC, Weiland LH - The value of thymectomy in myasthenia gravis: a computer assisted matched study. Ann Surg 184:453, 1976.
4. Dau PC, Lindstrom JM, Cassel CK, Denys EH, Shev EE, Spitler LE - Plasmapheresis and immunossuppressive drug therapy in myasthenia gravis. New Engl J Med 297:1135, 1977.
5. Drachman DB - Myasthenia gravis. New Engl J Med 298:136, 1978.
6. Drachman DB - Myasthenia gravis. New Engl J Med 298:186, 1978.
7. Drachman DB - Present and future treatment of myasthenia gravis. New Engl J Med 316:743, 1987.
8. Engel AG, Lambert EH, Howard FM - Immune complexes (IgG and C₃) at the motor end-plate in myasthenia gravis: ultra-strutural and light microscopic localization and electrophysiologic correlations. Mayo Clin Proc 52:267, 1977.
9. Engel AG - Myasthenia gravis and myasthenic syndromes. Ann Neurol 16:519, 1984.
10. Hankins JR, Mayer RF, Satterfield JR, Turney SZ, Attar S, Sequeira AJ, Thompson BW, McLaughlin JS - Thymectomy for myasthenia gravis: 14 -year experience. Ann Surg 201:618, 1985.
11. Haynes BF, Harden EA, Olanow CW, Eisenbarth GS, Wechsler AS, Hensley LL, Roses AD - Effect of thymectomy on peripheral lymphocyte subsets in myasthenia gravis: selective effect on T cells in patients with thymic atrophy. J Immunol 131:773, 1983.
12. Jacobsen H, Thorlacius S, Aaarli JA - Plasmapheresis in myasthenia gravis: clinical results and changes in serum proteins. Acta Neurol Scand 65(Suppl 90):128, 1982.
13. Kurtzke JF - Epidemiology of myasthenia gravis. Advances in Neurology, vol 19. Raven Press, New York, 1978.
14. Lisak RP, Barchi RL - Myasthenia gravis. Major Problems in Neurology, vol 11, 1982.
15. Matell G - Myasthenia gravis: an autoimmune receptor disease. Acta Neurol Scand 65(Suppl 90):112, 1982.
16. McQuillen MP, Leone MG - A treatment carol: thymectomy revisited. Neurology 27:1103, 1977.
17. Moore HJ, Woods EL - Myasthenia gravis: associated antibodies in asymptomatic thymoma. J Thorac Cardiovasc Surg 89:308, 1985-
18. Munsat TL - Anticholinesterase abuse in myasthenia gravis. J Neurol Sci 64:5, 1984.
19. Namba T, Brunner NG, Grob D - Myasthenia gravis in patients with thymoma, with particular reference to onset after thymectomy. Medicine 57:411, 1978.
20. Newsom Davis J, Vicent A, Ward CD, Wilson SG, Pinching AJ - Plasmapheresis exchange: short and long-term benefits. In Dau PC (ed): Plasmapheresis and the Immunobiolgy of Myasthenia Gravis. Houghton Mifflin, Boston, 1979, pg 199.
21. Niakam E, Harati Y, Rolak LA - Immunossuppressive drug therapy in myasthenia gravis. Arch Neurol 43:155, 1986.
22. Osserman KE - Myasthenia Gravis. Grune & Stratton, New York, 1958, pg 78, 86.
23. Pascuzzi RM, Branch Coslett H, Johns TR - Long-term corticosteroid treatment of myasthenia gravis: report of 116 patients. Ann Neurol 15:291, 1984.
24. Pinching AJ, Peters DK, Newson-Davis J - Remission of myasthenia gravis following plasma exchange. Lancet 2:1373, 1976.
25. Rowland LP, Korengold MC, Jaffe IA, Berg L, Shy GM - Prostigmine induced muscle weakness in myasthenia gravis patients. Neurology 5:89, 1955.
26. Scadding GK, Havard CWH, Lange MJ, Domb I - The long term experience of thymectomy for myasthenia gravis. J Neurol Neurosurg Psychiat 48:401, 1985.
27. Schumm F, Wiethölter H, Moghadam AF, Dichgan J - Thymectomy in myasthenia with pure ocular symptoms. J Neurol Neurosurg Psychiat 48:332, 1985.
28. Sghirlanzoni A, Peluchetti D, Mantegazza R, Fiacchino F, Cornelio F - Myasthenia gravis: prolonged treatment with steroids. Neurology 34:170, 1984.
29. Simpson JA - An evaluation of thymectomy in myasthenia gravis. Brain 81:112, 1958.
30. Simpson JA - Myasthenia gravis: a new hypothesis. Scot Med J 5:419, 1960.
31. Souroujon MC, Pachner AR, Fuchs S - The treatment of passively transferred experimental myasthenia with ariti-idiotypic antibodies. Neurology 36:622, 1986.
32. Spence PA, Morin JE, Katz M - Role of plasmapheresis in preparing myasthenic patients for thymectomy: inicial results. Canad J Surg 27:303, 1984.
33. Tejedor ED, Orozco JP - Miastenia grave y síndromes miasténicas. In Congreso Nacional de Neurología (jun, 1984). Anais, San Sebastián, 1984.
34. 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 316:719, 1987.
35. Thorlacius S, Aaarli JA, Jacobsen H, Halvorsen K - Plasma excnange in myasthenia gravis: clinical effect. Acta Neurol Scand 72:464, 1985.
36. Werneck LC, Teive HAG - Miastenia grave: avaliação clínica e terapêutica de 55 casos. Arq Neuro-Psiquiat (São Paulo) 45:379, 1987.

