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Biomedical subjects

E R Barbosa

Publications and source records attributed to E R Barbosa.

At least 37 records · Page 2Linked to original sources
PubMed1992

Postvaccinal parkinsonism.

A 5-year-old boy, with a history of fever beginning 15 days after a vaccination for measles, developed a rigid-akinetic syndrome 3 days after the fever began. A spinal tap obtained 1 week after the onset of fever showed pleocytosis with a monocellular pattern. A CT scan of the head and EEG did not disclose any abnormality. An MRI performed 3 months after the event, however, showed clear-cut evidence of bilateral substantia nigra lesions, suggesting secondary gliosis. The response to levodopa was good, but adverse reactions appeared early. The child is now 7 years old. Bromocriptine, deprenyl, and levodopa have produced a remarkable improvement of the parkinsonian features.

Bromocriptine↗
PubMed1992

[Zinc in the treatment of hepatolenticular degeneration: report of 3 cases].

Three patients with symptoms and signs of hepatolenticular degeneration (HLD) who developed serious renal side effects of D-penicillamine (DP) had their therapeutic schedule changed to zinc. Patient 1, a 55 year-old man had been well until 12 years old, when skeletal changes (osteomalacia) due to tubular renal disturbance began. His diagnosis of HLD had first been established at age of 32 when he presented with "wing-beat" tremor. He was then begun on DP and his neurological symptoms resolved within one year of initiating therapy but skeletal abnormalities remained unchanged as a sequel. During the next 22 years the patient was continued on DP therapy but with poor compliance. Then the reappearance of his neurological manifestations occurred several times. By the age of 53, after one year without therapy, his neurological status has worse. DP was reinstituted but some weeks later his renal laboratory parameters became severely affected. DP was discontinued and zinc sulfate (220 mg three times daily) was introduced. On this therapeutic regimen his renal laboratory parameters returned to previous level after one month. Within one year on this therapeutic regimen neurological manifestations were resolved. After 31 months on zinc treatment he remains neurologically asymptomatic and his renal function is satisfactory. Patient 2, a 41 year old woman had been her diagnosis of HLD at age of 20, when following the diagnosis of the disease in her old brother, she was found to have the laboratory features of HLD and bilateral Kayser-Fleischer rings. DP treatment was recommended at that time but she quit the follow-up. When she was 23, an esophageal variceal bleeding occurred.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1992

[Parkinsonism secondary to ethylene oxide exposure: case report].

Ethylene oxide is a gas widely used in the production of industrial chemicals. It is also used to sterilize heat-sensitive medical supplies. Previous reports of acute and chronic exposure have described neurotoxic effects like peripheral neuropathy and cognitive impairment. We describe a pure parkinsonian syndrome following acute ethylene oxide intoxication. A 39-years-old male was referred to our Movement Disorders Clinic for evaluation of a parkinsonian syndrome. He was acutely exposed to ethylene oxide four years before and remained comatose for three days, and gradually regained consciousness. At that time he showed a global parkinsonian syndrome including bradykinesia, rigidity and rest tremor, with a severe motor disability; no other neurological disorders were found. The symptomatology was partially controlled with biperidene and levodopa plus carbidopa. Two years later he developed L-dopa induced dyskinesias. Four years after the intoxication he was evaluated at our clinic. General examination showed no abnormalities. Neurologic examination revealed a normal mental status. Motor evaluation disclosed moderate bradykinesia, rigidity and rest tremor, shuffling gait, poor facial mimic, stooped posture, and his speech was low and monotonous; deep tendon reflexes were brisk. The Hoehn-Yahr disability score was degree IV. Routine laboratory and radiological exams showed results within normal limits. The CSF examination was normal. Brain computed tomography and magnetic ressonance were normal. A trial with bromocriptine and levodopa plus carbidopa did not improve dyskinesia, and he was put on a schedule including amantadine and biperidene with improvement to grade III in Hoehn-Yahr scale. In the present case there was a clear relation between the acute exogenous intoxication and irreversible parkinsonism.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

[Parkinson disease associated to a brain tumor: a case report].

