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Arquivos de Neuro-Psiquiatria
Print version ISSN 0004-282X
Arq. Neuro-Psiquiatr. vol.45 no.4 São Paulo Dec. 1987
http://dx.doi.org/10.1590/S0004-282X1987000400004
Miastenia grave: avaliação clinica e terapêutica de 55 casos
Myasthenia gravis: clinical and therapeutic evaluation of 55 cases
Lineu Cesar WerneckI; Hélio A. Ghizoni TeiveII
IProfessor Adjunto de Neurologia - Trabalho realizado na Especialidade de Neurologia do Departamento de Clínica Médica, Hospital de Clínicas, Universidade Federal do Paraná - (UFPR), Curitiba
IIResidente de Neurologia - Trabalho realizado na Especialidade de Neurologia do Departamento de Clínica Médica, Hospital de Clínicas, Universidade Federal do Paraná - (UFPR), Curitiba
RESUMO
Foram estudados 55 casos de miastenia grave, avaliando os sinais e sintomas clínicos que ocorreram no início e na evolução dos pacientes, bem como os procedimentos diagnósticos e avaliação das medidas terapêuticas utilizadas. No manejo dos pacientes foram utilizados anticolinesterásicos, corticoesteróides, azathioprina, plasmaferese e timectomia, sem que fosse possível encontrar relação estatística significante entre os diferentes procedimentos com respeito à resolução da doença. Dos 55 casos, 9 (16,6%) obtiveram remissão total, 41 (74,5%) permaneceram com a doença ativa necessitando de tratamento sintomático e 5 (9.09%) faleceram. Houve relação estatística significante entre os sinais de insuficiência respiratória, crises miastênicas e timoma somente no grupo de pacientes que faleceram (um por insuficiência respiratória restritiva secundária a cifoescoliose severa, três com timoma e um caso durante a timectomia).
SUMMARY
It is reported the study of 55 myasthenia gravis cases, with a review of the symptoms and signals, comparing initial symptoms ant those found at the time of the first evaluation. The mean age of the patients was 31.05 ± 18.46 and the mean time of follow-up 5.88 ± 5.47 years. Also the results of the diagnostic tests is reported, as well as the results of the treatment. The patients were treated with anticholinesterasics, corticoesteroids, azathioprine, plasma-exchange and thymectomy. A statistical analysis with the chi-square test was unable to show a difference between the above procedures, regarding the definitive resolution of the disease. From the 55 cases, 9 (16.6%) had total remission, 41 (74.5%) remained with symptoms and need some kind of treatment, and 5 (9.09%) died (three patients with thymoma, one patient with congenital myasthenia gravis, severe cyphoscoliosis and pulmonary restriction, and one patient during the thymectomy procedure). It was found a relation between the signs of respiratory insuficiency, myasthenic crisis and thymoma only in the group of patients who died. A review about the diagnostic methods and treatment in the current days is made.
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REFERÊNCIAS
1. ADAMS, S.L.; MATHEWS, J. & GRAMMER, L.C. - Drugs that may exacerbate myasthenia gravis. Ann. Emerg. Med. 13:532, 1984. [ Links ]
