Miastenia grave tratamento clínico x cirúrgico

Werneck, Lineu Cesar; Moreira, Paulo Roberto

doi:10.1590/S0004-282X1991000400007

Resumos

A indicação da timectomia na miastenia grave ainda é controversa e, na atualidade, é difícil encontrar séries comparando tratamento conservador, devido à indicação gene. ralizada da timectomia. Na presente série foram estudados 65 casos divididos em três grupos : (1) 15 pacientes timectomizados e 50 com tratamento conservador; (2) 15 pacientes timectomizados pareados com 15 pacientes semelhantes clinicamente, mas tratados conservadoramente; (3) 49 pacientes tratados com corticosteróides, com 16 sem corticosteróides. Estes três grupos foram comparados quanto a idade, idade de início dos sintomas, tempo de doença, formas clinicas, escala funcional e intensidade dos sintomas, seguidos durante vários anos, estudando-se o número de remissões, melhora, estabilidade ou piora dos sintomas e número de óbitos. Somente tiveram melhora dos sintomas (p<0,05) os timectomizados do grupo 1; os demais parâmetros de todos os grupos não se mostraram estatisticamente significativos. Esses resultados sugerem que o tipo de tratamento pouco interferiu na evolução da miastenia grave neste grupo de pacientes.

The indication for thymectomy in myasthenia gravis still is controversial, and it is uncommon to find in the present days studies comparing conservative treatments, due to the widespread surgical treatment adopted in most centers. We studied 65 cases divided into three groups of patients: (1) 15 thymectomyzed patients and 50 with conservative treatment; (2) 15 thymectomyzed patients pared with 15 on conservative treatment; (3) 49 patients treated with corticosteroids against 16 without corticosteroids. These three groups where compared regarding age, age when the symptoms began, disease duration, clinical severity and functional scale, studying remission, stability or worsening of the symptoms and death rate after several years of treatment. It was found a reduction of the symptoms (p<0.05) in the thymectomized patients of group 1; the remaining parameters of all three groups did not show any statistical significance. These results suggest that the type of treatment did not interfere with evolution of myasthenia gravis in this group of patients.

CONTENTS CONTEÚDO

Lineu Cesar Werneck^I; Paulo Roberto Moreira^II

^IProfessor Adjunto de Neurologia - Trabalho realizado no Serviço de Doenças Musculares, Especialidade de Neurologia, Departamento de Clínica Médica, Hospital de Clínicas (HC), Universidade Federal do Paraná (UFPR)

^IINeurologista, Bolsista do CNPq. Pesquisa mantida pelo CNPq (Bolsa 820224/88-0) - Trabalho realizado no Serviço de Doenças Musculares, Especialidade de Neurologia, Departamento de Clínica Médica, Hospital de Clínicas (HC), Universidade Federal do Paraná (UFPR)

RESUMO

A indicação da timectomia na miastenia grave ainda é controversa e, na atualidade, é difícil encontrar séries comparando tratamento conservador, devido à indicação gene. ralizada da timectomia. Na presente série foram estudados 65 casos divididos em três grupos : (1) 15 pacientes timectomizados e 50 com tratamento conservador; (2) 15 pacientes timectomizados pareados com 15 pacientes semelhantes clinicamente, mas tratados conservadoramente; (3) 49 pacientes tratados com corticosteróides, com 16 sem corticosteróides. Estes três grupos foram comparados quanto a idade, idade de início dos sintomas, tempo de doença, formas clinicas, escala funcional e intensidade dos sintomas, seguidos durante vários anos, estudando-se o número de remissões, melhora, estabilidade ou piora dos sintomas e número de óbitos. Somente tiveram melhora dos sintomas (p<0,05) os timectomizados do grupo 1; os demais parâmetros de todos os grupos não se mostraram estatisticamente significativos. Esses resultados sugerem que o tipo de tratamento pouco interferiu na evolução da miastenia grave neste grupo de pacientes.

SUMMARY

The indication for thymectomy in myasthenia gravis still is controversial, and it is uncommon to find in the present days studies comparing conservative treatments, due to the widespread surgical treatment adopted in most centers. We studied 65 cases divided into three groups of patients: (1) 15 thymectomyzed patients and 50 with conservative treatment; (2) 15 thymectomyzed patients pared with 15 on conservative treatment; (3) 49 patients treated with corticosteroids against 16 without corticosteroids. These three groups where compared regarding age, age when the symptoms began, disease duration, clinical severity and functional scale, studying remission, stability or worsening of the symptoms and death rate after several years of treatment. It was found a reduction of the symptoms (p<0.05) in the thymectomized patients of group 1; the remaining parameters of all three groups did not show any statistical significance. These results suggest that the type of treatment did not interfere with evolution of myasthenia gravis in this group of patients.

