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Updated: Jun 17, 2026
Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
Published on: June 9, 2018
Composite UHDRS Correlates With Progression of Imaging Biomarkers in Huntington's Disease
Carlos Estevez-Fraga1, Rachael I Scahill1, Alexandra Durr2
1Huntington's Disease Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK.
Insights
The composite Unified Huntington's Disease Rating Scale (cUHDRS) tracks Huntington's disease progression. Worse cUHDRS scores correlate with brain atrophy and altered white matter integrity, supporting its use in clinical trials.
Area of Science:
- Neuroimaging
- Neurodegeneration
- Clinical Trials
Background:
- The composite Unified Huntington's Disease Rating Scale (cUHDRS) is a key measure for assessing Huntington's disease (HD) progression.
- It is utilized as a primary outcome in clinical trials for huntingtin-lowering therapies.
Purpose of the Study:
- To evaluate the volumetric and structural connectivity correlates of the cUHDRS in individuals with HD.
- To establish the biological relevance of the cUHDRS by linking it to neuroimaging biomarkers.
Main Methods:
- 119 premanifest and 119 early-HD participants underwent voxel-based morphometry for gray and white matter volume analysis.
- Tract-based spatial statistics were used to examine correlations between baseline fractional anisotropy (FA), diffusivity measures, and baseline cUHDRS scores.
Main Results:
- Longitudinal decreases in occipito-parietal cortex and centrum semiovale volumes correlated with worsening cUHDRS scores over time.
- Lower baseline cUHDRS scores were associated with reduced basal ganglia and surrounding white matter volumes, as well as decreased FA and increased diffusivity.
Conclusions:
- The cUHDRS demonstrates biological relevance by correlating with established neuroimaging biomarkers.
- These findings support the cUHDRS's utility in tracking atrophy progression in Huntington's disease and its role in clinical trials.
Background:
The composite Unified Huntington's Disease Rating Scale (cUHDRS) is a multidimensional measure of progression in Huntington's disease (HD) being used as a primary outcome in clinical trials investigating potentially disease-modifying huntingtin-lowering therapies.
Objective:
Evaluating volumetric and structural connectivity correlates of the cUHDRS.
Methods:
One hundred and nineteen premanifest and 119 early-HD participants were included. Gray and white matter (WM) volumes were correlated with cUHDRS cross-sectionally and longitudinally using voxel-based morphometry. Correlations between baseline fractional anisotropy (FA); mean, radial, and axial diffusivity; and baseline cUHDRS were examined using tract-based spatial statistics.
Results:
Worse performance in the cUHDRS over time correlated with longitudinal volume decreases in the occipito-parietal cortex and centrum semiovale, whereas lower baseline scores correlated with decreased volume in the basal ganglia and surrounding WM. Lower cUHDRS scores were also associated with reduced FA and increased diffusivity at baseline.
Conclusion:
The cUHDRS correlates with imaging biomarkers and tracks atrophy progression in HD supporting its biological relevance. © 2021 International Parkinson and Movement Disorder Society.
