title: Positron emission tomographic findings in Filipino X-linked dystonia-parkinsonism.
abstract: Regional and global metabolic rates for glucose (rCMRGlc and GMR) were estimated using [18F]fluorodeoxyglucose and positron emission tomography in 3 patients with Filipino X-linked dystonia-parkinsonism (lubag). In all 3 patients a selective reduction in normalized striatal glucose metabolism (rCMRGlc/GMR) was observed compared with 15 normal volunteer subjects. Presynaptic nigrostriatal function was assessed in these patients using [18F]fluorodopa and positron emission tomography. Striatal rate constants for [18F]flurodopa 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 localized to the striatum and that clinical parkinsonism in these patients may be secondary to extranigral factors.
authors.affiliation: Department of Neurology, North Shore University Hospital/Cornell University Medical College, Manhasset, NY 11030.
authors.forename: D
authors.initials: D
authors.lastname: Eidelberg
authors.forename: S
authors.initials: S
authors.lastname: Takikawa
authors.forename: K
authors.initials: K
authors.lastname: Wilhelmsen
authors.forename: V
authors.initials: V
authors.lastname: Dhawan
authors.forename: T
authors.initials: T
authors.lastname: Chaly
authors.forename: W
authors.initials: W
authors.lastname: Robeson
authors.forename: R
authors.initials: R
authors.lastname: Dahl
authors.forename: D
authors.initials: D
authors.lastname: Margouleff
authors.forename: P
authors.initials: P
authors.lastname: Greene
authors.forename: A
authors.initials: A
authors.lastname: Hunt
mesh_terms: D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism; D016022:Case-Control Studies; D004421:Dystonia / Q000000981:diagnostic imaging / Q000235:genetics* / Q000378:metabolism; D008040:Genetic Linkage; D005947:Glucose / Q000378:metabolism; D006801:Humans; D008297:Male; D010300:Parkinson Disease / Q000000981:diagnostic imaging / Q000235:genetics* / Q000378:metabolism; D010679:Philippines; D014055:Tomography, Emission-Computed; D014960:X Chromosome
chemical_list: D005947:Glucose