RELATIONSHIP BETWEEN STRIATAL GLUCOSE CONSUMPTION AND COPPER EXCRETION IN PATIENTS WITH WILSONS-DISEASE TREATED WITH D-PENICILLAMINE

In 12 patients with Wilson's disease treated with D-penicillamine (DPA), the regional cerebral metabolic rate of glucose consumption of the lentiform and caudate nucleus was analysed using the Fluorodeoxyglucose-18 method and correlated with the clinical symptoms of the patients, the ceruloplasmin level, the serum level of free copper and the 24-h copper excretion. The more copper was eliminated, the higher was the basal ganglia glucose consumption. On the other hand, in seven patients who had been treated for more than 7 years a significant decline of the basal ganglia glucose consumption was observed, suggesting too low a maintenance dose of DPA.