We are presenting an uncommon case of cerebral tumor whose major manifestation was parkinsonism. The patient was a 50-year-old woman presented with a 5-month history of tremor of the right hand, particularly at rest, and headache. On neurological examination of March, 1987 there were: slight right-sided hemiparesis with symmetrical hyperreflexia; discrete bradykinesia in combination with cogwheel rigidity also on the right-side; resting tremor of the right hand; and bilateral papilledema. The neuropsychological examination disclosed: nominative aphasia, impaired recent memory and right-left disorientation. The computed tomography showed a large, left frontotemporal tumor. Angiograms of the left internal and external carotid arteries revealed a tumor blush in the left frontotemporal region supplied by a enlarged middle meningeal artery. An electromyogram revealed a 4-6 HZ tremor on right hand. A course of treatment with dexamethasone 16 mg/day and levodopa plus benzerazine (500 mg/day) was unsuccessful. A left fronto-temporo-parietal craniotomy was performed and an attached sphenoid wing tumor was macroscopically completely removed. Microscopy indicated that the tumor was a meningioma. Postoperatively, the patient made an uneventful recovery. After two weeks, her right-sided palsy and parkinsonism had disappeared, and neuropsychological deficits improved. Two months later there was no abnormalities on neurological and neuropsychological examination. It was concluded that the parkinsonism was caused by mechanical pressure on the basal ganglia.

Female↗
PubMed1991

[Hepatolenticular degeneration: evaluation of neurological course in 76 treated cases].

A series of 76 patients with hepatolenticular degeneration (HLD) followed up at the Hospital of the University of São Paulo Medical School between 1963 and 1988 have been studied focusing the evolution under treatment of neurological features. The assessment of neurological symptoms included 12 specific items, and it was made by a scoring system ranging from 0 to 3 (absent, mild, moderate and severe). Global score varied between 0 to 36. A functional disability scale (0, normal; 1, mild; 2, moderate; 3, severe) was also adopted. All patients received D-penicillamine but three of them developed serious renal toxic side-effects, and this drug was replaced by zinc (sulphate or acetate). Fifty-nine patients (77.6%) improved, 8 (10.6%) remained unchanged, and 9 (11.8%) got worse. The main features related to bad results were poor compliance, predominance of dystonic symptoms, and short time treatment (death due hepatic complications). Eight patients (10.5%) developed transient worsening of neurological symptoms during the first weeks of treatment. Three patients who have D-penicillamine replaced by zinc salts presented satisfactory evolution. From them, only one had neurological symptoms. Death due to infectious complications related to severe motor disability occurred in three patients, representing only 10.7% of fatal evolution.

Female↗
PubMed1987

[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↗
PubMed1987

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↗
PubMed1986

[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↗
PubMed1985

[Hepatolenticular degeneration: critical evaluation of the diagnostic criteria in 95 cases].

Ninety-five cases of hepatolenticular degeneration have been studied, focusing particularly the clinical and laboratory characterization of the disease. On the clinical viewpoint the variability of the starting symptoms and the frequency of the Kayser-Fleischer rings were analyzed. As regards the laboratory findings the ceruloplasmin, blood and urinary copper, and aminoaciduria levels have been evaluated, as well as the radiological and scintillographic study of joints and bones, cranial computerized tomography and liver biopsy. In 54.4% of the cases the opening clinical picture was neurological, in 31.1% hepatic, in 14.4% psychiatric, in 7.8% osteoarticular, in 2.2% ophtalmologic (Kayser-Fleischer rings), in 1.1% hematologic (hemolytic anemia), and in 1.1% cardiac. Kayser-Fleischer rings were present in 84 of the 92 cases in which they have been searched for (91.3%). Concerning the laboratory findings, hypoceruloplasminemia was found in 98.8% of the cases in which it was investigated, hyperaminoaciduria in 94.7%, hypocupremia in 87.0%, increased cupruresis in 78.2%, osteoporosis in 79.4%, scintillographic changes of the joints in 67.6%; the CT-scan, performed in 11 cases, showed low attenuation areas in the basal ganglia of 2 patients. The significance of the mentioned laboratory findings and the presence of the Kayser-Fleischer rings for the diagnosis of Wilson's disease is discussed.

Amino Acids↗
PubMed1985

[Benign familial chorea: report of 2 cases].

Two cases (siblings) of benign familial chorea are reported. The family's pedigree shows an autosomic dominant form of inheritance with incomplete penetrance. The differential diagnosis is discussed as well a brief literature revision is made.

