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Biomedical subjects

A Barbeau

Publications and source records attributed to A Barbeau.

At least 163 records · Page 9Linked to original sources

HLA and complement typing in olivo-ponto-cerebellar atrophy.

HLA antigen typing was carried out in a family with an autosomal dominant form of spinocerebellar degeneration [possibly olivoponto cerebellar atrophy (O.P.C.A.)--Type 1]. Eleven ataxic patients, three possibly ataxic subjects, two unrelated spouses and 13 clinically normal at risk siblings were typed for ABO and Rh blood groups, HLA-A and HLA-B antigens, C4 component of the complement and a number of other serum proteins (Clq, beta-1A, beta-1C, C5, beta-lipoproteins). No solid evidence for linkage between the ataxia gene and the HLA or C4 loci could be demonstrated in this family. Certain serum proteins, and particularly beta-lipoproteins were found to be significantly reduced in some sub-groups of subjects.

Adolescent↗

Oropharyngeal dysphagia in patients with oculopharyngeal muscular dystrophy.

Sixteen patients with an established diagnosis of oculopharyngeal muscular dystrophy underwent clinical, radiologic and manometric assessment. Secondary pharyngo-oral and pharyngonasal regurgitations are usually associated with this condition and chronic aspiration with consequent bronchorrhea is common. Such patients may present with marked oropharyngeal dysphagia. Cineradiologic findings correlated well with the mamometric results. The pharynx showed very weak contractions of longer duration than those observed in normal subjects. The proximal esophageal sphincter had a normal resting and closing pressure; however, relaxation and coordination of the sphincter were substantially different from those in a control group. Eleven patients underwent cricopharyngeal myotomy. All had notable improvement of their symptoms. Surgery on the sphincter results in a substantial decrease in its resting pressure; pharyngeal contraction remains unaltered.

Aged↗

Peptides in Parkinson's disease.

Peptides have found a progressively more important role in the biochemistry and physiology of the brain in recent years. Many possess neurological and behavioral actions in addition to their function in endocrinology. This paper reviews the formation and distribution, as well as the neurological action, of peptides probably involved in Parkinson's disease: beta-LPH and its derivatives (MSH, ACTH4-10, beta-endorphin). It also recounts the experience of many authors with prolyl-leucyl-glycine-amide (PLG) in that disease. Finally we propose a new theory of the etiology of Parkinson's disease, based on a postulated deficiency in the important trophic function upon catecholaminergic neurons of A.P.U.D. cells.

Brain↗

A sensitive radiometric assay for cysteic acid decarboxylase activity in crude enzyme preparations of rat liver and brain.

1. A method is described for the synthesis of L-[U-14C]cysteic acid from L-[U-14C] cysteine hydrochloride and for its subsequent utilisation as a substrate for cysteic acid decarboxylase activity in liver and brain. 2. The enzyme determination relies on the entrapment of radio-labelled carbon dioxide in Hyamine hydroxide. 3. The assay is sensitive, reliable and convenient and is particularly suitable for measuring the activity of the decarboxylase in crude enzyme preparations.

Animals↗

Seven cases of Gilles de la tourette's syndrome: partial relief with clonazepam: a pilot study.

The histories of seven consecutive cases of Gilles de la Tourette's syndrome are presented to exemplify the range of clinical manifestations in this disease and to collate preliminary results with the new benzodiazepine, clonazepam, as possible adjuvant therapy of this disorder. Controlled trials with clonazepam alone and in association with haloperidol are now justified. Five of our 7 patients had a positive family history of tics, and 2 a confirmed family history of gout. Because clonazepam improves myoclonia and tics and because its mechanism of action possibly involves serotonin, we thought it worthwhile to study simultaneously the relative roles of serotonin and dopamine metabolism in the production of tics, and their relationship to possible defects in purine metabolism in Gilles de la Tourette's syndrome.

Adolescent↗

Platelet dopamine uptake in Huntington's chorea and Gilles de la Tourette's syndrome: effect of haloperidol.

