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Biomedical subjects

H L Busard

Publications and source records attributed to H L Busard.

10 recordsLinked to original sources
PubMed1993

Ritanserin, a selective 5-HT2/1C antagonist, and negative symptoms in schizophrenia. A placebo-controlled double-blind trial.

The effectiveness of ritanserin, a selective 5-HT2 and 5-HT1c antagonist, in reducing negative symptoms in schizophrenia was investigated in a double-blind, placebo-controlled trial. Trial treatment was added to a stable neuroleptic treatment in 33 schizophrenic patients with predominantly negative symptoms. Ritanserin reduced the negative symptoms, as measured with the SANS. The main reduction was for the items facial expression, global affective flattening, and relationships with friends and peers. Also a reduction in total BPRS score was found, which approached statistical significance. Significant reductions were observed for the BPRS items emotional withdrawal and depressive mood. Ritanserin or other drugs blocking 5-HT2 and/or 5-HT1c receptors could be important in reducing specific symptoms in schizophrenic patients.

Antipsychotic Agents↗
PubMed1991

Adult polyglucosan body disease: the diagnostic value of axilla skin biopsy.

The diagnostic value of axilla skin biopsy has been investigated in a patient with adult polyglucosan body disease. The biopsy data have been compared with those of control subjects and with those from previously reported patients with Lafora's disease. In a patient with adult polyglucosan body disease and in patients with Lafora's disease, an abundance of polyglucosan bodies was found in the myoepithelial cells of the axillary apocrine glands. In the control group of subjects, polyglucosan bodies were only sporadically seen. Axilla skin biopsy is, therefore, an easy and reliable method for confirming the diagnosis of adult polyglucosan body disease.

Adolescent↗
PubMed1990

Polyglucosan bodies in sural nerve biopsies.

The presence of polyglucosan bodies in sural nerves collected over a 16-year period was studied in relation to age, sex, presence of polyneuropathy, and concomitant presence of central nervous system disorder. Polyglucosan bodies have been seen in only one patient without a polyneuropathy. This patient was suffering from Lafora's disease. In all other sural nerves positive for polyglucosan bodies a polyneuropathy was present. Within this group the prevalence of polyglucosan bodies was positively correlated with age, and if a central nervous system disorder was associated, this prevalence was more distinct. With semiquantitative measurements of the surface of polyglucosan bodies a significant correlation was found between age and percentage of large bodies.

Adolescent↗
PubMed1989

Polyglucosan bodies in intramuscular motor nerves.

The presence of polyglucosan bodies was studied in intramuscular motor nerves of 292 muscle biopsies. These biopsies were classified into five diagnostic categories and investigated for the presence of polyglucosan bodies in relation to age and sex. Their presence was nonspecific in patients over 20 years, the only correlation being with ageing. Under 20 years, their presence pointed to the diagnosis of Lafora's disease. In cases in which both a muscle biopsy and a sural nerve biopsy were performed, the former appeared to contain these polyglucosans more frequently.

Adolescent↗
PubMed1987

Axilla skin biopsy: a reliable test for the diagnosis of Lafora's disease.

Skin biopsies from the axilla were examined in 7 patients with clinical signs and symptoms of Lafora's disease, in 9 relatives (7 parents and 2 siblings), and in 17 patients with chronic or progressive degenerative neurological disorders and chronic epilepsy, who served as controls. In the biopsies of all patients with Lafora's disease, typical periodic acid-Schiff (PAS)-positive inclusions were present in the myoepithelial cells of the secretory acini of the apocrine glands and/or in the cells of the eccrine duct. No abnormalities were found in the biopsies from relatives and controls. We conclude that axilla skin biopsy is an easy and reliable test for diagnosing Lafora's disease, and is preferable to liver biopsy, but has no value for carrier detection.

Axilla↗
PubMed1984

Autosomal dominant paroxysmal kinesigenic choreoathetosis. An electroneurophysiological study.

A case of an 11-year-old boy with an autosomal dominant form of paroxysmal kinesigenic choreoathetosis is presented. Routine EEG, sleep EEG recording, and registration of visual evoked potentials and somatosensory evoked potentials were normal. EEG with videomonitoring and registration of event-related potentials, however, showed abnormalities, which are discussed in detail. Our data provide further arguments in support of the hypothesis that paroxysmal kinesigenic choreoathetosis is the expression of a dysbalance in the cortico-striopallidal-thalamic loop, and has an extrapyramidal genesis.

Athetosis↗
PubMedDate not supplied

Pyruvate metabolism in Lafora disease.

Lafora disease is an autosomal recessive and progressive degenerative disorder of the central nervous system (CNS). The pathogenic mechanism has been presumed to be an inborn error of carbohydrate metabolism, although this has never been proved. In a case of proven Lafora disease, pyruvate metabolism, which has a central position in carbohydrate metabolism, was studied in body fluids under various conditions and in brain biopsy material. No abnormalities in this metabolic pathway were found. This finding plus earlier reports in the literature exclude a defect in glycolysis; thus, a disturbance of carbohydrate metabolism as the pathogenic mechanism of Lafora disease is unlikely.

Adolescent↗
PubMedDate not supplied

Lafora disease: a quantitative morphological and biochemical study of the cerebral cortex.

Morphological and biochemical studies were performed on a brain biopsy from a patient with typical Lafora disease. Qualitative morphological investigation of the cortex showed that the Lafora bodies were most abundant in layers III and V of the cerebral cortex. They were exclusively located in the neurons and their processes. Quantitative morphological investigation of the cerebral cortex revealed abnormalities of the pyramidal cells of layers III and V. The neurons were often slightly atrophic. There was a reduction in the number of dendrites, in maximal dendritic length, and in the number of spines on the apical dendrites and on the side branches. Because of the biochemical study of the pyruvate metabolism of the gray and white matter of the cortex did not show abnormalities, a mitochondrial dysfunction is not likely.

Adolescent↗
PubMedDate not supplied

Polyglucosan bodies in brain tissue: a systematic study.

The relation between age, sex and presence of polyglucosan bodies in the brain has been systematically studied in 64 patients who did not suffer from neurological brain disorders and in 2 cases with Lafora's disease. In the normal brain the number of polyglucosan bodies is related to increasing age. Under the age of 40, polyglucosan bodies can be found sporadically in cases without a neurological disorder, but in the cases of Lafora's disease their number is uncomparatively higher. A sex relation has not been found.

Adolescent↗