2. APPEL., S.H.; ALMON, R.R. & LEVY, N. - Acetylcholine receptor antibodies in myasthenia gravis. N. Engl. J. Med. 293:760, 1975. [ Links ]
3. ARSURA, E.; BRUNNER, N.G.; NAMBA, T. & GROB, D. - High-dose intravenous methylprednisone in myasthenia gravis. Arch. Neurol. 42:1149, 1985. [ Links ]
4. ARSURA, E.L.; BICK, A.; BRUNNER, N.G.; NAMBA, T. & GROB, D. - Highdose intravenous immunoglobulin in the management of myasthenia gravis. Arch, int. Med. 146:1365, 1986. [ Links ]
5. ASSIS, J.L. - Tratamento das formas severas de miastenia pelo ACTH por via intravenosa. Arq. Neuro-Psiquiat. (São Paulo) 18:359, 1960. [ Links ]
6. ASSIS, J.L. - Corticosteróides no tratamento da miastenia grave. Arq. NeuroPsiquiat. (São Paulo) 33:33, 1975. [ Links ]
7. ASSIS, J.L. - Miastenia grave: evolução das pesquisas na Clínica Neurológica do Hospital das Clínicas da Faculdade de Medicina da USP. Arq. Neuro-Psiquiat. (São Paulo) 44:406, 1986. [ Links ]
8. ASSIS, J.L. & CURI, N. - Timectomia na miastenia gravis: avaliação dos resultados em 73 pacientes. Arq. Neuro-Psiquiat. (São Paulo) 36:16, 1978. [ Links ]
9. ASSIS, J.L.; MARCHIORI, P.E.; SCAFF, M. & ZAMBOM, A.A. - Tratamento da miastenia grave mediante imunossupressão medicamentosa não esteróide. Arq. Neuro-Psiquiat. (São Paulo) 44:109, 1986. [ Links ]
10. ASSIS, J.L,.; MARCHIORI, P.E.; ZAMBOM, A.A.; CURY, N.; FILOMENO, L.T.B. & SCAFF, M. - Immunossupression with corticoesteroids and thymectomy in myasthenia gravis and evaluation of immediate and short therm results in 20 patients. Arq. Neuro-Psiquiat. (São Paulo) 43:17, 1985. [ Links ]
11. ASSIS, J.L.; MARCHIORI, P.E.; ZAMBOM, A.A. & SCAFF, M. - Doenças associadas a miastenia grave em um grupo de 304 pacientes. Arq. Neuro-Psiquiat. (São Paulo) 42:221, 1984. [ Links ]
12. ASSIS, J.L.; SCAFF, M.; ZAMBOM, A.A. & MARCHIORI, P.E. - Doenças da tireóide e miastenia grave. Arq. Neuro-Psiquiat. (São Paulo) 42:226, 1984. [ Links ]
13. BEHAN, P.O. - Combined plasma exchange and immunossupression in myasthenia gravis. Lancet 2:741, 1969. [ Links ]
14. BEHAN, P.O.; SHAKIR, R.A.; SIMPSON, J.A.; BURNETT, A.K.; ALLAN, T.L. & HAASE, G. - Plasma-exchange combined with immunosuppresive therapy in myasthenia gravis. Lancet 1:438, 1979. [ Links ]
15. BLALOCK, A.; MASON, M.F.; MORGAN, H.J. & RIVEN, S.S. - Myasthenia gravis and tumors of the thymic region: report of a case in which the tumor was removed. Ann. Surg. 110:544, 1939. [ Links ]
16. BOWEN, M. & PABST, H.F. - Immunologic response in vitro after thymectomy in patients with myasthenia gravis. CM.A. Journal 115:619, 1976. [ Links ]
17. BRADLEY, R.J. - Myasthenia gravis and anti-idiotype theory of autoimmunity. Lancet 1:396, 1984. [ Links ]
18. BRUNNER, N.G.; NAMBA, T. & GROB, D. - Corticosteroids in management of severe, generalized myasthenia gravis: effectiveness and comparison with corticotropin therapy. Neurology 22:603, 1972. [ Links ]
19. BUCKINGHAM, J.N.; HOWARD, F.M.; BERNATZ, P.E.; PAYNE, W.S.; HARRISON E.G.; O BRIEN, P.C. & WEILAND, L.H. - The value of thymectomy in myasthenia gravis: a computer assisted matched study. Ann. Surg. 184:453, 1976. [ Links ]
