title: Metabolic topography of the hemiparkinsonism-hemiatrophy syndrome.
abstract: We estimated regional and global metabolic rates for glucose using 18F-fluorodeoxyglucose (FDG) and PET in six patients with hemiparkinsonism-hemiatrophy syndrome (HPHA; mean age, 41.0 +/- 12.4 years). We used 18F-fluorodopa (FDOPA) and PET in two patients to quantify presynaptic 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 10 patients with typical unilateral Parkinson's disease (unilat-PD; mean age, 58.2 +/- 13.8 years). All six HPHA patients demonstrated significant metabolic reductions (> 3 SD) in the contralateral basal ganglia or frontal cortex as compared with normal control values. Mean normalized glucose metabolism was reduced in the contralateral caudate and lentiform nuclei (p < 0.005) as compared with that in unilat-PD and normal controls. In both patients studied with FDOPA, contralateral striatal uptake was significantly reduced (> 3 SD) as compared with normal control values. These results suggest that the clinical manifestations of HPHA arise through a combination of pre- and postsynaptic nigrostriatal dopaminergic dysfunction. FDG and PET may be useful in differentiating this disorder from typical unilat-PD.
authors.affiliation: Department of Neurology, Columbia University, New York, NY.
authors.forename: S
authors.initials: S
authors.lastname: Przedborski
authors.forename: N
authors.initials: N
authors.lastname: Giladi
authors.forename: S
authors.initials: S
authors.lastname: Takikawa
authors.forename: T
authors.initials: T
authors.lastname: Ishikawa
authors.forename: V
authors.initials: V
authors.lastname: Dhawan
authors.forename: P
authors.initials: P
authors.lastname: Spetsieris
authors.forename: T
authors.initials: T
authors.lastname: Chaly
authors.forename: S
authors.initials: S
authors.lastname: Fahn
authors.forename: D
authors.initials: D
authors.lastname: Eidelberg
mesh_terms: D000328:Adult; D001284:Atrophy; D001921:Brain / Q000000981:diagnostic imaging* / Q000378:metabolism* / Q000473:pathology; D003847:Deoxyglucose / Q000031:analogs & derivatives / Q000378:metabolism; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives / Q000378:metabolism; D005260:Female; D019788:Fluorodeoxyglucose F18; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010300:Parkinson Disease / Q000000981:diagnostic imaging / Q000378:metabolism*; D013577:Syndrome; D014055:Tomography, Emission-Computed
chemical_list: D019788:Fluorodeoxyglucose F18; C043437:fluorodopa F 18; D004295:Dihydroxyphenylalanine; D003847:Deoxyglucose; D005947:Glucose