José Lamartine de Assis

Atrophy of the tongue with persistent articulation disorder in myasthenia gravis: report of 10 patients.

Ten patients with atrophy of the tongue, from a group of 752 with generalized acquired myasthenia gravis (MG), were studied. Tongue atrophy developed late in the majority of patients and was accompanied by tongue paresis (70% of the cases) and eventually associated to atrophy of other muscles of the palate, especially the uvula. All the patients exhibited severe forms of MG with bulbar involvement, mainly persistent dysphonia and dysphagia, almost always refractory to treatment. There is no correlation among atrophy of the tongue, sex, and thymus pathology. There is correlation between severeness of symptoms and early, persistent and treatment refractory dysphonia and dysphagia.

Myasthenia gravis and thymoma: evaluation of 41 patients

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.

Tratamento da miastenia grave mediante imunossupressão medicamentosa não esteróide

Fourteen patients (twelve of them were women) with severe myasthenia gravis who had not responded to any treatment at all, were treated by cytostatic drugs. Both azathioprine and cyclophosphamide single or in combination are used. All patients except two were thymectomized and almost all were treated by plasmapheresis and corticosteroid before and during the cytostatic treatment. All patients take cholinesterase inhibitors. No serious complications were observed. The patients received 100-200mg of azathioprine and/or 100-200mg of cyclophosphamide daily by oral route, the first during 20 months and the second one during 6 months. Three patients received cyclophosphamide lg daily by venous rute during 6 months at 15-20 days intervals. The preliminary results were favourable occurring important improvement in 71.4% of the cases. Their performance increased considerably. The progress of the disease was generaly stabilized. Exacerbations and crises did not reccur, except in one case. Only three patients had not responded to the treatment, one responded poorly but better later and one had an exacerbation after the 24o month. This patient returned to azathioprine.

Doenças da tireoide e miastenia grave

In a group of 304 myasthenic patients 15 cases with thyropathies were reported: nine with hyperthyroidism, one with hypothyroidism and five with nontoxic goiter. Four patients presented diffuse simple goiter and one a multinodular goiter with normal thyroid function. No patient came from an endemic goiter region, not even familial goiter. The prevalence and influence of hyperthyroidism on myasthenic symptomatology were studied. Our findings suggest that there is no clinical correlation between both myasthenia symptomatology and thyroid dysfunction, neither significant influence on myasthenic symptoms when the endocrine disorders improve.Os autores estudam o comportamento da tireoide em um grupo de 304 pacientes com miastenia grave, constatando 15 casos de tireopatias, sendo 9 de hipertireoidismo, um de hipotireoidismo e 5 de bocio sem alteracoes da funcao tireoidiana. Nenhum paciente era proveniente de regiao de bocio endemico e nao havia bocio familiar. A tireotoxicose, que foi a disfuncao mais frequente, foi estudada quanto a sua prevalencia em pacientes miastenicos, quanto a sua influencia sobre os sintomas da miastenia grave e quanto a epoca do seu aparecimento.

Tratamento dos casos graves de miastenia: ação do ACTH e cortisona. Considerações a propósito de três casos

Sao apresentados 3 casos de miastenia grave em que os recursos terapeuticos classicos nao deram resultado favoravel. Os 3 pacientes foram, entao, submetidos ao tratamento pelo ACTH e cortisona; em todos houve remissao da sintomatologia, remissao que perdurou ate cerca de um mes apos a suspensao da hormonioterapia. O tratamento foi repetido varias vezes de acordo com a evolucao da doenca. E de notar que em 2 pacientes (casos 1 e 2), foram estabelecidas doses minimas de manutencao controladas durante cerca de 40 dias. O ACTH foi usado por via intravenosa em doses variaveis de 2,5 a 25 mg, diluido em 250 a 1000 ml de soro glicosado isotonico, na velocidade de 20 a 25 gotas por minuto. Em todos os casos, durante a hormonioterapia, foi administrada prostigmina em doses muito pequenas.

Tratamento conservador e timectomia na miastenia grave: resultados avaliados a curto e longo prazos

A restrospective survey of the records of 33 patients with generalized aquired myasthenia gravis treated at Hospital das Clinicas, Medical School of The University of Sao 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 (x2); the U-Mann-Whitney was used for evalution 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 responses 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 occured when analysed for a long period, we believe the early thymectomy to be benefic. Our patients who had their operation at a younger age and specially those with recent disease had a maximum improvement.

Miastenia grave: evolução das pesquisas na clínica neurológica do hospital das clínicas da Faculdade de Medicina da USP

The evolution of investigations in Myasthenia gravis in the Division of Neurology, University of Sao Paulo Medical School, during the period from 1956 to 1986 is reported. The most important findings in the therapeutical, clinical and immunological researches are showed.The evolution of investigations in Myasthenia gravis in the Division of Neurology, University of Sao Paulo Medical School, during the period from 1956 to 1986 is reported. The most important findings in the therapeutical, clinical and immunological researches are showed.

Enzyme‐linked immunosorbent assay for detection of antibodies to extractable muscle antigens in myasthenia gravis

ABSTRACT A purified citric acid soluble extract from human skeletal muscle (AEMA) and a phosphate‐buffered saline extract from rabbit muscle acetone powder (EMA) were used to coat polystyrene beads in an enzyme — linked immunosorbent assay (ELISA). From 54 patients with myasthenia gravis, positive results were observed in 14. Five of 6 patients with thymoma had high levels of antibodies. With the diagnostic difficulties in detecting small and medium‐sized thymoma, a sensitive assay for detection of antibodies to muscle antigen may be an important supplementary tool to detect tumors at early stages.

Cisto neuroenterico: registro de um caso

E relatado caso de doente do sexo masculino, com 15 anos de idade, apresentando compressao da medula espinal em nivel toracico alto (T-2), causada por cisto enterico. A natureza do processo foi determinada na laminectomia de T-1 - T-3 e o diagnostico etiologico foi confirmado pelo exame histologico. Apos a cirurgia o paciente melhorou progressivamente das desordens motoras.The case of 15 years old boy with an enterogenous cyst causing compression of the spinal cord at T-2 is reported. The diagnosis was made by thoracic laminectomy and confirmed by histological examination. The motor disorders improved progressively after surgery.

Familial myasthenia gravis: report of four cases

Two pairs of siblings with myasthenia gravis, belonging to two different families, are reported. This is the only record of familial myasthenia during the past twenty years, in a total of 145 patients seen at the Neurological Clinic of the São Paulo Medical School. In spite of the fact that myasthenia gravis does not show hereditary characteristics, the peculiar features of the four cases justify the present report. The two pairs of siblings were born from non myasthenic nor consanguineous parents. The disease started at birth showing bilateral partial eyelid ptosis in all patients. The course of the illness has been favorable. There was no thymoma.