Presynaptic nigrostriatal function in genetically tested asymptomatic relatives from the pallido-ponto-nigral degeneration family

Details

Serval ID
serval:BIB_09D66A2C2697
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Presynaptic nigrostriatal function in genetically tested asymptomatic relatives from the pallido-ponto-nigral degeneration family
Journal
Neurology
Author(s)
Kishore  A., Wszolek  Z. K., Snow  B. J., de la Fuente-Fernandez  R., Arwert  F., Wijker  M., Schulzer  M., Calne  D. B., Vingerhoets  F. J.
ISSN
0028-3878 (Print)
Publication state
Published
Issued date
12/1996
Volume
47
Number
6
Pages
1588-90
Notes
Journal Article
Research Support, Non-U.S. Gov't --- Old month value: Dec
Abstract
Pallido-ponto-nigral degeneration (PPND) is a dominantly inherited disorder with parkinsonism. We performed PET with [18F]fluorodopa (FD) and, later, gene testing in 12 asymptomatic relatives at risk from a family with PPND and compared the striatal FD uptake constant (Ki) in them with 4 symptomatic individuals and 10 normal control subjects. Four relatives with positive linkage had a significantly reduced Ki from the normal control subjects but to a lesser degree than the symptomatic patients. The mean Ki in the relatives with negative linkage (n = 8) did not differ from normal control subjects. In conclusion, we identified reduced dopaminergic function in asymptomatic relatives with positive genetic linkage from the PPND family. Most of the reduction in this disorder occurs in the fifth decade, when the disease manifests clinically.
Keywords
Adult Corpus Striatum/*radionuclide imaging Globus Pallidus/*radionuclide imaging Humans Middle Aged Parkinson Disease/*radionuclide imaging Pons/*radionuclide imaging Substantia Nigra/*radionuclide imaging Tomography, Emission-Computed
Pubmed
Web of science
Create date
25/01/2008 12:49
Last modification date
20/08/2019 12:31
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