Huntington's disease (HD) produces progressive and ultimately widespread impairment of brain function. Neostriatal atrophy alone cannot account for whole-brain losses seen postmortem, and the mutant huntingtin protein and its neuropathologic sequelae are evident throughout the brain. Whole-brain atr
Whole-brain atrophy as a measure of progression in premanifest and early Huntington's disease
โ Scribed by Susie M.D. Henley; Edward J. Wild; Nicola Z. Hobbs; Chris Frost; David G. MacManus; Roger A. Barker; Nick C. Fox; Sarah J. Tabrizi
- Publisher
- John Wiley and Sons
- Year
- 2009
- Tongue
- English
- Weight
- 305 KB
- Volume
- 24
- Category
- Article
- ISSN
- 0885-3185
No coin nor oath required. For personal study only.
โฆ Synopsis
Abstract
Therapeutic trials in Huntington's disease (HD) are challenging as clinical progression is slow and variable and reliable biomarkers are lacking. We used magnetic resonance imaging and the brain boundary shift integral to quantify wholeโbrain atrophy rates over 1 year in early and premanifest HD subjects, and controls. Early HD subjects had statistically significantly (P = 0.007) increased (threefold higher) rates of wholeโbrain atrophy compared with controls. Higher atrophy rates were associated with longer CAG repeat length. MRIโbased measures of wholeโbrain atrophy may have potential as a measure of progression in HD. ยฉ 2009 Movement Disorder Society
๐ SIMILAR VOLUMES
## Abstract __Objectives__: To investigate the functionโstructure relationship of white matter within different stages of Huntington's disease (HD) using diffusion tensor imaging (DTI). __Experimental design__: From the TRACKโHD study, an early stage HD group and a premanifest gene carrier group (P