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Full text available only in PDF format.

Dr. Lineu C. Werneck - Especialidade Neurologia - Hospital de Clinicas, UFPR - Rua General Carneiro 181, 3º andar - 80000 Curitiba PR - Brasil.

1. Abramsky O, Tarrab R, Aharonov A. Immunosuppression of experimental autoimmune; myasthenia gravis by hydrocortisone and azathioprine. J Immunol 1975, 117:225-228.
2. Arsura E, Brunner NG, Namba T, Grob D. High-dose intravenous methylprednisone in myasthenia gravis. Arch Neurol 1985, 42:1149.
3. Assis JL, Marchiori PE, Zambon AA, Scaff M. Tratamento conservador e timectomia na miastenia grave. Arq Neuro-Psiquiat (São Paulo) 1987, 45:119-130.
4. Berthold CGS, Hans GM, Thomas K. Long-term treatment with azathioprine abolishes thymic lymphoid follicular hyperplasia in myasthenia gravis. Lancet 1987, 19:682.
5. Blalock A. Thymectomia in the treatment of myasthenia gravis: report of 20 cases. J Thorac Surg 1944, 13:316-339.
6. Blalock A, Meason AF, Morgan HJ, Riven SS. Myasthenia gravis and tumours of thymic region: report of case in which tumour was removed . Ann Surg 1939, 110:544-561.
7. Buckingham JM, Howard FM, Bernatz PE, Payne WS, Harrison EGJr, O'Brien PC, Weiland LH. The value of thymectomy in myasthenia gravis: a computer-assisted matched study. Ann Surg 1976, 184:453-458.
8. Daroff RB. Ocular myasthenia: diagnosis and therapy. Neuro-Opthalmol 1980, 10:62-71.
9. Donaldson MD, Ansher M, Horan S, Rutherford MD, Ringel SP. The relationship of age to outcome in myasthenia gravis. Neurology 1990, 40:786-790.
10. Engel AG. Acquired autoimmune myasthenia gravis. In Engel AG, Banker BR (eds) : Myology Basic Clinical, New York: McGraw-Hill, 1986.
11. Fraser K, Simpson JA, Crawford J. The place of surgery in the treatment of myasthenia gravis. Br J Surg 1978, 65:301-304.
12. Geld HV, Oosterhuis HJGH. Autoantibodies and myasthenia gravis: epithelial cells of the thymus. Ann NY Acad Sci 1966, 135:631-637.
13. Genkins G, Papatestas AE, Horowitz SH, Kornfeld P. Studies in myasthenia gravis : early thymectomy. Am J Med 1975, 58:517-524.
14. Grob D, Norman GB, Namba T. The nature course of myasthenia gravis and effect of therapeutic measures. Ann NY Acad Sci 1981, 377:652-669.
15. Hankins JR, Mayer RF, Saterfield JR, Turney SZ, Attar S, Sequeira AJ, Thompson BW, McLaughlin JS. Thymectomy for myasthenia gravis: 14 years experience. Ann Surg 1984, 201:618-624.
16. Haynes BF, Harden EA, Olanow CW, Eisenbarth GS, Weschsler 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 1983, 131:773-777.
17. Lisak RP. Myasthenia gravis: mechanisms and management. Hosp Pract 1983, 18:101-109.
18. Mann JD, Johns TR, Campa JF. Long-term administration of corticosteroids in myasthenia gravis. Neurology 1976, 26:729-740.
19. Mattila S, Pirskanen R, Heinonen J. Thymectomy for myasthenia gravis. Scand J Thorac Cardiovasc Surg 1976, 10-183-188.
20. McQuillen MP, Leone MG. The treatment carol: thymectomy revisited. Neurology 1977, 27:1103-1106.
21. Morgutti M, Conti BM, Sghirlansoni A. Cellular immune response to acetylcholine receptor in myasthenia gravis : II. Thymectomy and corticosteroids. Neurology 1979, 29:734-738.
22. Mossman S, Vincent A, Davis JN. Myasthenia gravis without acetylcholine-receptor antibody : a distinct disease entity. Lancet 1986, 1:116-119.
23. Mulder DG, Herrmann C, Buckberg GD. Effect of thymectomy in patients with myasthenia gravis. Am J Surg 1974, 128:202-206.
