Serum dopamine beta hydroxylase activity and metoclopramide provocative test in torsion dystonia

Abstract

A familial study of 13 Ashkenasy Jewish patients suffering from torsion dystonia (TD) of various origin, was carried out using clinical observation and determination of serum dopamine-beta-hydroxylase (DBH) activity. None of the following criteria: Jewish origin, dominant trait, slow progression and axial topography are specific for an elevated serum DBH activity. A significantly high serum DBH activity was found in TD patients and their relatives compared with controls, particularly in the childhood onset group. Intravenous injection of 20 mg Metoclopramide (MP) did not change serum DBH activity in 12 patients and 17 relatives. In one patient with symptomatic dystonia and two of her maternal relatives (from different fathers) serum DBH activity increased after i.v. injection of MP. In these three cases the serum DBH activity doubled and tripled. It is suggested that the MP induced elevation of serum DBH activity in relatives of TD patients indicates a genetic disposition.

Characterization of the rat mutant dystonic (dt): a new animal model of dystonia musculorum deformans

“…Abnormalities in catecholamine metabolism have been suggested on the basis of some limited clinical data. Specifically, increased activity of plasma dopamine P-hydroxylase (Askenasy et al, 1980;Wooten et al, 1973), elevation of plasma norepinephrine (Zeigler, 1976), and decreased ventricular fluid levels of the norepinephrine metabolite, 3-methoxy-4-hydroxyphenylglycol (MHPG) (Wolfson et al, 1983), have been reported. There is also some evidence that patients with adult-but not childhoodonset dystonia may be distinguished by decreased cerebrospinal levels of the dopamine metabolite homovanillic acid (Tabaddor et al, 1978).…”

Is monoamine oxidase inhibitor induced myoclonus serotoninergically mediated?

“…Apomorphine and DA agonists were shown to suppress HTP-induced myoclonus and haloperidol or pimozide blocked this effect (Volkamn et al, 1978). L-Dopa induced myclonus was blocked by 5-HT antagonists and interrelations among receptors were proved (Klawans et al, 1975;Askenasy et al, 1980). L-Tryptophan induced myoclonus was observed in patients suffering from Down's syndrome, epilepsy and other brain diseased (Lieberman et al, 1986;Lowe et al, 1959;Keller, 1977).…”

Central and peripheral effects of iminodipropionitrile on catecholamine metabolism in rats

“…More recently, the authors of a thorough biochemical and neuropathological study of two patients who suffered from dystonia musculorum deformans concluded that the most consistent changes in these individuals were found in the central NE system (Hornykiewicz et al, 1986). It has also been previously reported that both plasma NE and dopamine beta-hydroxylase were increased in torsion dystonia (Askenasy et al, 1980;Epstein et al, 1974;Wooten et al, 1973;Ziegler et al, 1976). Thus, both the animal and human studies suggest that the norepinephrine system may be important in the manifestations of dyskinetic abnormalities.…”