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Biomedical subjects

M Scaff

Publications and source records attributed to M Scaff.

At least 55 records · Page 3Linked to original sources

Failure of treatment of myasthenia gravis by cyclosporin-A: A case report.

Recently, cyclosporin-A (Cy-A) has been used in the treatment of myasthenia gravis (MG). This drug could be employed in some patients refractory to classic treatments or that develop undesirable side effects. It is reported the case of a 22 year-old woman with generalized and severe MG, and diabetes mellitus. She had been submitted to thymectomy and reoperated, to the classic ethiopathogenic methods of therapy, and to total body irradiation. No therapeutical results were observed. Also, she developed transient and slow bone marrow depression, and liver dysfunction. Owing to these limitations and to the absence of response to treatments mentioned. Cy-A use was attempted in this case. Unfortunately, Cy-A did not influence the myasthenic symptomatology. Cy-A also failed in suppressing anti-AChR production, which increased during Cy-A therapy. Results observed in this case are in disagreement with literature data on the subject.

Adult↗

[Acute polyradiculoneuritis and acquired immunodeficiency virus].

A 50-year-old man with positive test for human immunodeficiency virus (HIV) by enzyme-linked-immunoassy and Western-blot, without clinical manifestations of acquired immunodeficiency syndrome (AIDS), developed acute polyradiculoneuritis and was treated by plasmapheresis with improvement. We believe that chemical homologies of antigenic determinants between HIV and P2 protein of peripheral nervous system and myelin basic protein may induce crossed-reaction, thus developing acute polyradiculoneuritis and central nervous system involvement, respectively. The nervous system involvement hy HIV also occur in the HI-viremy, seric conversion alone, and AIDS with or without oportunistic infections.

Acute Disease↗

Mitochondrial dysfunction in myasthenia gravis. Report of a case.

The case of an 11-year-old boy with external ophthalmoparesia, tetraparesia and bilateral eyelid ptosis is reported. He was 7-years-old when first symptoms appeared. Anticholinesterasic drugs were used. He was submitted to muscle biopsy. The results of histochemistry analysis showed storage of granulous material at the subsarcolemmal region of muscle fibers by SDH. Increase in the number of mitochondria with electron dense bodies was found at electron microscopy. Anticholinesterasic drugs administration was interrupted and consequently he got worse, and bouts of dyspnea occurred. Due to this worsening anticholinesterasic agents were reintroduced together with prednisone, and he improved. Due to clinical and histological expressions we think it is possible that morphological mitochondrial alterations may occur also in myasthenia gravis.

Child↗

HTLV-I associated myelopathy in Brazil: a preliminary report.

In this preliminary report the authors present the finding of a high prevalence (37.5%) of seropositivity of antibodies to HTLV-I tested by Western blotting in a sample of 16 Brazilian patients with chronic myelopathies of undetermined origin.

Blotting, Western↗

Lissauer form of paretic neurosyphilis with a cerebral mass lesion.

A 40-year-old male patient with progressive dementia presented adversive seizures, and CT scan showed an enlarging focal mass lesion in the right cerebral hemisphere. Cerebrospinal fluid examination and brain biopsy confirmed the diagnosis of neurosyphilis. After a course of penicillin therapy there was disappearance of the cerebral mass lesion and the CT scan showed focal atrophy in the right cerebral hemisphere. This case suggests that Lissauer form of paretic neurosyphilis may present as a focal mass lesion.

Adult↗

[Neuropsychological dysfunctions in Parkinson's disease: a study of 64 cases].

Sixty-four cases of idiopathic Parkinson's disease were studied focusing the neuropsychological performance. To all subjects a brief mental status questionnaire was administered (30 items). Cases with a performance below normal and meeting the dementia criteria of the Diagnosis and Statistical Manual of Mental Disorders, third edition (DSM III), were considered demented. This evaluation showed that 12 of the 64 patients had dementia, a prevalence rate of 18.7%. Patients that had neuropsychological performance below normal showed more severe bradykinesia than normals. Short term memory, abstract thinking, visual gnosia, calculations, dynamic motor function of hands, constructional ability and recent memory were the most affected psychological functions. The significance of the mentioned findings is discussed and the literature reviewed.

Aged↗

[Conservative treatment and thymectomy in myasthenia gravis: short- and long-term evaluated results].

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.

Adolescent↗

Abdominal ultrasonography in hepatolenticular degeneration. A study of 33 patients.

The ultrasonographic study of 33 patients with hepatolenticular degeneration showed the following main changes: disorders of the hepatic echotexture (29 cases), changes of the splenic dimensions (21), liver shrinkage (10), cholelithiasis (8), hepatomegaly and ascites (1). The disorders of liver echotexture exhibited different patterns, from slight to severe changes of the hepatic echogenicity, associated with anatomic distortions of the liver, such as alterations of outline and decrease of dimensions. Liver shrinkage was always accompanied by splenomegaly. The scarcity of hepatomegaly may be explained by the fact that the ultrasonography was performed after the onset of treatment with penicillamine, or by other factors still unknown, copper perhaps possessing a stronger fibrogenetic action than other hepatotoxic agents. Cholelithiasis was very frequent in females (6 of 13 patients), its incidence tending to increase with age. Regarding males there was no increase when the incidence was compared to the general population. The two sole males were young, an unusual finding in normal men.

Abdomen↗

[Dermatopolymyositis: evaluation of 63 patients].

Sixty-three patients with dermatopolymyositis were evaluated from the clinical, laboratory and therapeutical aspects during a period of 15 years: 39 are women and 24 men. The mean age was 36.8 +/- 15.6 years. No correlation was observed between clinical and isolated therapeutics employed; when corticosteroids and cytolytic drugs were used simultaneously, the clinical response was satisfactory. No special fact was seen that can predict the therapeutical response.

Adolescent↗

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

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.

Animals↗

[Hypoattenuating lesions of the basal ganglia associated with pyramido-extrapyramidal features: report of two cases in brothers].

Two cases (siblings) with computed tomography showing slow attenuation of basal ganglia are reported. In case 1 the abnormality is larger, bilateral and the most important neurological features are: dystonic postures mainly in lower extremities and very active patellar reflexes with sustained knee clonus on both sides. In case 2 the tomographic examination disclosed a similar, but more discrete lesion only on the left side; the neurological examination is normal despite a history of frequent falls. The authors discuss the differential diagnosis from a clinical and tomographic approach.

Basal Ganglia Diseases↗

[Treatment of myasthenia gravis by immunosuppressive non-steroidal drugs].

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 1g daily by venous route 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 generally stabilized. Exacerbations and crises did not recur, 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 24th month. This patient returned to azathioprine.

Adolescent↗

Immunosuppression with corticosteroids and thymectomy in myasthenia gravis: an evaluation of immediate and short term results in 20 patients.

A comparative study was conducted on two groups of patients with the generalized severe form of myasthenia gravis. The first group of 20 patients received oral daily doses of 60-100 mg of prednisone prior to thymectomy. The control group of 20 were submitted to surgery without prior corticosteroid treatment. The study included statistical analysis of the clinical results and surgical complications for both groups. The authors concluded that the use of steroids preoperatively is beneficial.

Adolescent↗