Cognitive processes of apathy in Huntington's Disease show high sensitivity to disease progression

Background Disease-modifying treatments for Huntington’s disease (HD) are entering clinical trials: there is a pressing need for objective outcome measures of disease progression. Our previous work showed an association between 2 novel, objective cognitive tasks and apathy - a core feature of disease progression in HD. Objective Evaluate the longitudinal validity and sensitivity of the novel Persistence and Maze tasks to assess their utility as clinical outcome measures in HD. Methods 83 participants positive for the HD gene and 54 controls performed a battery of established and novel tools, at baseline and 12 month follow up. Results The Maze task was found to be the most sensitive measure of change at 12 months, including the current gold-standard measure (the composite disease progression score). Conclusion The Maze task has potential as a novel outcome measure of disease progression in HD and may have utility in other major neurodegenerative diseases