Datas de Publicação

Publicação nesta coleção
06 Jun 2011
Data do Fascículo
Mar 1989

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Assis JL, Marchiori PE, Scaff M, Zambon AA - Tratamento conservador e timectomia na miastenia grave. Arq Neuro-Psiquiat (São Paulo) 45:119, 1987.

[2] 2. Bever CT, Aquino AV, Penn AS, Lovelace RE, Rowland LP - Prognosis of ocular myasthenia. Ann Neurol 14:516, 1983.

[3] 3. Buckingham JN, Howard FM Jr, Bernatz PE, Spencer Payne W, Harrison EG Jr, O'Brien PC, Weiland LH - The value of thymectomy in myasthenia gravis: a computer assisted matched study. Ann Surg 184:453, 1976.

[4] 4. Dau PC, Lindstrom JM, Cassel CK, Denys EH, Shev EE, Spitler LE - Plasmapheresis and immunossuppressive drug therapy in myasthenia gravis. New Engl J Med 297:1135, 1977.

[5] 5. Drachman DB - Myasthenia gravis. New Engl J Med 298:136, 1978.

[6] 6. Drachman DB - Myasthenia gravis. New Engl J Med 298:186, 1978.

[7] 7. Drachman DB - Present and future treatment of myasthenia gravis. New Engl J Med 316:743, 1987.

[8] 8. Engel AG, Lambert EH, Howard FM - Immune complexes (IgG and C₃) at the motor end-plate in myasthenia gravis: ultra-strutural and light microscopic localization and electrophysiologic correlations. Mayo Clin Proc 52:267, 1977.

[9] 9. Engel AG - Myasthenia gravis and myasthenic syndromes. Ann Neurol 16:519, 1984.

[10] 10. Hankins JR, Mayer RF, Satterfield JR, Turney SZ, Attar S, Sequeira AJ, Thompson BW, McLaughlin JS - Thymectomy for myasthenia gravis: 14 -year experience. Ann Surg 201:618, 1985.

[11] 11. Haynes BF, Harden EA, Olanow CW, Eisenbarth GS, Wechsler AS, Hensley LL, Roses AD - Effect of thymectomy on peripheral lymphocyte subsets in myasthenia gravis: selective effect on T cells in patients with thymic atrophy. J Immunol 131:773, 1983.

[12] 12. Jacobsen H, Thorlacius S, Aaarli JA - Plasmapheresis in myasthenia gravis: clinical results and changes in serum proteins. Acta Neurol Scand 65(Suppl 90):128, 1982.