20. CURRIER, R.D.; ROUTH A.; HICKNAB, B. & DOUGLAS, M.A. - Thymus irradiation for myasthenia gravis. Radiology 146:199, 1983. [ Links ]
21. DAU, P.C.; LINDSTROM, J.M.; CASSEL, C.K.; DENYS, E.H.; SHEV, E.E. & SPIT LER, L.E. - Plasmapheresis and immunosuppressive drug therapy in myasthenia gravis. N. Engl. J. Med. 297:1134, 1977. [ Links ]
22. DRACHMANN, D.B. - Myasthenia gravis and thyroid gland. N. Engl. J. Med. 266:330, 1962. [ Links ]
23. DRACHMANN, D.B. - Myasthenia gravis. N. Engl. J. Med. 298:136, 1978. [ Links ]
24. DRACHMANN, D.B. - Myasthenia gravis. N. Engl. J. Med. 298:186, 1978. [ Links ]
25. DRACHMANN, D.B.; ANGUS, C.W.; ADAMS, R.N., MICHELSON, J.D. & HOFFMAN, G.J. - Myasthenic antibodies cross-link acetylcholine receptors to accelerate degradation. N. Engl. J. Med. 298:1116, 1978. [ Links ]
26. DURELLI, L.; COCITO, D. & BERGAMINI, L. - Rapid improvement ot myasthenia gravis after plasma exchange. Ann. Neurol. 13:220, 1983. [ Links ]
27. ENGEL, A.G. - Acquired autoimmune myasthenia gravis. In A.G. Engel & B.R. Banker: Myology, Basic and Clinical. McGraw-Hill, New York, 1986. [ Links ]
28. ENGEL, A.G. - Myasthenia gravis and myasthenic syndromes. Ann. Neurol. 16:519, 1984. [ Links ]
29. ENGEL, A.G.; LAMBERT, E.H. & HOWARD, F.M. - Immune complexes (IgG and C3) at the motor end-plate in myasthenia gravis. Mayo Clin. Proceed. 52:267, 1977. [ Links ]
30. ENGEL, W.K.; FESTOFF, B.W.; PATTEN, B.M.; SWERDLOW, M.L.; NEWBALL, H.H. & THOMPSON, M.D. - Myasthenia gravis. Ann. int. Med. 81:225, 1974. [ Links ]
31. Early thymectomy for myasthenia gravis. (Editorial) Brit. med. J. 2:262, 1975. [ Links ]
32. FATEH-MOGHADAM, A.; BESINGER, U. & GEURSEN, R.G. - High-dose intravenous gammaglobulin for myasthenia gravis. Lancet 1:848, 1984. [ Links ]
33. GAJDOS, P.H.; OUTIN, H.; ELKHARRAT, D.; BRUNEL, D.; ROHAN-CHABOT, P.; RAPHAEL, J.C.; GOULON, M.; GOULON-GOEAU, C. & MOREL, E. - High-dose intravenous gammaglobulin for myasthenia gravis. Lancet 1:406, 1984. [ Links ]
34. GELD, H. & OOSTERHUIS, H.J.G.H. - Autoantibodies and myasthenia gravis: epithelial cell of the thymus. Ann. N.Y. Acad. Sei. 135:631, 1966. [ Links ]
35. GENKINS, G.; PAPATESTAS, A.E.; HOROWITZ, S,H. & KORNFELD, P. - Studies in myasthenia gravis: early thymectomy. Electrophysiologic and pathologic correlations. Amer. J. Med. 58:517, 1975. [ Links ]
36. GOLDMAN, A.J.; HERRMANN, C.; KEESEY, J.C.; MULDER, D.G. & BROWN, W.J. - Myasthenia gravis and invasive thymoma: a 20-year experience. Neurology 25:1021, 1975. [ Links ]
37. GROB, D.; BRUNNER, N.G. & NAMBA, T. - The natural course of myasthenia gravis and effect of therapeutic measures. Ann. N.Y. Acad. Sei. 377:652, 1981. [ Links ]
38. GROB, D. & NAMBA, T. - Corticotropin in generalized myasthenia gravis. J. Amer. med. Assoc. 198:127, 1966. [ Links ]
39. HERMANN, J.R.; LINDSTRON, J.M.; KEESEY, J.C. & MULDER, D.G. -Myasthenia gravis: current concepts. Westn. J. Med. 142:797, 1985. [ Links ]
40. HOHFELD, R.; KALI ES, I.; KOHLE.ISEN, B.; HEININGER, K.: CONTITRONCONI, B. & TOYKA, K.V. - Myasthenia gravis: stimulation of antireceptor autoantibodies by autoreactive T cell lines Neurology 36:618, 1986. [ Links ]
