Association between Age and Striatal Volume Stratified by CAG Repeat Length in Prodromal Huntington Disease

PLoS Curr. 2011 May 11;3:RRN1235. doi: 10.1371/currents.RRN1235.


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.