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Biomedical subjects

I Shoulson

Publications and source records attributed to I Shoulson.

At least 73 records · Page 4Linked to original sources
PubMed1986

Huntington's disease in Venezuela: neurologic features and functional decline.

We studied 65 Huntington's disease patients and 225 at-risk individuals over the past 4 years. The rate of decline of these untreated patients from Venezuela was similar to that seen in US patients who had received neuroleptic drugs. Chorea, oculomotor dysfunction, and dysdiadochokinesis were early symptoms; parkinsonian features and dystonia came later. Juvenile patients declined nearly twice as fast as adult-onset patients. No distinctive neurologic phenotypes were seen in children of two affected parents.

Adult↗
PubMed1986

Familial Tourette's syndrome: report of a large pedigree and potential for linkage analysis.

We studied a large Mennonite kindred affected by chronic motor tics (CMTs) and vocal tics in a probable autosomal dominant pattern. We administered a standardized questionnaire to 69 family members and reviewed our videotapes of 47. Using DSM III criteria and independent ratings, we diagnosed 10 subjects as having definite Tourette's syndrome (TS), 3 with definite CMTs, 15 with probable TS, and 1 with probable CMT. The clinical features of this family are presented. This is the largest known kindred with TS. Permanent lymphoblastoid cell lines have been established from 67 family members for genetic linkage analysis.

Adolescent↗
PubMed1986

Double-blind assessment of potential pergolide-induced cardiotoxicity.

Possible pergolide-induced cardiotoxicity has been reported in open trials. Over a 6-month period of observation, we prospectively analyzed ECGs and 24-hour ambulatory ECG in 23 patients with Parkinson's disease who were randomized in a double-blind fashion to pergolide or placebo treatments. Pergolide therapy was associated with a mild and transient bradycardiac effect, but no clinically significant cardiotoxicity.

Aged↗
PubMed1986

On chorea.

Explore the source record for details and available documents.

Chorea↗
PubMed1985

Alterations in L-glutamate binding in Alzheimer's and Huntington's diseases.

Brain sections from patients who had died with senile dementia of the Alzheimer's type (SDAT), Huntington's disease (HD), or no neurologic disease were studied by autoradiography to measure sodium-independent L-[3H]glutamate binding. In brain sections from SDAT patients, glutamate binding was normal in the caudate, putamen, and claustrum but was lower than normal in the cortex. The decreased cortical binding represented a reduction in numbers of binding sites, not a change in binding affinity, and appeared to be the result of a specific decrease in numbers of the low-affinity quisqualate binding site. No significant changes in cortical binding of other ligands were observed. In brains from Huntington's disease patients, glutamate binding was lower in the caudate and putamen than in the same regions of brains from control and SDAT patients but was normal in the cortex. It is possible that development of positron-emitting probes for glutamate receptors may permit diagnosis of SDAT in vivo by means of positron emission tomographic scanning.

Alzheimer Disease↗
PubMed1985

Flow cytometric detection of lymphocyte alterations in Huntington's disease.

Several recent reports indicate that patients with Huntington's Disease (HD) may manifest membrane abnormalities in a wide variety of cells including peripheral blood lymphocytes. In this study, flow cytometry is used in conjunction with the fluorescent membrane probe, 8-anilino-1-naphthalene sulfonate (ANS), to examine peripheral blood lymphocytes from 16 HD patients and 14 age- and diet-matched control subjects. Increased ANS fluorescence intensity of lymphocytes (p less than 0.02) was found in HD patients as compared to control subjects. These differences are masked when the mean fluorescence of the total leukocyte population is measured, possibly explaining conflicting data of other investigators. These observed differences in ANS fluorescence intensity between HD patients and control subjects support the concept of a gene defect which may be expressed as membrane alterations in non-neural as well as neural cells. The selective alterations of lymphocytes may also reflect altered immunological activity reported in HD.

Adult↗
PubMed1985

Severe compression neuropathy following sudden onset of parkinsonian immobility.

Prolonged immobility with adoption of unusual fixed body postures may lead to compression neuropathy. Although parkinsonism has not been considered to predispose to nerve compression, we report three patients with Parkinson's disease and on-off motor fluctuations who developed severe compression neuropathy following a sudden onset off period. The stereotyped posture assumed by parkinsonian patients during an off period appears to make them especially prone to injury of the radial nerve in the upper arm and the brachial plexus.