[13] 13. Kurtzke JF - Epidemiology of myasthenia gravis. Advances in Neurology, vol 19. Raven Press, New York, 1978.

[14] 14. Lisak RP, Barchi RL - Myasthenia gravis. Major Problems in Neurology, vol 11, 1982.

[15] 15. Matell G - Myasthenia gravis: an autoimmune receptor disease. Acta Neurol Scand 65(Suppl 90):112, 1982.

[16] 16. McQuillen MP, Leone MG - A treatment carol: thymectomy revisited. Neurology 27:1103, 1977.

[17] 17. Moore HJ, Woods EL - Myasthenia gravis: associated antibodies in asymptomatic thymoma. J Thorac Cardiovasc Surg 89:308, 1985-

[18] 18. Munsat TL - Anticholinesterase abuse in myasthenia gravis. J Neurol Sci 64:5, 1984.

[19] 19. Namba T, Brunner NG, Grob D - Myasthenia gravis in patients with thymoma, with particular reference to onset after thymectomy. Medicine 57:411, 1978.

[20] 20. Newsom Davis J, Vicent A, Ward CD, Wilson SG, Pinching AJ - Plasmapheresis exchange: short and long-term benefits. In Dau PC (ed): Plasmapheresis and the Immunobiolgy of Myasthenia Gravis. Houghton Mifflin, Boston, 1979, pg 199.

[21] 21. Niakam E, Harati Y, Rolak LA - Immunossuppressive drug therapy in myasthenia gravis. Arch Neurol 43:155, 1986.

[22] 22. Osserman KE - Myasthenia Gravis. Grune & Stratton, New York, 1958, pg 78, 86.

[23] 23. Pascuzzi RM, Branch Coslett H, Johns TR - Long-term corticosteroid treatment of myasthenia gravis: report of 116 patients. Ann Neurol 15:291, 1984.

[24] 24. Pinching AJ, Peters DK, Newson-Davis J - Remission of myasthenia gravis following plasma exchange. Lancet 2:1373, 1976.

[25] 25. Rowland LP, Korengold MC, Jaffe IA, Berg L, Shy GM - Prostigmine induced muscle weakness in myasthenia gravis patients. Neurology 5:89, 1955.

[26] 26. Scadding GK, Havard CWH, Lange MJ, Domb I - The long term experience of thymectomy for myasthenia gravis. J Neurol Neurosurg Psychiat 48:401, 1985.

[27] 27. Schumm F, Wiethölter H, Moghadam AF, Dichgan J - Thymectomy in myasthenia with pure ocular symptoms. J Neurol Neurosurg Psychiat 48:332, 1985.

[28] 28. Sghirlanzoni A, Peluchetti D, Mantegazza R, Fiacchino F, Cornelio F - Myasthenia gravis: prolonged treatment with steroids. Neurology 34:170, 1984.

[29] 29. Simpson JA - An evaluation of thymectomy in myasthenia gravis. Brain 81:112, 1958.

[30] 30. Simpson JA - Myasthenia gravis: a new hypothesis. Scot Med J 5:419, 1960.

[31] 31. Souroujon MC, Pachner AR, Fuchs S - The treatment of passively transferred experimental myasthenia with ariti-idiotypic antibodies. Neurology 36:622, 1986.

[32] 32. Spence PA, Morin JE, Katz M - Role of plasmapheresis in preparing myasthenic patients for thymectomy: inicial results. Canad J Surg 27:303, 1984.

[33] 33. Tejedor ED, Orozco JP - Miastenia grave y síndromes miasténicas. In Congreso Nacional de Neurología (jun, 1984). Anais, San Sebastián, 1984.

[34] 34. 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 316:719, 1987.

[35] 35. Thorlacius S, Aaarli JA, Jacobsen H, Halvorsen K - Plasma excnange in myasthenia gravis: clinical effect. Acta Neurol Scand 72:464, 1985.

[36] 36. Werneck LC, Teive HAG - Miastenia grave: avaliação clínica e terapêutica de 55 casos. Arq Neuro-Psiquiat (São Paulo) 45:379, 1987.