Adolescent↗
PubMed1983

[Huntington chorea: report of 16 cases].

The neuropsychiatric aspects and both hereditary and therapeutic study from 16 patients with Huntington's chorea are presented. The differential diagnosis from a clinical approach has been emphasized. The results of this research and those of the literature were compared.

Adult↗
PubMed1982

[Myasthenia gravis and pemphigus foliaceus].

Myasthenia gravis associated with thymoma affecting a 76-year-old white woman is reported. Two years later, after an immunosuppressive treatment with prednisone and irradiation of the tumor, bullous lesions appeared on the skin sparing the mucosae. The histopathologic features and the direct and indirect immunofluorescence tests indicated the existence of pemphigus foliaceus. The association of Myasthenia Gravis with pemphigus foliaceus is very rare, and the present case is actually the third reported in the literature.

Aged↗
PubMed1980

[Juvenile parkinsonism: considerations apropos of 10 cases].

Ten cases of juvenile parkinsonism are reported. A brief review of the literature is made and the nosologic boundaries of the disease are discussed. Comments are made on some clinical, genetic and therapeutic features of the illness in this series and according to the literature data.

Adolescent↗
PubMed1978

Osteoarthropathy of hepatolenticular degeneration.

Ninety-six joints of 25 patients with hepatolenticular degeneration have been studied by means of scintigraphs with technetium (99mTc), a method which can disclose early synovial inflammation and postinflammatory articular degeneration. Signs of synovitis were found in 22 joints of 10 patients (40%) and degenerative changes were present in 15 joints of eight patients (32%). Eleven patients complained of joint pains; in four of them they were the presenting symptom. The radiological study showed osteoporosis in 22 patients (88%); flexion deformity was found in two cases, anomalous osteophytes in two, and signs of growth arrest, features of rheumatoid arthritis and aseptic necrosis in one case each. The pathogenesis of osteoarticular changes in Wilson's disease is discussed.

Adolescent↗
PubMed1978

Heart involvement in hepatolenticular degeneration.

The case of a 10-year-old boy with a mild abdominal form of hepatolenticular degeneration, showing clinical, laboratory and histological manifestations of heart involvement is reported. The microscopical examination of myocardial fragments collected by intracavitary puncture biopsy, using hematoxylin-eosine and the histochemical method with rubeanic acid, showed a medium degree of myocardial damage and the presence of copper deposition in the tissue. Copper concentration on a myocardial biopsy fragment was almost 10 times the normal heart content. Comments are made on the relationship of the clinical, laboratory and pathological findings with the degree of copper storage on the myocardium.

Cardiomyopathies↗
PubMed1978

[Primary amebic meningoencephalomyelitis. Report of a case].

A case of primary amebic meningoencephalomyelitis due to Naegleria sp observed in a 14 years old boy is reported. Symptoms due to myelitis at the dorsal level assumed ascending character during the first days of disease. Manifestations due to encephalic involvemente were discrete. Cerebrospinal fluid changes were marked by pleocytosis, and the eosinophil cells participation in the cytomorphological profile was persitently high. Amphotericin-B was used intravenously (25 mgm/day) until 1,500 mgm of total dosis. Remission of encephalitis manifestations was prompt, as well as of meningeal signs. Cerebrospinal fluid changes disappeared progressively. Sensitive-motor changes due to spinal cord involvement persisted as permanent sequelae. Naegleria sp., was isolated from the cerebrospinal fluid in the acute stage of the disease, as well as, from the water of a lagoon where the patient used to swim.

Adolescent↗
PubMedDate not supplied

Relationship between obsessive-compulsive disorders and diseases affecting primarily the basal ganglia.

Obsessive-compulsive disorder (OCD) has been reported in association with some neurological diseases that affect the basal ganglia such as Tourette's syndrome, Sydenham's chorea, Parkinson's disease, and Huntington's disease. Furthermore, studies such as neuroimaging, suggest a role of the basal ganglia in the pathophysiology of OCD. The aim of this paper is to describe the association of OCD and several neurologic disorders affecting the basal ganglia, report the existing evidences of the role of the basal ganglia in the pathophysiology of OCD, and analyze the mechanisms probably involved in this pathophysiology.

Basal Ganglia↗