Miastenia grave: avaliação clinica e terapêutica de 55 casos

Werneck, Lineu Cesar; Teive, Hélio A. Ghizoni

doi:10.1590/S0004-282X1987000400004

Resumos

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).

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.

Miastenia grave: avaliação clinica e terapêutica de 55 casos

Myasthenia gravis: clinical and therapeutic evaluation of 55 cases

Lineu Cesar Werneck^I; Hélio A. Ghizoni Teive^II

^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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Especialidade de Neurologia, Hospital das Clínicas - Rua General Carneiro 181, 139 andar - 80000 - Curitiba, PR Brasil.

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Publicação nesta coleção
22 Jun 2011
Data do Fascículo
Dez 1987

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[1] 1. ADAMS, S.L.; MATHEWS, J. & GRAMMER, L.C. - Drugs that may exacerbate myasthenia gravis. Ann. Emerg. Med. 13:532, 1984.

[2] 2. APPEL., S.H.; ALMON, R.R. & LEVY, N. - Acetylcholine receptor antibodies in myasthenia gravis. N. Engl. J. Med. 293:760, 1975.

[3] 3. ARSURA, E.; BRUNNER, N.G.; NAMBA, T. & GROB, D. - High-dose intravenous methylprednisone in myasthenia gravis. Arch. Neurol. 42:1149, 1985.

[4] 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.

[5] 5. ASSIS, J.L. - Tratamento das formas severas de miastenia pelo ACTH por via intravenosa. Arq. Neuro-Psiquiat. (São Paulo) 18:359, 1960.

[6] 6. ASSIS, J.L. - Corticosteróides no tratamento da miastenia grave. Arq. NeuroPsiquiat. (São Paulo) 33:33, 1975.

[7] 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.

[8] 8. ASSIS, J.L. & CURI, N. - Timectomia na miastenia gravis: avaliação dos resultados em 73 pacientes. Arq. Neuro-Psiquiat. (São Paulo) 36:16, 1978.

[9] 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.

[10] 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.

[11] 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.

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