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Journal Article

Citation

Farrell HJ, Andrews SC, Ryan NP, Davis MC, Gordon S, Stout JC, Fisher F. J. Huntingtons Dis. 2019; 8(1): 87-95.

Affiliation

Calvary Health Care Bethlehem, Statewide Progressive Neurological Disease Service, Caulfield South, VIC, Australia.

Copyright

(Copyright © 2019, IOS Press)

DOI

10.3233/JHD-180323

PMID

30776017

Abstract

BACKGROUND: DriveSafe DriveAware (DSDA) has been validated as an off-road screening tool for predicting on-road driving performance in clinical populations, but its utility in people with Huntington's disease (HD) is unknown.

OBJECTIVE: Our aim was to evaluate the utility of DSDA in people with HD by demonstrating sensitivity of DSDA scores to HD progression and exploring associations between DSDA performance and cognitive functions that are essential to driving and impaired in people with HD.

METHODS: We administered the iPad application version of DSDA to 26 pre-symptomatic and symptomatic participants with HD. Disease progression was assessed via measures of motor impairment, disease burden and functional capacity. Standardised neuropsychological tests were used to assess cognitive function across several domains including attention, processing speed, planning, and visuoperception.

RESULTS: Results underscore the sensitivity of DSDA to HD progression and cognitive impairment; that is, poorer DSDA performance was associated with greater HD severity and poorer cognitive ability across the domains of attention, processing speed, and planning. Nevertheless, we identified a proportion of participants with HD who were predicted to pass on-road testing based on DSDA, but scored in the impaired range on multiple cognitive tests. These participants tended to score closer to the cut-off score used by DSDA to categorise pass/fail outcomes.

CONCLUSIONS: Our findings demonstrate potential for use of DSDA in the HD population, however, significant variability in cognitive performance among those predicted to 'pass' on-road driving assessment suggests the screening tool requires further development for use with HD drivers.


Language: en

Keywords

Huntington’s disease; automobile driving; cognitive dysfunction; disease progression

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