Characterization of dopaminergic dysfunction in familial progressive supranuclear palsy: an 18F-dopa PET study.
We analyzed (18)F-dopa PET data from 11 members of kindreds with familial progressive supranuclear palsy (PSP) to characterize their cerebral dopaminergic dysfunction. Three clinically-affected PSP patients showed reduced (18)F-dopa uptake in the striatum, orbitofrontal cortex and amygdala. One asymptomatic subject exhibited progressive putamen dopaminergic dysfunction. 60% of subjects with abnormal (18)F-dopa scans developed PSP subsequently. This is the first in vivo documentation of cortical dopaminergic deficiency in PSP. Reduced striatal (18)F-dopa uptake in susceptible relatives may predict later clinical disease.
Tai
Y F
YF
MRC Clinical Sciences Centre and Division of Neurosciences, Hammersmith Hospital, Imperial College London, United Kingdom.
Ahsan
R L
RL
de Yébenes
J G
JG
Pavese
N
N
Brooks
D J
DJ
Piccini
P
P
D000368:Aged; D000679:Amygdala / Q000000981:diagnostic imaging / Q000378:metabolism / Q000503:physiopathology; D002540:Cerebral Cortex / Q000000981:diagnostic imaging / Q000378:metabolism / Q000503:physiopathology; D003342:Corpus Striatum / Q000000981:diagnostic imaging / Q000378:metabolism / Q000503:physiopathology; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives* / Q000493:pharmacokinetics; D018450:Disease Progression; D004298:Dopamine / Q000378:metabolism*; D015536:Down-Regulation / Q000502:physiology; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008032:Limbic System / Q000000981:diagnostic imaging* / Q000378:metabolism* / Q000503:physiopathology; D008297:Male; D019965:Neurocognitive Disorders / Q000000981:diagnostic imaging / Q000378:metabolism / Q000503:physiopathology; D010375:Pedigree; D049268:Positron-Emission Tomography / Q000379:methods; D011237:Predictive Value of Tests; D012016:Reference Values; D013494:Supranuclear Palsy, Progressive / Q000000981:diagnostic imaging* / Q000378:metabolism* / Q000503:physiopathology
C043437:fluorodopa F 18; D004295:Dihydroxyphenylalanine; D004298:Dopamine