tiodonium-chloride and Hepatolenticular-Degeneration

In 48 patients with hepatocerebral dystrophy the principal parameters of the catecholamine metabolism were examined. Pronounced changes characterized primarily by a considerable diminution of dopamine and noradrenaline excretion and an increase of the excretion of their immediate precursor, i.e. DOPA, were noted. On the basis of the data obtained it was assumed that the synthesis of these neurotransmitting catecholamines was depressed. In the course of treatment with d-penicillamine which is capable of mobilizing copper and stimulating its excretion from the body a statistically significant increase of the dopamine excretion (as compared with its initial level) was noted, and in patients with the graver rigid-arrhythmohyperkinetic form of the disease the excretion of not only dopamine, but also noradrenaline, was increased. It is supposed that the disturbances of catecholamine metabolism in hepatocerebral dystrophy have a complicated mechanism, and are associated with both the grave hepatic pathology and disturbances of copper metabolism. In this connection additional methods of treating that disease are offered. These methods influence catecholamine metabolism, and act primarily on extrapyramid motor, and also psychic disorders. The studies carried out by the author make a contribution to the existing concepts of the biochemical mechanisms of the hepatocerebral dystrophy pathogenesis, and expand the possibilities of treating the grave hereditary disease.

Topics: Adolescent; Adult; Catecholamines; Copper; Dopamine; Dopamine beta-Hydroxylase; Epinephrine; Hepatolenticular Degeneration; Homovanillic Acid; Humans; Norepinephrine; Thiophenes; Vanilmandelic Acid