Journal article
Composite UHDRS Correlates With Progression of Imaging Biomarkers in Huntington's Disease
Movement disorders, Vol.36(5), pp.1259-1264
01/20/2021
DOI: 10.1002/mds.28489
PMID: 33471951
Abstract
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.
Evaluating volumetric and structural connectivity correlates of the cUHDRS.
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.
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.
The cUHDRS correlates with imaging biomarkers and tracks atrophy progression in HD supporting its biological relevance. © 2021 International Parkinson and Movement Disorder Society.
Details
- Title: Subtitle
- Composite UHDRS Correlates With Progression of Imaging Biomarkers in Huntington's Disease
- Creators
- Carlos Estevez-Fraga - Huntington's Disease Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UKRachael I Scahill - Huntington's Disease Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UKAlexandra Durr - Sorbonne Université, Paris Brain Institute (ICM), AP-HP, Inserm, CNRS, Pitié-Salpêtrière University Hospital, Paris, FranceBlair R Leavitt - Centre for Molecular Medicine and Therapeutics, Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia, CanadaRaymund A C Roos - Department of Neurology, Leiden University Medical Center, Leiden, The NetherlandsDouglas R Langbehn - Department of Psychiatry, University of Iowa, Iowa City, Iowa, USAGeraint Rees - Wellcome Centre for Neuroimaging, UCL Queen Square Institute of Neurology, University College London, London, United KingdomSarah Gregory - Huntington's Disease Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UKSarah J Tabrizi - Huntington's Disease Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK
- Resource Type
- Journal article
- Publication Details
- Movement disorders, Vol.36(5), pp.1259-1264
- DOI
- 10.1002/mds.28489
- PMID
- 33471951
- NLM abbreviation
- Mov Disord
- ISSN
- 0885-3185
- eISSN
- 1531-8257
- Publisher
- United States
- Language
- English
- Date published
- 01/20/2021
- Academic Unit
- Psychiatry; Iowa Neuroscience Institute
- Record Identifier
- 9984066147902771
Metrics
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