234  Using functional status to aid interpretation of cUHDRS scores in patients with Huntington’s disease

Abstract

The composite Unified Huntington’s Disease Rating Scale (cUHDRS) is a scoring algorithm that combines Total Functional Capacity (TFC), Total Motor Score, Symbol Digit Modalities Test and Stroop Word Reading measures. Our aim was to enhance understanding of cUHDRS scoring by linking to established measures of meaningful daily function and independence in individuals with early-to-moderate-manifest Hunting- ton’s disease (HD).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), cUHDRS score ranges were calculated. Patients were divided into groups around each integer score; 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 achieved were calculated.cUHDRS score groupings ranged 3–18 (N=3,484). For patients in the 14–18 cUHDRS score groupings, ≥50% achieved 25 FA items and had a mean IS=95. For patients in the lowest cUHDRS score group, cUHDRS=3, only 12 FA items were achievable by ≥50% and mean IS=65.cUHDRS scores reflect the differing levels of function in individuals with early-to-moderate-manifest HD. The cUHDRS can better differentiate between individuals with Stage 1 HD than commonly used measures of function.rachel.blair@roche.com