The author emphasizes the difficulty of the subject since on the one hand there is no assurance about the value of the treatment methods performed commonly at the moment, and on the other hand there is an evident lack of prospectively controlled trials and double-blind studies. Mostly this dilemma occurs by the lack at the moment of any specific treatment. The author deals with the four therapeutic methods most commonly used: cholinergic drugs, thymectomy, immunosuppressive drugs and plasmapheresis. At last, the experimental specific immunotherapy by the use of antibodies and suppressive cells and the irradiation methods are discussed.

-We evaluated the epidemiological, clinical, laboratory and therapeutical aspects of 41 patients with thymomatous myasthenia gravis. Thirty five patients (85.36%) were submitted to thymectomy. Follow-up ranged from two to 18 years. Diagnosis of thymoma was based upon clinical investigations and CT scan of the anterior mediastinum and in 11 patients supported by immunological tests of anti-striated muscle antibodies with a positive result in more than 80% of cases. Histopathologic examination of all thymomectomized patients confirmed the diagnosis of thymoma. There was a significant predominance of benign over malignant thymoma. Occurred higher prevalence of male patients and of patients over 40 years of age. The therapeutical strategy to control myasthenic clinical findings was the same as that for non-thymomatous myasthenia gravis. The corticosteroids associated to cytotoxic drugs were less often used. Radiotherapy of the anterior mediastinum was more often used in patients having invasive tumors submitted to surgery or not. With regard to survival and control of myasthenia gravis, especially in younger patients and in those submitted to early surgery, results of treatment were surprisingly favorable.KEY WORDS: myasthenia gravis, thymoma, thymectomy.Miastenia grave e timoma: avaliação de 41 pacientes RESUMO -Avaliamos 41 pacientes com miastenia grave timomatosa sob os aspectos epidemiológico, clínico e terapêutico. Trinta e cinco pacientes (85,36%) foram timectomizados. O seguimento clínico variou de dois meses até 18 anos. O diagnóstico do timoma foi fundamentado no estudo de imagem do mediastino (tomografia axial computadorizada) e, em 11 pacientes, complementado com a determinação sérica de anticorpos para músculo estriado com resultado positivo em mais de 80% dos casos e confirmado pelo exame anátomo-patológico do timo realizado em todos os pacientes operados. Ocorreu predomínio significante de timomas benignos sobre timomas malignos, forma clínica generalizada severa, frequente envolvimento do sexo masculino e, em pacientes com mais de 40 anos de idade. A estratégia terapêutica para o controle dos sintomas miastênicos foi a mesma que para os pacientes não timomatosos. O emprego de imunossupressão medicamentosa esteróide associada a drogas citotóxicas foi menos frequente. A radioterapia foi usada com mais frequência nos pacientes portadores de tumores invasivos operados ou não. PALAVRAS-CHAVE: miastenia grave, timoma, timectomia.Acquired myasthenia gravis (MG) is an immunological disease with antibody activity against the acetylcholine nicotine receptor (antiAChR) of the neuromuscular junction, with fluctuant weakness of the skeletal muscle which improves with the administration of cholinergic drugs (CD). These antibodies are produced by B lymphocytes activated by helper T lymphocytes antigen-specific (CD4) 1,2 in the thymus and peripheral blood of patients with MG 3,4 .

A retrospective survey of the records of 33 patients with generalized acquired myasthenia gravis treated at Hospital das Clínicas, Medical School of The University of São Paulo, Brazil and in private practice, with and without thymectomy, is reported. Nineteen were thymectomized and 14 non operated. Both groups were demographically homogeneous and were followed from 8 to 24 years. Parameters for evaluating the natural history of disease and other ones for a comparative analysis of the improvement or remissions were established. The influence of the individualized therapy was evaluated by McNemar test (chi 2); the U-Mann-Whitney was used for evaluation of the populational behavior groups; variable parameter with possible influence in the useful life were studied by multivariate analysis. The difference of distance between both groups was not significant: (D2 = 0.08894); T2 = 7.17 (Hottelling test). There were differences of the isolate clinic parameter response in both groups, but the global analysis of the parameters did not permit to discriminate them, possibly because of interaction of the parameters influencing the analytic result. Inspite of the fact that superposition of the parameter occurred when analysed for a long period, we believe the early thymectomy to be beneficial. Our patients who had their operation at a younger age and specially those with recent disease had a maximum improvement.

O desenvolvimento de doenças autoimunes em alguns pacientes tratados com D-penicilamina (DPA) com desordens na junção neuromuscular (JNM)subclínicaO objeto deste registro é apresentar um desses casos.OBSERVAÇÃO M.T.L., 41 anos, branca, procedente de São Paulo, acompanhada no Hospital das Clínicas da FMUSP por apresentar manchas na pele, parestesias nos dedos e fenômeno de Raynaud, desde os 13 anos, e dores poliarticulares. Houve espessamento insidioso da pele e hemiatrofia no dimídio esquerdo. Foi diagnosticada esclerose sistêmica progressiva. Foi submetida a diversas terapêuticas, sem melhora. Há um ano iniciou DPA (250 mg/dia). Seis meses após queixou-se de "visão dupla". O emprego de DPA foi mantido devido a melhora clínica. Posteriormente, semiptose palpebral bilateral, mais acentuada a direita e fraqueza nos quatro membros limitavam seus movimentos. Referia disfagia, tosse e dispnéia esporádica, há 5 anos. Nos antecedentes pessoais, queixava-se de "bronquite" há cinco anos. Exame clínico -emagrecida, pálida, pressão arterial

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