METABOLIC TOPOGRAPHY OF THE HEMIPARKINSONISM-HEMIATROPHY SYNDROME

We estimated regional and global metabolic rates for glucose using F-1 8-fluorodeoxyglucose (FDG) and PET in six patients with hemiparkinsoni sm-hemiatrophy syndrome (HPHA; mean age, 41.0 +/- 12.4 years). We used F-18-fluorodopa (FDOPA) and PET in two patients to quantify presynapt ic nigrostriatal dopaminergic function. We compared measures of brain glucose metabolism and striatal FDOPA uptake with those calculated for 10 age-matched normal volunteers (mean age, 35.1 +/- 8.0 years) and 1 0 patients with typical unilateral Parkinson's disease (unilat-PD; mea n age, 58.2 +/- 13.8 years). All six HPHA patients demonstrated signif icant metabolic reductions (> 3 SD) in the con tralateral basal gangli a or frontal cortex as compared with normal control values. Mean norma lized glucose metabolism was reduced in the contralateral caudate and lentiform nuclei (p < 0.005) as compared with that in unilat-PD and no rmal controls. In both patients studied with FDOPA, contralateral stri atal uptake was significantly reduced (> 3 SD) as compared with normal control values. These results suggest that the clinical manifestation s of HPHA arise through a combination of pre- and postsynaptic nigrost riatal dopaminergic dysfunction. FDG and PET may be useful in differen tiating this disorder from typical unilat-PD.