Fluorodopa and raclopride PET analysis of patients with Machado-Joseph disease.
Identifieur interne : 002606 ( Ncbi/Checkpoint ); précédent : 002605; suivant : 002607Fluorodopa and raclopride PET analysis of patients with Machado-Joseph disease.
Auteurs : H. Shinotoh [Canada] ; B. Thiessen ; B J Snow ; S. Hashimoto ; P. Macleod ; I. Silveira ; G A Rouleau ; M. Schulzer ; D B CalneSource :
- Neurology [ 0028-3878 ] ; 1997.
English descriptors
- KwdEn :
- Adolescent, Adult, Caudate Nucleus (metabolism), Cerebellum (metabolism), Dihydroxyphenylalanine (analogs & derivatives), Dihydroxyphenylalanine (pharmacokinetics), Dopamine Antagonists (metabolism), Female, Humans, Machado-Joseph Disease (diagnostic imaging), Machado-Joseph Disease (metabolism), Male, Middle Aged, Parkinson Disease (diagnostic imaging), Parkinson Disease (metabolism), Putamen (metabolism), Raclopride, Reference Values, Salicylamides (metabolism), Tissue Distribution, Tomography, Emission-Computed.
- MESH :
- chemical , analogs & derivatives : Dihydroxyphenylalanine.
- diagnostic imaging : Machado-Joseph Disease, Parkinson Disease.
- metabolism : Caudate Nucleus, Cerebellum, Dopamine Antagonists, Machado-Joseph Disease, Parkinson Disease, Putamen, Salicylamides.
- chemical , pharmacokinetics : Dihydroxyphenylalanine.
- Adolescent, Adult, Female, Humans, Male, Middle Aged, Raclopride, Reference Values, Tissue Distribution, Tomography, Emission-Computed.
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.
PubMed: 9339702
Affiliations:
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pubmed:9339702Le document en format XML
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<front><div type="abstract" xml:lang="en">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.</div>
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<name sortKey="Snow, B J" sort="Snow, B J" uniqKey="Snow B" first="B J" last="Snow">B J Snow</name>
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