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Biomedical subjects

M Scaff

Publications and source records attributed to M Scaff.

At least 37 records · Page 2Linked to original sources

[Precocious Parkinson's disease associated with "eye-of-the-tiger" type pallidal lesions].

We report the case of a 56-years-old woman patient, born to unrelated parents, who since 26-years-old gradually developed bradykinesia, rigidity, tremor of both hands, and speech and gait difficulties. Her past history was unremarkable. There was no family history of neurologic disease. She was admitted to our Hospital at age 39 and at that time she presented a full parkinsonian syndrome. The following tests were normal or negative: routine blood studies, serum copper, ceruloplasmin and cerebrospinal fluid examination. There was not Kayser-Fleicher ring, and fundoscopic examination revealed no abnormalities. Levodopa was introduced and response was good for more than ten years, despite early-onset of dyskinesias (three months after the introduction of the drug). After 30 years under levodopa she still presents a moderate response but with severe fluctuations of the motor performance. Except for slowness of cognition she developed no other neuropsychological impairments, and a recent neurological examination disclosed no abnormalities besides a parkinsonian syndrome. One year ago, a magnetic resonance imaging (MRI) was performed and showed bilateral, symmetrical lesions with "eye-of-the-tiger" pattern. This case illustrates the pathological heterogeneity of early-onset parkinsonism and suggests the possibility to find the typical MRI lesions seen in Hallervorden-Spatz disease in other degenerative affections involving globus pallidus.

Diagnosis, Differential↗

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.

Adult↗

[Kinesigenic paroxysmal choreoathetosis. Report of a case treated with carbamazepine].

Paroxysmal choreoathetosis (PC) is a rare entity, and professionals who study movement disorders do not find it easily. Paroxysmal occurrence of dystonic, choreic, athetosic movements is the typical feature of this syndrome. The onset is always subtle and the attacks can last from few seconds to hours. Sporadic cases and more common familial cases have been reported. The therapeutic approach with anticonvulsant drugs like carbamazepine is not always successful. However, there is a good response to this drug in the kinesigenic form of PC. The case of a 21 years old male patient is reported here. The onset occurred during puberty, at 14 years old. Neurologic examination was normal between attacks. Subtle and brief choreic, athetosic and dystonic limb and legs movements precipitated by sudden quick and unexpected passive movements, as by startle, were observed during the attacks. There was no familial history. Routine laboratorial investigation, EEG, CT scan and MRI did not show significant changes. Carbamazepine in low dosages (100 mg/day) brought a complete control of the attacks.

Adult↗

Management of hydrocephalus in Paget's disease. Case report.

The authors report on a case of Paget's disease with hydrocephalus in an elderly female with a favourable result after ventriculoperitoneal shunt. The neurological examinations at the time of admission revealed mild dementia, ataxic gait and urinary incontinence. Skull X-ray, CT-scan and MRI showed hydrocephalus and basilar impression. The pathophysiological hypothesis is discussed and the technical difficulties during the shunt surgery are described.

Aged↗

[Neurologic evaluation of 118 patients in the first postoperative period of cardiovascular surgery].

118 patients (79 mean and 39 women, mean age of 50.7 years) who underwent cardiovascular surgery and extracorporeal oxygenation were prospectively evaluated for neurologic complication and its correlation with risk factors. 71 were submitted to coronary artery graft by-pass (RM), 18 to valve replacement (TV), 6 to prosthetic valve replacement (RV), 11 to commissurotomy(Co), 5 to thoracic aortic aneurysm correction (An Ao T) and 7 to other surgeries (OT). All of them received extracorporeal oxygenation. No deaths were registered; 14 (11.9%) patients had neurologic abnormalities: delirium in 7 cases, ischemic stroke in 6, epileptic seizure in 3. Patients with systemic arterial hypertension and older patients exhibited a statistically significant (p < 0.05) higher risk of complication. Compared to data of the literature, we had a lower index of morbidity and mortality.

Adolescent↗

Chorea as a sign of systemic lupus erythematosus activity. Case report.

Chorea may occur as a neurological manifestation of systemic lupus erythematosus and is often associated with detection of antiphospholipid antibodies. No evidence of chorea as a sign of lupus activity has been established. We describe a patient with systemic lupus erythematosus associated with antiphospholipid antibodies who developed chorea, which has been considered a sign of lupus activity.

Adolescent↗

[Neurological events in infective endocarditis].