41. JOHNS, T.R.; CROWLEY, W. J.; MILLER, J.Q. & CAMPA, F. - The syndrome of myasthenia and polymyositis with comments on therapy. Ann. N.Y. Acad. Sei. 183:64, 1971. [ Links ]
42. JOHNS II, T.R. - Managing patients who have myasthenia gravis. Westn. J. Med. 142:810, 1985. [ Links ]
43. KAO, I. & DRACHMAN, D.B. - Thymic muscle cells bear acetylcholine receptors: possible relation to myasthenia gravis. Science 196:74, 1977. [ Links ]
44. KAO, I. & DRACHMAN, D.B. - Myasthenic immunoglobulin accelerates acetylcholine receptor degradation. Science 196:527, 1977. [ Links ]
45. KELLY, J.J.; DAUBE, J.B.; LENNON, V.A.; HOWARD, F.M. & YOUNGE, R. - The laboratory diagnosis of mild myasthenia gravis. Ann. Neurol. 23:238, 1982. [ Links ]
46. KIMURA, J. - Electrodiagnosis in Diseases of Nerve and Muscle: Principles and Practice. F.A. Davis, Philadelphia, 1983. [ Links ]
47. KORNFELD, P.; AMBINDER, E.P.; PAPATESTAS, A.E.; BENDER, A.N. & GENKINS, G. - Plasmapheresis in myasthenia gravis: controled study. Lancet 2:629, 1979. [ Links ]
48. LEE, K.O.; GUAN, R.; EE, B. & CHEAH, J.S. - Thyrotoxicosis, myasthenia gravis and periodic paralysis in a Chinese man. Postgrad. Med. J. 61:49, 1985. [ Links ]
49. LISAK, R.P. - Myasthenia gravis: mechanisms and management. Hosp. Practice 18:101, 1983. [ Links ]
50. LISAK, R.P.; LATAMORE, C.; LEVINSON, A.I.; ZWEIMAN, B. & MOSKOVITZ, A.R. - Suppressor T Cells in myasthenia gravis and antibodies to acetylcholine receptor. Ann. Neurol. 19:87, 1986. [ Links ]
51. LUNDH, H.; NILSSON, O. & ROSEN, I. - Effects of 4-aminopyridine in myasthenia gravis. J. Neurol. Neurosurg. Psychiat. 42:171, 1979. [ Links ]
52. MANN, J.D.; JOHNS, T.R. & CAMPA, J.F. - Long-term administration of corticoesteroids in myasthenia gravis. Neurology 26:729, 1976. [ Links ]
53. MARCHIORI, P.E.; SCAFF, W.; COSSERMELLI, W. & ASSIS, J.L. - Miastenia grave induzida por d-penicilamina em paciente com esclerose sistêmica progressiva. Arq. Neuro-Psiquiat. (São Paulo) 42:380, 1984. [ Links ]
54. MATTILA, S.; PIRSKANEN, R. & HEINONEM, J. - Thymectomy for myasthenia gravis. Scand. J. thor. cardiovasc. Surg. 10:183, 1976. [ Links ]
55. McQUILLEN, M.P. - Prednisone schedule for myasthenia gravis. N. Engt. J. Med. 290:631, 1974. [ Links ]
56. McQUILLEN, M.P. & LEONE, M.G. - A treatment carol: thymectomy revisited. Neurology 27:1103, 1977. [ Links ]
57. MILLER, R.G. - Plasmapheresis in myasthenia gravis. Ann. Neurol. 9:396, 1981. [ Links ]
58. MULDER, D.H.; HERRMANN, C. & BUCKBERG, G.D. - Effect of thymectomy in patients with myasthenia gravis: a sixteen year experience. Amer. J. Surg. 128:202, 1974. [ Links ]
59. NAKAN, E.; HARATI, Y. & ROLAK, L.A. - Immunosuppressive drug therapy in myasthenia gravis. Arch. Neurol. 43:155, 1986. [ Links ]
60. NAMBA, T.; BRUNNER, N. & GROB, D. - Myasthenia gravis in patients with thymoma, with particular reference to onset after thymectomy. Medicine 57:411, 1978. [ Links ]
61. NEWSON-DAVIS, J.; PINCHING, A. J.; VINCENT, A. & WILSON, S.G. - Function of circulating antibody to acetylcholine receptor in myasthenia gravis: investigation by plasma exchange. Neurology 28:266, 1978. [ Links ]