24. Namba T, Bruner NG, Grob D. Myasthenia gravis in patients with thymoma, with particular reference to onset after thymectomy. Medicine 1978, 57:411-433.
25. Namba T, Nakata Y, Grob D. The role of cellular and humoral immune factors in the neuromuscular block of myasthenia gravis. Ann NY Acad Sci 1976, 274:493-518.
26. Newson-Davis J, Willcox N, Calder L. Thymus cells in myasthenia gravis: selectively enhanced production of anti-acethylcholine receptor antibody by autologous blood lymphocytes. N Engl J Med 1981, 305:1313-1318.
27. Niakan E, Harati Y, Rolak LA. Immunosuppressive drug therapy in myasthenia gravis. Arch Neurol 1986, 43:155-156.
28. Olanow CW, Wechsler AS, Roses AD. A prospective study of thymectomy and serum acetylcholine receptor antibodies in myasthenia gravis. Ann Surg 1982, 196:113-121.
29. Papatestas AE, Alpert AE. Osserman KE. Studies in myasthenia gravis: effects of thymectomy. Am J Med 1971, 50:465-474.
30. Parkes JD, McKinna JA. Effects of thymic extract on the neuromuscular junction. Nature 1966, 214:1116.
31. Pascuzzi KM, Branch OH, Johns TR. Long-term corticosteroid treatment of myasthenia gravis : report of 116 pacients. Ann Neurol 1984, 15:291-298.
32. Perlo VP, Schwab RS, Viets HR, Poskanzer DA, Osserman KE, Genkins GM Myasthenia gravis: evaluation of treatment in 1355 patients. Neurology 1960, 16:431-439.
33. Reinhard H, Kalias I, Kohleizen B. Myasthenia gravis: stimulation of antireceptor autoantibodies by autoreactive T-cell lines. Neurology 1986, 36:618-621.
34. Richman DP, Patrick J, Arianson BGW. Cellular immunity in myasthenia gravis: response to purified acetylcholine receptor and autologous thymocytes. N Engl J Med 1976, 294:694.
35. Rowland LP. Controversies about the treatment of myasthenia gravis. J Neurol Neu-rosurg Psychiat 1980, 43:644-659.
36. Sanders DB, Johns TR, Ellefrawi ME. Experimental autoimmune myasthenia gravis in rats : modification by thymectomy and prednisolone. Arch Neurol 1977, 34:75-79.
37. Scadding GK, Havard CWH. Pathogenesis and treatment of myasthenia gravis. Br Med J 1981, 283:1008-1012.
38. Soadding GK, Havard CWH, Lance MJ, Comb I. The long term experience of thymectomy for myasthenia gravis. J Neurol Neurosurg Psychiat 1985, 48:401-406.
39. Seybold ME, Drachman DB. Gradual increasing doses of predinisone in myasthenia gravis. N Engl J Med 1974, 290:81-84.
40. Seybold ME, Lindstrom JM. Patterns of acetylcholine receptor antibody fluctuation in myasthenia gravis. Ann NY Acad Sci 1981, 377:292-305.
41. Sghirlanzoni A, Peluchetti D, Mantegazza R. Myasthenia gravis: prolonged treatment with steroids. Neurology 1984, 34:170-174.
42. Simpson JA. An evaluation of thymectomy in myasthenia gravis. Brain 1958, 81:112-144.
43. Simpson JA. Myasthenia gravis. In Walton JN, Scarlato G (eds) : Muscle Diseases. Amsterdam: Excerpta Medica, 1969.
44. Simpson JA. Myasthenia gravis and myasthenic syndromes. In Walton J (eds) : Disorders of Voluntary Muscle New York: Churchill Livingstone, 1981.
45. Smiley JD, Bradley J, Laly D, Ziff M. Immunoglobulin synthesis in vitro by human thymus: comparison of myasthenia gravis and normal thymus. Clin Exp Immunol 1969, 4:387-399.
46. Tosta ED, Fernandes RNM. Miastenia grave: tratamento com timectomia, corticóide e plasmaferese. Arq Neuro-Psiquiat (São Paulo) 1989, 47:39-50.
47. Werneck LC, Teive HAG. Miastenia grave: avaliação clínica e terapêutica de 55 casos. Arq Neuro-Psiquiat (São Paulo) 1987, 45:379-390.

Miastenia grave tratamento clínico x cirúrgico

Myasthenia gravis: surgical x conservative treatment.

