Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A study of the incidence of R. fever in an area in southeast Teheran during the period 1972-1974 revealed 92 cases. The crude annual incidence of R. fever ranged from a high of 58 cases to a low of 51 cases per 100,000 population. The age and sex specific rates revealed the incidence to be strikingly higher in the 5-19 year age group, reaching roughly 80/100,000. There was an approximately similar number of males and females with R. fever. Among the R. fever patients, there were 6 cases of chorea, 5 girls and 1 boy. From 92 R. fever cases, 49 (53%) developed carditis. Among these patients, 35 (71%) were female in contrast to 14 (29%) male cases. Overall there were 59 cases (62%) which were initial attacks and 33 cases (38%) which were recurrences. During the trial there were also 7 recurrences of R fever and 4 deaths. The seasonal pattern showed that the majority of cases occurred during the winter and spring months. From the R. fever/R.H.D. patients, 63 (70%) and from cases with carditis alone, 24 (40%) were hospitalized.
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PMID:Rheumatic fever and rheumatic heart disease among 56,8000 inhabitants in southeast Teheran from 1972-1974. 1 55

Kainic acid lesion of cell bodies in the dorsal striatum enhanced the stereotypy-producing effects of d-amphetamine without affecting the sterotypy produced by the direct receptor agonist apomorphine. This pattern of results parallels that found in patients suffering from Hungtington's chorea, thus strengthening the parallels between the kainic acid animal model and the human disease state initially suggested on biochemical gounds. The present results further suggest a dissociation of the mechanisms involved in the production of stereotypy by these two drugs, perhaps in terms of differential involvement of the striato-nigral negative feedback loop.
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PMID:Kainic acid lesions of the striatum dissociate amphetamine and apomorphine stereotypy: similarities to Huntingdon's chorea. 2 76

The striatum constitutes the most voluminous basal ganglia in man. It is issued from ganglionic eminences which are very early bound by limbic kernels. If the cortical and reticulo-spinal projections have been first described the existence of anatomical connexions with the limbic system offers a large number of functional possibilities. The knowledge of the distribution of the different chemical substances which are present within this structure as well as the enzymes necessary for their synthesis and destruction permits to establish a chemical mapping, the dopaminergic one being the best known. The dopaminergic synaptic function in the striatum helps to understand the respective roles of the pre and post-synaptic receptors as well as the mechanisms by which the other neuromediators can modulate the dopaminergic activity, the cyclic nucleotides being often necessary for this action. These fundamental data subtend the mechanism of action of most of the drugs which are involved in extrapyramidal phenomenons (neuroleptics, dopaminergic agonists) and allows to put forth physiopathological hypothesis on Parkinson disease, Huntington chorea, as well as certain induced or spontaneous dyskinetic states. The functions of the striatum are then evoked: if the role of this structure in motor control is critical, its involvement in complex behaviours is strongly suggested.
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PMID:[Neuropharmacological data on the striatum]. 3 37

This study reviews the available literature concerning clinical evidence of the effects of ovarian steroid hormones on the brain. The negative effects of estrogen on chorea gravidarum and in women using oral contraception (OC) is well known. Estrogens, however, can at times be beneficial in patients with movement disorders. The fall of progesterone level at menstruation can be associated with catamenial epilepsy, while estrogen increases epileptiform electroencephalogram activity. Both estrogens and progesterone affect neurotransmitters by altering absolute concentration and turnover in brain tissue. By interfering with monoamine oxidase, estrogens may increase dopamine, and it is possible that they can worsen brain pseudotumor, although data available are not very clear on this point. The possible role of ovarian or male steroid hormones in other neurological disorders is unknown. Further studies are needed, since it is apparent that not all types of abnormal movements respond in the same way to treatment with such steroids.
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PMID:Ovarian steroid hormones and cerebral function. 4 83

Enzymes concerned with neurotransmitter metabolism were measured postmortem in 50 regions from the brains of 11 chronic schizophrenics, 2 patients with senile dementia, 1 depressive, and 18 controls. Enzymes studied were tyrosine hydroxylase, dopa decarboxylase, glutamic decarboxylase, choline acetyltransferase (CAT), and acetylcholinesterase. The schizophrenic group had high CAT activities in the hippocampus, caudate, putamen, and nucleus accumbens; the other patients from the same hospital did not. A compensatory response to long- or short-term drug usage is considered, but correlations are hard to establish in the group studied. An alternative hypothesis proposes that the high levels are a compensatory response to defective cholinergic receptors in the affected areas. On this hypothesis, and by analogy with chorea, dopaminergic antagonists would act in schizophrenia by helping to reestablish cholinergic-dopaminergic balance.
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PMID:Possible changes in striatal and limbic cholinergic systems in schizophrenia. 4 82

The frequency of HLA antigens was determined in 133 patients with acquired valvular heart disease and compared with the frequency of HLA antigens in 1,000 normal individuals by the relative incidence ratio method of Woolf (1955). No significant increases were observed. However, when the patients were divided into those with no rheumatic history and those who have had rheumatic fever or chorea, a significant increase in AW 30/31 and A29 was observed in the group with no rheumatic history.
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PMID:HLA antigens and acquired valvular heart disease. 5 62

A micromethod for the investigation of the fatty acid composition of sphingomyelin in presented. In the cerebral white matter of 17 normal adult brains, analyzed for reference, the predominant fatty acids are C 18:0 and C 24:1. Our results are in agreement with those of other authors. Short chained fatty acids are relatively increased in young children; this shift is typical of "immature" myelin. Similar changes are described here in old persons and cases of non-specific brain damage associated with demyelination (autolysis, chronic uremia, juvenile chorea). Sphingomyelin fatty acid composition can be considered a sensitive measure of both disturbed myelination and demyelination.
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PMID:The fatty acid composition of sphingomyelin from adult human cerebral white matter and changes in childhood, senium and unspecific brain damage. 7 95

A familial disorder was characterized by chorea, ataxia, myoclonus, convulsions, dementia, and mental retardation. In five cases, the main lesion affected cerebellar dentate nuclei, with nerve cell loss, gliosis, chromatolysis, and grumose degeneration. Fibrous glial cell proliferation was detected in the globus pallidus.
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PMID:Familial chorea and myoclonus epilepsy. 9 88

Ten consecutive patients with chorea of different etiologies were treated with trifluoroperazine, 7 were adults and 3 children; all the patients received symptomatic benefit from the medication, the effective dose ranged from 4 to 16 milligrams. The medication was well tolerated and proved to be effective a long period of time. Biochemical and pathophysiological aspects of chorea and its relations to the medication are discussed.
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PMID:Trifluoroperazine for the symptomatic treatment of chorea. 12 26

Diphenylhydantoin (DPH) diminished the therapeutic effects of levodopa both in patients with parkinsonism and in patients with chronic manganese poisoning, as well as the levodopa-dependent dyskinesia for which the former were selected. In patients with Huntington chorea, it enhanced chorea and mental agitation and, thus, failed to conform with the postulated pharmacological reciprocity between Parkinson disease and Huntington chorea. These findings are in agreement with experiments done in animals in which DPH blocked a neuronal response to dopamine.
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PMID:Diphenylhydantoin. Blocking of levodopa effects. 12 56


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