62. NEWSON-DAVIS, J.; WILSON, S.G.; VINCENT, A. & WARD, C. - Long-therm effects of repeated plasma exchange in myasthenia gravis. Lancet 1:464, 1979. [ Links ]
63. OOSTERHUIS, H.J.G.H. & HAAS, W.H.D. - Rheumatic diseases in patients with myasthenia gravis. Acta neurol. scand. 44:219, 1968. [ Links ]
64. OSSERMAN, K.E. & GENKINS, G. - Critical reappraisal of the. use of edrophonium chloride tests in myasthenia gravis and significance of clinical classification. Ann. N.Y. Acad. Sci. 135:312, 1966. [ Links ]
65. OSSERMAN, K.E. & GENKINS, G. - Studies in myasthenia gravis. J. amer. med. Assoc. 198:123, 1966. [ Links ]
66. OSSERMAN, K.E. & KAPLAN, L.I. - Rapid diagnostic test for myasthenia gravis: increased muscle strenght, without fasciculations, after intravenous administration of edrophonium chloride. J. Amer. med. Assoc. 150:265, 1952. [ Links ]
67. OSSERMAN, K.E. & TENG, P. - Studies in myasthenia gravis: a rapid diagnostic test. Further progress with edrophonium chloride. J. Amer. med. Assoc. 160:153, 1956. [ Links ]
68. OZDEMIR, C. & YOUNG, R.R. - Electrical testing in myasthenia gravis. Ann. N.Y. Acad. Sci. 183:287, 1971. [ Links ]
69. PAPATESTAS, A.E.; ALPERT, L.I.; OSSERMAN, K.E.; OSSERMAN, R.S, & KARK, A.E. - Studies in myasthenia gravis: effects of thymectomy. Results on 185 patients with nonthymomatous and thymomatous myasthenia gravis, 1941-1969. Amer. J. Med. 50:465, 1971. [ Links ]
70. PASCUZZI, R.M.; COSLETT, H.B. & JOHNS, T.R. - Long-therm corticosteroid treatment of myasthenia gravis: report of 116 patients. Ann. Neurol. 15:291, 1984. [ Links ]
71. PERLO, V.P.; ARNASON, B.; POSKANZER, D.; CASTLEMAN, B.; SCHWAB, R.S.; OSSERMAN, K.E.; PAPATESTIS, A.; ALPERT, L. & KARK, A. - The role of thymectomy in the treatment of myasthenia gravis. Ann. N.Y. Acad. Sci. 183:308, 1971. [ Links ]
72. PINCHING, A. J.; PETERS, D.K. & NEWSON-DAVIS, J. - Remission of myasthenia gravis following plasma-exchange. Lancet 2:1373, 1976. [ Links ]
73. REIS, G.; LILJESTRAND, A. & MATELL, G. - Treatment of severe myasthenia gravis with large doses of ACTH. Ann. N.Y. Acad. Sci. 135:409, 1966. [ Links ]
74. ROWLAND, L.P. - Immunosuppressive drugs in treatment of myasthenia gravis. Ann. N.Y. Acad. Sci. 183:350, 1971. [ Links ]
75. ROWLAND, L.P. - Contr versies about the treatment of myasthenia gravis. J. Neurol. Neurosurg. Psychiat. 43:644, 1980. [ Links ]
76. SCADDING, G.K. & HAVARD, C.W. - Pathogenesis and treatment of myasthenia gravis. Brit. med. J. 283:1008, 1981. [ Links ]
77. SEYBOLD, M.E. - Myasthenia gravis: a clinical and basic review. J. Amer. med. Assoc. 250:2516, 1983. [ Links ]
78. SEYBOLD, M.E. & DRACHMAN, D.B. - Gradually increasing doses of prednisone in myasthenia gravis. N. Engl. J. Med. 290:81, 1974. [ Links ]
79. SGHIRLANZONI, A.; PELUCHETTI, D.; MANTEGAZZA, R.; FIACCHINO, F. & CORNELIO, F. - Myasthenia gravis: prolonged treatment with steroids. Neurolgy 34:170, 1984. [ Links ]
80. SIMPSON, J.A. - Myasthenia gravis and myasthenic syndromes. In J. Walton: Disorders of Voluntary Muscle. Churchill & Livingstone, Edinburg, 1981. [ Links ]
