title: Fluorodopa and raclopride PET analysis of patients with Machado-Joseph disease.
abstract: We performed [18F]6-fluoro-L-dopa (6-FD) and [11C]raclopride (RAC) PET studies in six patients with Machado-Joseph disease (MJD) (age, 17 to 61 years; duration of illness, 3 to 10 years), normal controls (n = 10 in 6-FD-PET, n = 8 in RAC-PET), and patients with idiopathic parkinsonism (n = 15 in 6-FD-PET). The youngest patient with MJD had prominent dystonia and pyramidal features (type 1 MJD), whereas the remainder were prominently ataxic (types 2 and 3 MJD). Striatal RAC binding was normal in patients with MJD. Striatal 6-FD influx constants (Ki) were low in the range of idiopathic parkinsonism in two patients with MJD (youngest and oldest patients), whereas striatal Ki were normal in the remaining patients with MJD. The impairment of the nigrostriatal dopaminergic pathway did not correlate with the phenotype, CAG repeat length, disease duration, or age of onset of patients with MJD. Our results suggest that striatal D2 receptors are normal and the nigral damage is diverse in MJD.
authors.affiliation: Neurodegenerative Disorders Centre, University of British Columbia, Canada.
authors.forename: H
authors.initials: H
authors.lastname: Shinotoh
authors.forename: B
authors.initials: B
authors.lastname: Thiessen
authors.forename: B J
authors.initials: BJ
authors.lastname: Snow
authors.forename: S
authors.initials: S
authors.lastname: Hashimoto
authors.forename: P
authors.initials: P
authors.lastname: MacLeod
authors.forename: I
authors.initials: I
authors.lastname: Silveira
authors.forename: G A
authors.initials: GA
authors.lastname: Rouleau
authors.forename: M
authors.initials: M
authors.lastname: Schulzer
authors.forename: D B
authors.initials: DB
authors.lastname: Calne
mesh_terms: D000293:Adolescent; D000328:Adult; D002421:Caudate Nucleus / Q000378:metabolism; D002531:Cerebellum / Q000378:metabolism; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives* / Q000493:pharmacokinetics; D018492:Dopamine Antagonists / Q000378:metabolism; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000000981:diagnostic imaging* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D010300:Parkinson Disease / Q000000981:diagnostic imaging / Q000378:metabolism; D011699:Putamen / Q000378:metabolism; D020891:Raclopride; D012016:Reference Values; D012457:Salicylamides / Q000378:metabolism; D014018:Tissue Distribution; D014055:Tomography, Emission-Computed
chemical_list: D018492:Dopamine Antagonists; D012457:Salicylamides; C043437:fluorodopa F 18; D020891:Raclopride; D004295:Dihydroxyphenylalanine