Datas de Publicação

Publicação nesta coleção
22 Fev 2011
Data do Fascículo
Dez 1991

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

[1] 1. Abramsky O, Tarrab R, Aharonov A. Immunosuppression of experimental autoimmune; myasthenia gravis by hydrocortisone and azathioprine. J Immunol 1975, 117:225-228.

[2] 2. Arsura E, Brunner NG, Namba T, Grob D. High-dose intravenous methylprednisone in myasthenia gravis. Arch Neurol 1985, 42:1149.

[3] 3. Assis JL, Marchiori PE, Zambon AA, Scaff M. Tratamento conservador e timectomia na miastenia grave. Arq Neuro-Psiquiat (São Paulo) 1987, 45:119-130.

[4] 4. Berthold CGS, Hans GM, Thomas K. Long-term treatment with azathioprine abolishes thymic lymphoid follicular hyperplasia in myasthenia gravis. Lancet 1987, 19:682.

[5] 5. Blalock A. Thymectomia in the treatment of myasthenia gravis: report of 20 cases. J Thorac Surg 1944, 13:316-339.

[6] 6. Blalock A, Meason AF, Morgan HJ, Riven SS. Myasthenia gravis and tumours of thymic region: report of case in which tumour was removed . Ann Surg 1939, 110:544-561.

[7] 7. Buckingham JM, Howard FM, Bernatz PE, Payne WS, Harrison EGJr, O'Brien PC, Weiland LH. The value of thymectomy in myasthenia gravis: a computer-assisted matched study. Ann Surg 1976, 184:453-458.

[8] 8. Daroff RB. Ocular myasthenia: diagnosis and therapy. Neuro-Opthalmol 1980, 10:62-71.

[9] 9. Donaldson MD, Ansher M, Horan S, Rutherford MD, Ringel SP. The relationship of age to outcome in myasthenia gravis. Neurology 1990, 40:786-790.

[10] 10. Engel AG. Acquired autoimmune myasthenia gravis. In Engel AG, Banker BR (eds) : Myology Basic Clinical, New York: McGraw-Hill, 1986.

[11] 11. Fraser K, Simpson JA, Crawford J. The place of surgery in the treatment of myasthenia gravis. Br J Surg 1978, 65:301-304.

[12] 12. Geld HV, Oosterhuis HJGH. Autoantibodies and myasthenia gravis: epithelial cells of the thymus. Ann NY Acad Sci 1966, 135:631-637.

[13] 13. Genkins G, Papatestas AE, Horowitz SH, Kornfeld P. Studies in myasthenia gravis : early thymectomy. Am J Med 1975, 58:517-524.

[14] 14. Grob D, Norman GB, Namba T. The nature course of myasthenia gravis and effect of therapeutic measures. Ann NY Acad Sci 1981, 377:652-669.

[15] 15. Hankins JR, Mayer RF, Saterfield JR, Turney SZ, Attar S, Sequeira AJ, Thompson BW, McLaughlin JS. Thymectomy for myasthenia gravis: 14 years experience. Ann Surg 1984, 201:618-624.

[16] 16. Haynes BF, Harden EA, Olanow CW, Eisenbarth GS, Weschsler 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 1983, 131:773-777.

[17] 17. Lisak RP. Myasthenia gravis: mechanisms and management. Hosp Pract 1983, 18:101-109.

[18] 18. Mann JD, Johns TR, Campa JF. Long-term administration of corticosteroids in myasthenia gravis. Neurology 1976, 26:729-740.

[19] 19. Mattila S, Pirskanen R, Heinonen J. Thymectomy for myasthenia gravis. Scand J Thorac Cardiovasc Surg 1976, 10-183-188.

[20] 20. McQuillen MP, Leone MG. The treatment carol: thymectomy revisited. Neurology 1977, 27:1103-1106.

[21] 21. Morgutti M, Conti BM, Sghirlansoni A. Cellular immune response to acetylcholine receptor in myasthenia gravis : II. Thymectomy and corticosteroids. Neurology 1979, 29:734-738.

[22] 22. Mossman S, Vincent A, Davis JN. Myasthenia gravis without acetylcholine-receptor antibody : a distinct disease entity. Lancet 1986, 1:116-119.

[23] 23. Mulder DG, Herrmann C, Buckberg GD. Effect of thymectomy in patients with myasthenia gravis. Am J Surg 1974, 128:202-206.