PURPOSE: The study of frequency, modalities and course of neurological complications of infective endocarditis (IE), as well as the current indication and value of supplementary examinations. METHODS: Sixty-three patients with IE, 39 with native valve and 24 with valvar prosthesis, were prospectively studied; the mean age was 42 years and 45 (71.4%) were males. Two groups were formed: A) 41 patients without neurological events and B) 22 patients who presented 28 neurological events before or during hospitalization: ischemic cerebrovascular accident 20, hemorrhagic cerebrovascular accident 2, meningeal hemorrhage 2, meningitis 2, brain abscess 1 and seizure 1. All patients were submitted to neurological clinical examination; 57 computerized tomographies of the cranium, 28 arteriographies and 32 cerebrospinal fluid analysis were performed. RESULTS: The incidence of neurological events corresponded to 34.92% of IE patients, with a clear predominance (85.71%) of vascular as compared to infectious manifestations. Mortality was 2.32 times higher in group B patients (22.73% x 9.76%), albeit p = 0.256, and was not related to staphylococcal etiology. The neurological events were not related to sex, age and presence of valvar prosthesis. The presence of neurological complications was greater (p = 0.047) in patients with simultaneous infections in two valves (mitral and aortic) and also (p = 0.00884) in those with IE in prosthesis implanted for less than three months. All supplementary neurological examinations in group A were normal. CONCLUSION: 1) Occurrence of neurological events is a factor which influences the prognosis of IE; 2) supplementary neurological examinations did not reveal subclinical neurological complications; 3) neurological complications were significantly more frequent in patients with simultaneous mitral and aortic valve IE; 4) IE in prosthesis implanted for less than 3 months has a greater probability to develop a neurological picture as compared to IE in prosthesis implanted for more than 3 months.

Adolescent↗

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↗

Neurological complications after thrombolytic treatment for acute myocardial infarction: emphasis on unprecedented manifestations.

We examined 348 patients who underwent thrombolytic treatment for acute myocardial infarction. Nine patients (2.58%) developed neurological complications related directly or indirectly to this procedure. Cerebral hemorrhage occurred in 3 patients; 2 patients had transient ischemic attacks, 1 had syncope, another had psychomotor agitation and 2 patients presented seizures during the infusion of the thrombolytic agent, without hemodynamic abnormalities. This latter feature had never been described before.

Adult↗

[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↗

[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↗

[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↗

[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↗

Shy-Drager syndrome: a case report with polysomnography.

The authors report a case of Shy-Drager syndrome in a 53 year-old male patient. Autonomic failure was made evident by physical examination as well as laboratory tests. A sleep recording showed decreased percentage of REM sleep and apneas of the central type. The possible mechanisms for this sleep disorder are discussed.

Humans↗

Cerebrospinal fluid and serum antiphospholipid antibodies in multiple sclerosis, Guillain-Barré syndrome and systemic lupus erythematosus.

Immunoglobulins isotypes (IgG and IgM) for myelin basic protein (MBP), cerebrosides (CER), gangliosides (GANG) and cardiolipin (CARD) were detected in the cerebrospinal fluid (CSF) from 33 patients with multiple sclerosis (MS), 18 with Guillain-Barré syndrome (GBS) and 30 with systemic lupus erythematosus (SLE). In MS patients occurred positive and significant levels of IgG-MBP in 51.5% (p less than 0.05) and IgM-MBP in only 18.2%, IgG-CARD in 46.2%, as long as CER and GANG were detected in almost 20%. From serum samples of MS patients 20.6% presented IgG-MBP, while 53% showed positive levels for IgM-MBP. The CSF analysis of patients with GBS showed that 56.3% revealed IgG-MBP (p less than 0.05), 53% for IgM-MBP, 38.5% for IgG-CER and 23% for IgM-CER, while 50% of patients had IgG-CARD, as long as 31% also had IgG-GANG. The serum evaluation from 14 patients showed that 18.8% had positive concentrations of IgG-MBP and 56.3% presented IgM-MBP (p less than 0.05). Except for 50% of patients with SLE who presented positive CSF levels of IgG-CARD, only 24.1% had positive levels of IgG-MBP. We believe that the presence of antiphospholipid antibodies in CSF of the above mentioned diseases occurred as immune epiphenomena, but their appearance would permit the maintenance of and perpetuate the immune event.

Adult↗

Acetylcholine receptor antibody in myasthenia gravis.

Radioimmunoassay techniques were used to detect antibodies to the acetylcholine receptor (AAChR) in 164 patients with adult-onset myasthenia gravis. AAChR levels above 0.6 nM/l were considered pathological and were found in 67% of the patients with an average value of 58.99 +/- 125.02 nM/l (0.6-900.0). Correlation, with clinical functional status, the histopathological thymus alterations and the different therapeutics used did not disclose any statistically significant differences.

Adrenal Cortex Hormones↗