Objective: Explore the cognitive profile of patients with Huntington’s Disease (HD) using a digital cognitive battery

Background: Huntington’s Disease (HD) is a relentlessly progressive neurodegenerative disorder typically characterized by cognitive, psychiatric, oculomotor, chorea and gait abnormalities.  Traditional measures of disease impact and change rely on history, neurological examination and incorporation of the United Huntington’s Disease Rating Scale (UHDRS). Incorporating examiner independent objective multi-dimensional quantitative measures of people with HD (PwHD) performance as they relate to patient perception might enhance appreciation of real world value of disease impact along a continuum. This approach might provide unique insight to proactively identify opportunities to both improve outcomes and intervene to reduce adverse outcomes that might not be readily appreciated by clinical examination. The impact of varied degrees and combinations of cognitive impairment in PwHD as they relate to quality of life may not be easily identified in routine care but earlier identification of such impact might provide an opportunity to reduce adverse outcomes.

Method: Retrospective review of data collected through routine care of PwHD that were evaluated by multidimensional computerized cognitive testing. Cognitive domains collected were: Memory (MEM), Executive Function (EF), Visual Spatial (VS), Information Processing (IP), Verbal Function (VF), Attention (ATT), Motor Skills (MS), and a Global Cognitive Summary Score (GCS).

Results: 34 PwHD, average age 50+/- 12. The mean scores by each cognitive domain are: GCS = 74.3, MEM= 70.1, EF = 73.6, VS = 89.0, IP = 87.0, VF = 71.2, ATT = 67.4, and MS = 78.3. 73.5% of the PwHD tested as cognitively impaired in the aggregate global score. The average number of cognitive domains impaired was found to be 3.44.

Conclusion: Physical symptoms of HD are widely discussed and recognized. According to our analysis, the cognitive impact of Huntington’s Disease is apparent and severe as well. According to the objective computerized cognitive battery, PwHD on average have over 3 domains of cognition impaired. Factoring in this impairment is crucial to bettering patient care.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/cognition-in-huntingtons-disease/