Uptake of 14C-dopamine by human platelets has been studied in two diseases, namely Gilles de la Tourette's syndrome and Huntington's chroea, in which abnormal metabolism of dopamine has been implicated. Platelets from untreated Huntington's chorea patients showed a small increase in Km and Vmax.; platelets from patients in all other groups showed an uptake identical with the controls. Haloperidol (10(-5)M) was also shown to be a strong non-competitive inhibitor of 14C-DA uptake by platelets. This property is probably unrelated to the drugs' action in ameliorating the symptoms of Huntington's chorea which is likely related to the increase in cholinergic neuronal activity produced by neuroleptic blockade of dopamine receptors.

Adolescent↗

Effects of manganese, calcium, magnesium and lithium on the ouabain-induced seizure.

The effects of the intraventricularly administered cations (Mn2+, Ca2+, Mg2+ and Li+) against the seizure induced by ouabain (3 microgram) were investigated. Mn2+, Ca2+ and Mg2+ caused definite sedation and decreased locomotor activity. But Li+ was without significant behavioral effect at the doses applied. Among the cations used, Mn2+, Ca2+ and Mg2+ showed significant anticonvulsive effect on the ouabain-induced seizure. In comparison, on the dose and molar-to-molar basis, the potency of anticonvulsive action was in the following order: Mn2+ greater than Ca2+ greater than Mg2+. On the contrary, the higher dose of Li+ potentiated the ouabain-induced seizure. The importance of the increased Ca2+ level in the extracellular space or the inhibition of Ca2+ uptake as the anticonvulsive effect of Ca2+, Mn2+ and Mg2+ was discussed.

Animals↗

The inhibition by substrate analogues of gamma-aminobutyrate aminotransferase from mitochondria of different subcellular fractions of rat brain.

Cytoplasmic-mitochondria-enriched and synaptosome-enriched subcellular fractions were prepared from rat brain to study certain kinetic properties of gamma-aminobutyrate: alpha-oxoglutarate aminotransferase (GABA-T) (EC 2.6.1.19) from each of the sources. From this study two differences emerged. Firstly, the cytoplasmic enzyme exhibited an eightfold greater affinity for gamma-aminobutyric acid (GABA) than did the synaptosomal GABA-T; the Km being 6.5 mM and 53 mM, respectively. Secondly, synaptosomal GABA-T is comparatively more susceptible to inhibition by the substrate analogues 2,4-diaminobutyric acid (DABA) and aminooxyacetic acid (AOAA) than is the enzyme from the cytoplasmic mitochondrial fraction. In each case the inhibition was of a competitive nature with respect to GABA. The Ki for the DABA was 13 mM for the cytoplasmic-derived enzyme and 8mM for the synaptosomal enzyme. With AOAA the Ki was 0.1 muM and 0.06 muM for the synaptosomal and cytoplasmic mitochondrial enzyme, respectively. These results provide further evidence that GABA-T from cytoplasmic mitochondria is different in several respects from the enzyme found in synaptosomes.

4-Aminobutyrate Transaminase↗

Double-blind evaluation of oral L-prolyl-Lleucyl-glycine amide in Parkinson's disease.

A 4-month double-blind study comparing the effect of increasing oral doses (up to 1.0 g daily) of synthetic L-proyl-L-leucyl-glycine amide (PLG) and placebo in 20 parkinsonian patients showed no significant improvement in objective scores of functional disability. However, important trends and some significant results were observed with the lower doses of PLG. These essentially negative results may be attributed to poor intestinal absorption of the compound, a short biologic half-life in the blood, or administration of oral doses that were much higher than required, or a combination of factors. In further studies with this peptide, which are encouraged, the intravenous route should be used until the question of intestinal absorption is resolved.

Adult↗

Six years of high-level levodopa therapy in severely akinetic parkinsonian patients.

The clinical course and the side effects of high-level levodopa therapy over a six-year period were studied in 80 severely akinetic parkinsonian patients treated for the first time before June 1968. Levodopa improved the quality of life in greater than 53% of patients, but failed to modify the progression of the disease or change the prognosis. Seventeen "idiopathic Parkinson" patients died after a duration of illness of 9.5 years. Despite the drawbacks, levodopa is still the best available treatment for akinetic parkinsonism.

Activities of Daily Living↗