POSITRON EMISSION TOMOGRAPHIC FINDINGS IN FILIPINO X-LINKED DYSTONIA-PARKINSONISM

Regional and global metabolic rates for glucose (rCMRGlc and GMR) were estimated using [F-18]fluorodeoxyglucose and positron emission tomogr aphy in 3 patients with Filipino X-linked dystonia-parkinsonism (lubag ). In all 3 patients a selective reduction in normalized striatal gluc ose metabolism (rCMRGlc/GMR) was observed compared with 15 normal volu nteer subjects. Presynaptic nigrostriatal function was assessed in the se patients using [F-18]fluorodopa and positron emission tomography. S triatal rate constants for [F-18]fluorodopa uptake were found to be in the normal range in all 3 patients with lubag. These findings suggest that the extrapyramidal manifestations of lubag are metabolically loc alized to the striatum and that clinical parkinsonism in these patient s may be secondary to extranigral factors.