Farrell Hayden J, Andrews Sophie C, Ryan Nicholas P, Davis Marie-Claire, Gordon Stephanie, Stout Julie C, Fisher Fiona
School of Psychological Sciences, and, Institute of Cognitive and Clinical Neurosciences, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, VIC, Australia.
Calvary Health Care Bethlehem, Statewide Progressive Neurological Disease Service, Caulfield South, VIC, Australia.
J Huntingtons Dis. 2019;8(1):87-95. doi: 10.3233/JHD-180323.
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.
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.
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 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.
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.
DriveSafe DriveAware（DSDA）已被验证为一种用于预测临床人群道路驾驶表现的越野筛查工具，但其在亨廷顿舞蹈病（HD）患者中的效用尚不清楚。
我们的目的是通过证明DSDA分数对HD进展的敏感性，并探索DSDA表现与对驾驶至关重要且在HD患者中受损的认知功能之间的关联，来评估DSDA在HD患者中的效用。
我们对26名有症状和无症状的HD参与者使用了DSDA的iPad应用程序版本。通过运动障碍、疾病负担和功能能力的测量来评估疾病进展。使用标准化神经心理学测试来评估包括注意力、处理速度、规划和视觉感知在内的多个领域的认知功能。
结果强调了DSDA对HD进展和认知障碍的敏感性；也就是说，DSDA表现较差与HD严重程度较高以及在注意力、处理速度和规划领域的认知能力较差相关。然而，我们发现一部分HD参与者根据DSDA预测可以通过道路测试，但在多项认知测试中得分处于受损范围。这些参与者的得分往往更接近DSDA用于分类通过/未通过结果的临界分数。
我们的研究结果表明DSDA在HD人群中具有应用潜力，然而，预计在道路驾驶评估中“通过”的人群在认知表现上存在显著差异，这表明该筛查工具需要进一步开发以用于HD驾驶员。