81. SOPPI, E.; ESKOLA, J.; ROYTTA, M.; VEROMAA, T.; FANELIUS, M. &. LEHTONEN, A. - Thymoma with immunodeficiency (Good's syndrome) associated with myasthenia gravis and bening IgG gammopathy. Arch. int. Med. 145:1704, 1985. [ Links ]
82. SOUROUJON, M.C.; PACHNER, A.R. & FUCHS, S. - The treatment of passively transferred experimental myasthenia with anti-idiotypic antibodies. Neurology 36:622, 1986. [ Links ]
83. STALBERG, E. - Clinical electrophysiology in myasthenia gravis. J. Neurol. Neurosurg. Psychiat. 43:622, 1980. [ Links ]
84. SWIFT, T.R. - Disorders of neuromuscular transmission other than myasthenia gravis. Muscle & Nerve 4:334, 1981. [ Links ]
85. TAKAMORI, M.; OKTJMURA, S. & YASUDA, A. - Presynaptic modification of neuromuscular transmission by antiacetylcholine receptor antibody: myasthenic serum and monoclonal antibody produced by transformed lymphocytes. Neurology 36:942, 1986. [ Links ]
86. TOYKA, K.V.; DRACHMAN, D.B.; GRIFFIN, D.E.; PESTRONK, A.; WINKELSTEIN, J.A.; FISCHBECK, K.H. & KAO, I. - Myasthenia gravis: study of humoral immune mechanisms by passive transfer to mice. N. Engl. J. Med. 296:125, 1977. [ Links ]
87. VALBONESI, M.; GARELLI, S.; ZERBI, D.; FORLANI, G.; CORNELIO, F. & PELiUCHETTI, D. - Plasma exchange combined with cytotoxic drugs and lymphocytapheresis for myasthenia gravis. Vox Sang. 43:142, 1982. [ Links ]
88. VELDE, R.L. & FRIEDMAN, N.B. - Thymic myoid cells and myasthenia gravis. Amer. J. Path. 59:347, 1970. [ Links ]
89. VETTERS, J.M.; SAIKIA, N.K.; WOOD, J. & SIMPSON, J.A. - Pemphigus vulgaris and myasthenia gravis. Brit. J. Derm. 88:437, 1973. [ Links ]
90. VETTERS, J.M. & SIMPSON, J.A. - Comparison of thymic histology with response to thymectomy in myasthenia gravis. J. Neurol. Neurosurg. Psychiat. 37:1139, 1974. [ Links ]
91. VINCENT, A. & NEWSON-DAVIS, J. - Acetylcholine receptor antibody as a diagnostic test for myasthenia gravis: results in 153 validated cases and 2967 diagnostic assays. J. Neurol. Neurosurg. Psichiat. 48:1246, 1985. [ Links ]
92. WARMOLTS, J.R. & ENGEL, W.K. - Benefit from alternate-day prednisone in myasthenia gravis. N. Engl. J. Med. 286:17, 1972. [ Links ]
93. WARMOLTS, J.R.; ENGEL, W.K. & WHITAKER, J.N. - Alternate-day prednosine in a patient with myasthenia gravis. Lancet 2:1198, 1970. [ Links ]
94. WERNECK, L.C. - A lesão muscular na miastenia grave: estudo de 17 casos com histoquímica muscular. Arq. Neuro-Psiquiat. (São Paulo) 40:67, 1982. [ Links ]
95. WHITING, P.J.; VINCENTE, A. & NEWSON-DAVIS, J. - Myasthenia gravis: monoclonal antihuman acetylcholine receptor antibodies used to analyze antibody specificities and responses to treatment. Neurology 36:612, 1986. [ Links ]
96. WITTE, A.S.; CORNBLATH, D.R.; PARRY, G. J.; LISAK, R.P. & SCHATZ, N.J. - Azathioprine in the treatment of myasthenia gravis. Ann. Neurol. 15:602, 1984. [ Links ]
97. WOLF, S.M. & BARROWS, S. - Myasthenia gravis and systemic lupus erythematosus. Arch. Neurol. 14:254, 1966. [ Links ]
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