DOPAMINE D2 RECEPTOR-BINDING AND CEREBRAL GLUCOSE-METABOLISM RECOVER AFTER D-PENICILLAMINE-THERAPY IN WILSONS-DISEASE

Regional cerebral glucose metabolism (rCMRGlc) and dopamine D2 recepto r binding were measured in a 31-year-old, severely affected, untreated patient with Wilson's disease of 3 years' duration using positron emi ssion tomography and F-18-deoxyglucose and F-18-methylspiperone ([F-18 ]MSP), respectively. There was a severe reduction of striatal and extr astriatal rCMRGlc as well as of striatal [F-18]MSP accumulation rate. After 1 year of treatment with D-penicillamine, striatal and extrastri atal rCMRGlc and striatal [F-18]MSP accumulation rate reached almost n ormal levels. It is hypothesized that recovery of motor functions due to copper trapping therapy was associated with an increase in basal ga nglia activity and a re-expression or upregulation of dopamine D2 rece ptors.