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Updated: Jun 1, 2026

Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
Published on: June 9, 2018
Association between Age and Striatal Volume Stratified by CAG Repeat Length in Prodromal Huntington Disease
Elizabeth Aylward1, James Mills, Dawei Liu
1Center for Integrative Brain Research, Seattle Children's Research Institute; Department of Psychiatry, The University of Iowa Carver College of Medicine; Department of Biostatistics, The University of Iowa, College of Public Health; Department of Psychiatry, Pediatrics, and Neurology, The University of Iowa Carver College of Medicine and Division of Neurobiology, Department of Psychiatry, Johns Hopkins University.
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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