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Association between Age and Striatal Volume Stratified by CAG Repeat Length in Prodromal Huntington Disease
Author(s) -
Elizabeth Aylward,
James A. Mills,
Dawei Liu,
Peg Nopoulos,
Christopher A. Ross,
Ronald Pierson,
Jane S. Paulsen
Publication year - 2011
Publication title -
plos currents
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.282
H-Index - 49
ISSN - 2157-3999
DOI - 10.1371/currents.rrn1235
Subject(s) - huntington's disease , atrophy , brain size , disease , medicine , pathology , magnetic resonance imaging , radiology
Background: Longer CAG repeat length is associated with faster clinical progression in Huntington disease, although the effect of higher repeat length on brain atrophy is not well documented. Method: Striatal volumes were obtained from MRI scans of 720 individuals with prodromal Huntington disease. Striatal volume was plotted against age separately for groups with CAG repeat lengths of 38–39, 40, 41, 42, 43, 44, 45, 46, and 47–54. Results: Slopes representing the association between age and striatal volume were significantly steeper as CAG repeat length increased. Discussion: Although cross-sectional, these data suggest that striatal atrophy, like clinical progression, may occur faster with higher CAG repeat lengths.

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