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M Scaff

Publications and source records attributed to M Scaff.

122 records · Page 7Linked to original sources
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Juvenile myasthenia gravis and thymectomy: report of ten cases.

Ten patients from 7 to 12 years of age presented generalized acquired myasthenia gravis: six had a severe form and four a moderate one. All patients showed progressive worsening and poor response to the medical treatment; all of them were submitted to thymectomy by median sternotomy. Long-term results were beneficial for 60% of the patients who experimented a marked improvement or even complete remission. One patient improved without medication being followed for two years. Another patient has been in remission taking only a minimal dose of anticholinesterase drug for seven years. In most cases prednisone has been used as long-term maintenance in the postoperative phase. Short-term results were not favorable: one death occurred and 30% of the patients worsened.

Child↗
PubMedDate not supplied

Nervous system involvement in systemic lupus erythematosus: report of three cases.

Central nervous system involvement in systemic lupus erythematosus is rather frequent whereas peripheral nervous system involvement is much less common. The three patients studied by us had isolated manifestations uncommon in nature. The first one developed a sensory-motor polineuropathy with signs of axonal degeneration. It responded to the therapeutic association of corticosteroids with an immunosuppressive agent. Satisfactory recovery took place over a time span of a year. The second patient had encephalic and cerebral trunk involvement from which an irreversible dementia resulted. The third patient, who had recurrent aseptic meningitis, is asymptomatic for six months now. Patients one and two had no systemic manifestations at the time of nervous system involvement. Suspicion of systemic lupus erythematosus was made on the basis of past inspecific articular symptoms. The neurological and systemic manifestations may be sometimes simultaneous; they are usually followed by serologic changes. Isolated nervous system involvement may be seen with and without sorologic changes, and there may be found antibodies reactive with phospholipids (anticardiolipin, antigangliosides and anticerebrosides). The employment of nonsteroid immunosuppressive drugs associated with corticosteroids in small doses seems to be useful in cases of systemic lupus erythematosus with nervous system involvement.

Adolescent↗
PubMedDate not supplied

Wilson's disease: magnetic resonance imaging (MRI) with clinical correlations in 16 cases.

The purpose of this study was to evaluate MRI findings in a group of patients with Wilson's disease, trying to establish possible correlations between clinical and image data. Sixteen patients (8 males and 8 females), with ages ranging from 11 to 50 years, and duration of illness ranging from 5 months to 32 years, were submitted to MRI in a 1.5T System. Four patients were asymptomatic, 4 had mild neurological findings, 2 were moderately affected and the remaining 6 had a severe form of the disease. All patients were receiving D-penicillamine by the time of the study. The most symptomatic patients presented five or more sites of abnormalities on MRI. The putamen was affected in all symptomatic individuals and one asymptomatic and 11 of them presented dystonia on neurological examination. A striking feature was the peripheral localization of putaminal hyperintense lesions on T2 weighted images. In eight cases, striatum or "substantia nigra" lesions explained parkinsonism observed on neurological examination. MRI seems to be an efficient method to study neurological involvement of Wilson's disease allowing some interesting anatomo-clinical correlations.

Adolescent↗
PubMedDate not supplied

Thymectomy in over 50 years-old patients with myasthenia gravis: long and short-term results in 21 patients.

Thymectomy is considered to be an important method for the treatment of myasthenia gravis in older patients when the improvement cannot be achieved with the use certain drugs such as anticholinesterasic, corticosteroids and immunosuppressive agents. In our series of 281 thymectomized patients, 13 were male and 8 female, varying their age between 50 and 73 years. The duration of the disease prior to the thymectomy varied between 30 days and 15 years; in 12 of them (57%) it lasted already 2 years. Eleven patients had the severe and eight the moderate clinical form of the disease. Nine patients were treated with corticosteroids before submitted to thymectomy. In 11 patients the histopathological changes in the thymus were evaluated: eight had thymomas, one atrophic changes, one lipomatosis and one a normal thymus. The short-term follow-up revealed important improvement in 12 patients, slight improvement in four and worsening in two; two patients died. It is concluded that thymectomy may be indicated in older patients when their condition worsens in spite of the use of corticosteroids and immunosuppressive agents, in the presence of serological markers or computerized tomography image indicating thymoma, and intolerance for corticosteroids.

Aged↗