[24] 24. Namba T, Bruner NG, Grob D. Myasthenia gravis in patients with thymoma, with particular reference to onset after thymectomy. Medicine 1978, 57:411-433.

[25] 25. Namba T, Nakata Y, Grob D. The role of cellular and humoral immune factors in the neuromuscular block of myasthenia gravis. Ann NY Acad Sci 1976, 274:493-518.

[26] 26. Newson-Davis J, Willcox N, Calder L. Thymus cells in myasthenia gravis: selectively enhanced production of anti-acethylcholine receptor antibody by autologous blood lymphocytes. N Engl J Med 1981, 305:1313-1318.

[27] 27. Niakan E, Harati Y, Rolak LA. Immunosuppressive drug therapy in myasthenia gravis. Arch Neurol 1986, 43:155-156.

[28] 28. Olanow CW, Wechsler AS, Roses AD. A prospective study of thymectomy and serum acetylcholine receptor antibodies in myasthenia gravis. Ann Surg 1982, 196:113-121.

[29] 29. Papatestas AE, Alpert AE. Osserman KE. Studies in myasthenia gravis: effects of thymectomy. Am J Med 1971, 50:465-474.

[30] 30. Parkes JD, McKinna JA. Effects of thymic extract on the neuromuscular junction. Nature 1966, 214:1116.

[31] 31. Pascuzzi KM, Branch OH, Johns TR. Long-term corticosteroid treatment of myasthenia gravis : report of 116 pacients. Ann Neurol 1984, 15:291-298.

[32] 32. Perlo VP, Schwab RS, Viets HR, Poskanzer DA, Osserman KE, Genkins GM Myasthenia gravis: evaluation of treatment in 1355 patients. Neurology 1960, 16:431-439.

[33] 33. Reinhard H, Kalias I, Kohleizen B. Myasthenia gravis: stimulation of antireceptor autoantibodies by autoreactive T-cell lines. Neurology 1986, 36:618-621.

[34] 34. Richman DP, Patrick J, Arianson BGW. Cellular immunity in myasthenia gravis: response to purified acetylcholine receptor and autologous thymocytes. N Engl J Med 1976, 294:694.

[35] 35. Rowland LP. Controversies about the treatment of myasthenia gravis. J Neurol Neu-rosurg Psychiat 1980, 43:644-659.

[36] 36. Sanders DB, Johns TR, Ellefrawi ME. Experimental autoimmune myasthenia gravis in rats : modification by thymectomy and prednisolone. Arch Neurol 1977, 34:75-79.

[37] 37. Scadding GK, Havard CWH. Pathogenesis and treatment of myasthenia gravis. Br Med J 1981, 283:1008-1012.

[38] 38. Soadding GK, Havard CWH, Lance MJ, Comb I. The long term experience of thymectomy for myasthenia gravis. J Neurol Neurosurg Psychiat 1985, 48:401-406.

[39] 39. Seybold ME, Drachman DB. Gradual increasing doses of predinisone in myasthenia gravis. N Engl J Med 1974, 290:81-84.

[40] 40. Seybold ME, Lindstrom JM. Patterns of acetylcholine receptor antibody fluctuation in myasthenia gravis. Ann NY Acad Sci 1981, 377:292-305.

[41] 41. Sghirlanzoni A, Peluchetti D, Mantegazza R. Myasthenia gravis: prolonged treatment with steroids. Neurology 1984, 34:170-174.

[42] 42. Simpson JA. An evaluation of thymectomy in myasthenia gravis. Brain 1958, 81:112-144.

[43] 43. Simpson JA. Myasthenia gravis. In Walton JN, Scarlato G (eds) : Muscle Diseases. Amsterdam: Excerpta Medica, 1969.

[44] 44. Simpson JA. Myasthenia gravis and myasthenic syndromes. In Walton J (eds) : Disorders of Voluntary Muscle New York: Churchill Livingstone, 1981.

[45] 45. Smiley JD, Bradley J, Laly D, Ziff M. Immunoglobulin synthesis in vitro by human thymus: comparison of myasthenia gravis and normal thymus. Clin Exp Immunol 1969, 4:387-399.

[46] 46. Tosta ED, Fernandes RNM. Miastenia grave: tratamento com timectomia, corticóide e plasmaferese. Arq Neuro-Psiquiat (São Paulo) 1989, 47:39-50.

[47] 47. Werneck LC, Teive HAG. Miastenia grave: avaliação clínica e terapêutica de 55 casos. Arq Neuro-Psiquiat (São Paulo) 1987, 45:379-390.