Aged↗
PubMed1985

Long-term experience with pergolide therapy of advanced parkinsonism.

Nine patients with idiopathic Parkinson's disease were treated with pergolide to a daily maintenance dose of 2.2 +/- 0.9 mg (mean +/- SD) for 17.3 +/- 8.3 months. After 1 month, there was an average 68% increase in mobile on-time, but the improvement declined to 30% by 6 months, 23% by 1 year, and virtually disappeared by 18 months of therapy. Pergolide was discontinued in seven patients because of loss of efficacy (4 patients), confusion (1 patient), or myocardial infarction or ventricular ectopy (2 patients). Partial but temporary restoration of mobility was observed in seven patients who were switched to an alternate-day dosing schedule after 9.2 +/- 2.4 months. Two patients with advanced Shy-Drager syndrome were treated with pergolide without benefit.

Aged↗
PubMed1984

Benzodiazepine and GABA receptors in early Huntington's disease.

We compared autoradiographic measurements of GABA and benzodiazepine receptors in the brains of two early cases of Huntington's disease (HD), five controls, and four advanced cases of HD. These receptors increase in lateral globus pallidus and decrease in putamen early in the disease, before there is any extensive cell loss and atrophy. The cause of these receptor changes is unknown, but could imply dysfunction rather than death of striatal neurons.

Benzodiazepines↗
PubMed1984

Huntington's disease. A decade of progress.

Considerable investigative attention has been accorded to HD in the past decade. These efforts have been rewarded by substantive advances in our understanding of the clinical features and pathologic consequences of this disorder. The recent linkage of HD to a DNA segment mapped to chromosome 4 is a remarkable achievement that will help sustain investigative efforts and lead to therapeutic benefits for patients and families.

Brain↗
PubMed1983

Familial paroxysmal dystonic choreoathetosis and response to alternate-day oxazepam therapy.

A patient with familial paroxysmal dystonic choreoathetosis (FPDC) who was only transiently improved on daily benzodiazepine therapy showed sustained benefit from a regimen of alternate-day oxazepam therapy. Our findings confirm that FPDC is responsive to benzodiazepine therapy and suggest that an alternate-day dosing schedule, using a short-acting benzodiazepine, may minimize receptor down regulation and thereby sustain the therapeutic effect.

Athetosis↗
PubMed1983

Psychiatric syndromes in Huntington's disease.

Thirty patients with Huntington's disease, a genetically transmitted neuropsychiatric disorder that can be diagnosed reliably, were evaluated systematically for psychopathology, followed for extended periods, and treated with psychopharmacological medications when necessary. DSM-III criteria were used for establishing syndromic diagnoses. Twenty-four individuals demonstrated substantial behavioral abnormalities, including affective and schizophrenic syndromes, changes of personality, and disorders that could not be classified adequately. Pharmacotherapy was modestly beneficial in some cases. Consideration of the array of behavioral disturbances encountered in this pathogenetically unified disorder suggests that a dimensional approach to symptom classification might prove more useful heuristically than present typological methods.

Adult↗
PubMed1983

On-off effects in Parkinson's disease: a controlled investigation of ascorbic acid therapy.

Patients with PD who experience on-off effects have higher erythrocyte catechol-O-methyltransferase (COMT) activities and plasma 3-O-methyldopa concentrations than do patients without these levodopa-related motor fluctuations. We therefore administered ascorbic acid, a weak competitive inhibitor of COMT, to six PD patients with on-off effects. In this double-blind crossover investigation, ascorbic acid produced a modest improvement in functional performance. However, no fundamental change was observed in the pattern of on-off effects, severity of parkinsonism/dyskinesia, or self-assessment ratings. Ascorbic acid therapy reduced plasma concentrations of levodopa and 3-O-methyldopa but did not alter erythrocyte COMT activity. These findings are discussed in the context of the pharmacokinetic and pharmacodynamic factors that contribute to the pathogenesis of levodopa-induced dyskinesias and on-off motor fluctuations.

Aged↗