Document Detail

Familial progressive supranuclear palsy: detection of subclinical cases using 18F-dopa and 18fluorodeoxyglucose positron emission tomography.
MedLine Citation:
PMID:  11708994     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Progressive supranuclear palsy (PSP) is generally considered to be a sporadic disease; however, occasional cases of familial PSP have been described. The rarity of reports of familial PSP may be attributed in part to an inability to detect subclinical disease in affected relatives who subsequently die before symptoms clinically develop. OBJECTIVE: To study regional cerebral dopaminergic function and glucose metabolism in members of 2 large kindreds with familial PSP to identify subclinical cases. METHODS: Three clinically affected members from the 2 PSP kindreds were scanned with both (18)F-dopa and (18)fluorodeoxyglucose ((18)FDG) positron emission tomography (PET). Fifteen asymptomatic first-degree relatives were scanned with (18)F-dopa PET; 10 of them also underwent a second PET study with (18)FDG. RESULTS: All 3 clinically affected PSP patients showed a significant reduction in caudate and putamen (18)F-dopa uptake along with a significant reduction in striatal, lateral, and medial premotor area and dorsal prefrontal cortex glucose metabolism. In 4 of the 15 asymptomatic relatives, caudate and putamen (18)F-dopa uptake was 2.5 SDs lower than the normal mean. These 4 subjects and a fifth asymptomatic relative with normal (18)F-dopa uptake showed a significant reduction of cortical and striatal glucose metabolism in a pattern similar to that of their affected relatives. CONCLUSION: (18)F-dopa and (18)FDG PET allowed us to identify 5 cases with subclinical metabolic dysfunction among 15 subjects (33%) at risk for PSP, suggesting that this approach is useful for characterizing the pattern of aggregation in PSP kindreds.
P Piccini; J de Yebenez; A J Lees; R Ceravolo; N Turjanski; P Pramstaller; D J Brooks
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Archives of neurology     Volume:  58     ISSN:  0003-9942     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2001 Nov 
Date Detail:
Created Date:  2001-11-15     Completed Date:  2001-12-07     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1846-51     Citation Subset:  AIM; IM    
MRC Clinical Science Center, Imperial College School of Medicine, Hammersmith Hospital, DuCane Road, W12 ONN London, England.
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MeSH Terms
Caudate Nucleus / metabolism,  radionuclide imaging*
Dihydroxyphenylalanine / chemistry,  metabolism
Dopamine Agents / chemistry,  metabolism
Fluorine Radioisotopes / chemistry,  metabolism
Fluorodeoxyglucose F18 / metabolism
Genetic Predisposition to Disease
Middle Aged
Putamen / metabolism,  radionuclide imaging*
Radiopharmaceuticals / metabolism
Supranuclear Palsy, Progressive / diagnosis*,  metabolism,  radionuclide imaging
Tomography, Emission-Computed
Reg. No./Substance:
0/Dopamine Agents; 0/Fluorine Radioisotopes; 0/Radiopharmaceuticals; 63-84-3/Dihydroxyphenylalanine; 63503-12-8/Fluorodeoxyglucose F18

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