Brain compensation during visuospatial working memory in premanifest Huntington's disease

Abstract

Huntington's disease (HD) is a progressive autosomal dominant disorder characterised by impairment of movement, cognitive decline and psychiatric impairment. During the premanifest stages of HD (pre-HD), there are notable structural and functional brain changes detectable many decades prior to clinical diagnosis. Although caudate atrophy is one of the earliest and most prominent sites of neuropathology, there is also evidence of cortical atrophy, compromised white and grey matter integrity, cortical hypometabolism, and aberrant task-related functional magnetic resonance imaging (fMRI) activity. Pre-HD individuals typically show increased task-related fMRI activity suggested as a strategy to ..