Dopamine D2 receptor binding and cerebral glucose metabolism recover after D-penicillamine-therapy in Wilson's disease.
Regional cerebral glucose metabolism (rCMRGlc) and dopamine D2 receptor binding were measured in a 31-year-old, severely affected, untreated patient with Wilson's disease of 3 years' duration using positron emission tomography and 18F-deoxyglucose and 18F-methylspiperone ([18F]MSP), respectively. There was a severe reduction of striatal and extrastriatal rCMRGlc as well as of striatal [18F]MSP accumulation rate. After 1 year of treatment with D-penicillamine, striatal and extrastriatal rCMRGlc and striatal [18F]MSP accumulation rate reached almost normal levels. It is hypothesized that recovery of motor functions due to copper trapping therapy was associated with an increase in basal ganglia activity and a re-expression or upregulation of dopamine D2 receptors.
Department of Neurology, Heinrich Heine University, Düsseldorf, Germany.
G
G
Schlaug
H
H
Hefter
B
B
Nebeling
V
V
Engelbrecht
P
P
Weiss
G
G
Stöcklin
R J
RJ
Seitz
D000328:Adult; D001479:Basal Ganglia / Q000378:metabolism; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D003847:Deoxyglucose; D015259:Dopamine Agents; D005462:Fluorine Radioisotopes; D005947:Glucose / Q000378:metabolism*; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009474:Neurons / Q000378:metabolism; D010396:Penicillamine / Q000627:therapeutic use*; D017448:Receptors, Dopamine D2 / Q000378:metabolism*; D013134:Spiperone / Q000031:analogs & derivatives; D014055:Tomography, Emission-Computed
D015259:Dopamine Agents; D005462:Fluorine Radioisotopes; D017448:Receptors, Dopamine D2; D013134:Spiperone; C039167:3-N-methylspiperone; D003847:Deoxyglucose; D010396:Penicillamine; D005947:Glucose