[Characteristics of central neurotransmitter metabolism in hereditary extrapyramidal disorders]

The interrelationships were studied between catecholaminergic and cholinergic systems in 169 patients with extrapyramidal system diseases: 68 patients with torsion dystonia (58 with the rigid form and 10 with the hyperkinetic form), 10 with Hallervorden-Spatz disease, 61 with hepatolenticular degeneration, and in 40 with idiopathic tremor. The secretion of dopamine (DA), noradrenaline (NA), adrenaline (A) and their precursor--DOPA) as well as the activity of acetylcholinesterase (AChe)--the enzyme disintegrating acetylcholine--were determined. In the rigid form of torsion dystonia and in Hallervorden-Spatz disease reduced secretion of all catecholamines (mainly DA) and DOPA was observed, with decreased AChE activity. In the hyperkinetic form of torsion dystonia the secretion of DA was increased and AChE activity was higher. In the patients with idiopathic tremor the secretion of A and NA was decreased and AChE activity was reduced. In patients with hepatolenticular degeneration the secretion of NA and DA was decreased and that of their immediate precursor DOPA was increased. Changes of AChE activity showed a wide range. The observed disturbances reflect various forms of disturbances in the equilibrium between the catecholaminergic and cholinergic systems which are one of the leading pathogenetic mechanisms in the development of various extrapyramidal syndromes.