Session Information
Date: Monday, September 23, 2019
Session Title: Huntington’s Disease
Session Time: 1:45pm-3:15pm
Location: Agora 3 West, Level 3
Objective: To enhance understanding of the composite Unified Huntington’s Disease Rating Scale (cUHDRS) scoring by linking to established measures of meaningful daily function and independence in individuals with early-to-moderate-manifest Huntington’s disease (HD).
Background: The cUHDRS is a novel scoring algorithm that provides a relatively holistic and sensitive outcome assessment of cognitive, motor and functional impairment in early-to-moderate-manifest HD. It combines the Total Functional Capacity (TFC), Total Motor Score, Symbol Digit Modalities Test and Stroop Word Reading measures into a single metric. To aid score interpretation, it is important to understand what this score means in terms of typical functional status.
Method: Data from Enroll-HD were evaluated. For patients meeting the reference population for the cUHDRS (manifest HD, TFC 5–13, ≥20 years; N=3,490), the cUHDRS score range was calculated. Patients were divided into groups around each integer score (e.g., for cUHDRS=11: 10.5≤cUHDRS score<11.5). For each grouping, the mean HD stage, Independence Scale (IS) score, mean Functional Assessment (FA) score and number of FA items that ≥50% of individuals can achieve were calculated.
Results: In this population, cUHDRS score groupings ranged 2–18; however, only six individuals were cUHDRS=2, and so this grouping was excluded from subsequent analyses, leaving a range of 3–18 (N=3,484). For those patients in the 14–18 cUHDRS score groupings, ≥50% were able to achieve all 25 FA items and had a mean IS of 95. As cUHDRS scores declined, the number of FA items achievable and the level of independence declined. For patients in the lowest cUHDRS score group, cUHDRS=3 points, only 12 FA items were achievable by ≥50% and the mean IS=65. A figure was created, incorporating these results, to provide a simple guide to aid interpretation of cUHDRS scores, by linking cUHDRS scores to the measures of functional ability.
Conclusion: cUHDRS scores reflect the differing levels of function in individuals with early-to-moderate-manifest HD. Furthermore, the cUHDRS can better differentiate between individuals with Stage 1 HD than commonly used measures of function. The visual aid described here can be used to relate cUHDRS score to functional status in patients with HD.
To cite this abstract in AMA style:
D. Trundell, G. Palermo, J. Long, B. Leavitt, S. Schobel, S. Tabrizi. Using functional status to aid interpretation of cUHDRS scores in patients with Huntington’s disease [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/using-functional-status-to-aid-interpretation-of-cuhdrs-scores-in-patients-with-huntingtons-disease/. Accessed December 9, 2024.« Back to 2019 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/using-functional-status-to-aid-interpretation-of-cuhdrs-scores-in-patients-with-huntingtons-disease/