Infantile neuroaxonal dystrophy.
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The motor system of Dugesia gonocephala shows a striking similarity with the extrapyramidal system of high vertebrates and of man with the evidence of correlations between dopaminergic and cholinergic neurons. The utilization of this model seems to be useful in testing drugs which presumably act on dopaminergic or cholinergic transmission. In this model, the quantification of animal behaviour seems considerably easier when compared with the difficulties met in other animal models commonly employed. Besides, it might be anticipated that this model, if correctly used, can display interesting perspectives also in neuroendocrinological investigations.
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A putative neurotransmitter, 2-phenylethylamine, which is most highly concentrated in the extrapyramidal system of human brain, is able to reverse reserpine-induced parkinsonism in animals and elicit stereotypy. This action is only partially antagonized after catecholamine depletion by pretreatment with a-methyl-para-tyrosine, and fully blocked by pretreatment with haloperidol, a dopamine receptor blocker. Therefore, via direct and/or indirect actions, 2-phenylethylamine may serve a neuroregulatory role in the extrapyramidal system.
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The author investigated the subclinical tremor on the extremity muscles in stutterers at rest, without speaking. This tremor occurred in the native EMG record from the musculus abductor digiti quinti during isometric contraction. Because it occurred in a muscle not involved in speaking and moreover at rest, without speaking, the author excludes the possibility that it could be an increase in the amplitude of physiological tremor; she concludes that the cause of this tremor are changes in the function of the structures responsible for motor feedback, especially in the extrapyramidal and cerebellar field.
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Abundant preclinical evidence suggests that serotonin-containing neural systems may participate in the regulation of both extrapyramidal and neuroendocrine function. In an attempt to examine these possibilities in man, patients with various neurologic disorders received drugs believed to facilitate or inhibit serotonergic function. Extrapyramidal signs in patients with parkinsonism or Huntington's disease showed no consistent change with L-tryptophan or parachlorophenylalanine. Unexpectedly, L-5-hydroxytryptophan, given in combination with a peripheral decarboxylase inhibitor, caused a worsening of parkinsonian akinesia and rigidity. Fenfluramine, at doses which appeared to diminish central serotonin but not dopamine turnover, had no consistent effect on the severity of involuntary movements in patients with Huntington's chorea, but did produce a significant rise in plasma prolactin.
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