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J L de Assis

Publications and source records attributed to J L de Assis.

At least 37 records · Page 2Linked to original sources
PubMed1980

[Familial periodic paralysis: study of 8 cases].

The authors studied 8 patients with Familial Periodic Paralysis on the clinical, hydroelectrolytic, electrophysiologic, histologic and therapeutic points of view. There was significant predominance in males. The onset of the symptoms in our group began under fifteen years of age. The clinical manifestations were similar to those referred in the literature. The hypokalemic form was found in all patients except in one case that had hyperkalemic form. Natremias on the superior border of the normality, were seen in 21.4% of the cases, and in 21.4% the natremias were higher; this fact suggested aldosterone liberation of the muscle. The histologic examination performed in 5 patients, was normal in 20% of them and, in those cases with repetitive and frequent crises, atrophic muscle fibers were seen. Prophylactic treatments were made. The patients with hypokalemic form received supplementary doses of potassium.

Adolescent↗
PubMed1979

[Adult form of Niemann-Pick disease associated with Osler-Rendu-Weber syndrome. Report of a case].

The case of a 28-year-old female with an adult form of Niemann-Pick disease is described. The illness started at the age of 22 years. The characteristics actually established in the adult form of this lipidosis were discussed. Vision was impaired but there was no clinical evidence of importnat visual involvement. Neurologic and psychiatric examinations were always normal. The bone marrow contained foam cells. A cherry red spot was present in the macula. An uncommon association with Osler-Rendu-Weber disease was described. The patient maintained normal clinical and neurological conditions, that is, she continues to show an extraordinarily benign course with poor cerebral participation represented by a moderate localized cortical atrophy seen at the pneumoencephalogram.

Adult↗
PubMed1979

[Hematin treatment of acute intermittent porphyria. A case report (author's transl)].

A young woman with acute intermittent porphyria in profound relapse and severe nervous and respiratory involvement was treated by intravenous infusions of hematin, followed by improvement of symptoms. The diet with high carbohydrate and protein content and a B-adrenergic blocking agent, not showed any beneficial effect on acute attack. The infusion of hematin was followed by a lowering effect on urine porphybilinogen and delta-aminolevulinic acid and clinical improvement. The return to normal of porphyrin precursors in the urine was accompanied by almost complete clinical remission. The relationship of remission and repression of delta-aminolevulinic acid, decrease of urinary levels of porphyrin precursors, pulmonaries tests and electromyograms, were discussed.

Adolescent↗
PubMed1978

[Thymectomy in myasthenia gravis. Evaluation of the results in 73 patients].

An evaluation of results obtained after 73 thymectomies is reported. A transthoracic approach was used on all of the patients. Thymomas were found in 9 out of the 73 patients with myasthenia. All of the 64 patients without thymomas suffered from generalized myasthenia gravis with predominance of severe forms. The results of the early, intermediate and late post-operative course were analysed. A comparative study of results as related to age, sex, duration of disease and presence or absence of thymomas was made. Statistical analysis failed to show any significant difference in the results of thymectomy between selected and randomly chosen patients without thymomas.

Adolescent↗
PubMedDate not supplied

Thymectomy for myasthenia gravis: evaluation of results in 282 patients.

The results of thymectomy in 282 patients with acquired and generalised myasthenia gravis are presented. The study includes patients submitted to surgical treatment in the period between 1958 and 1990. Remission or marked improvement was obtained in 70% of the patients with follow-up extending from one to 25 years. The total post-operative mortality rate was 8% dying predominantly patients with thymoma. Median sternotomy was made in 278 patients, transcervical thymectomy in two patients and thyroidectomy with concomitant thymectomy in other two. Surgical methods, operatory phases, previous treatment with prednisone and long term evolution with final results are discussed.

Adolescent↗
PubMedDate not supplied

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↗