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Abstract Details

Using Functional Status to Aid Interpretation of Composite Unified Huntington’s Disease Rating Scale (cUHDRS) Scores in Patients with Huntington’s Disease (HD)
Movement Disorders
P6 - Poster Session 6 (12:00 PM-1:00 PM)
3-004

The cUHDRS is a novel scoring algorithm that provides a holistic and sensitive outcome assessment of cognitive, motor and functional impairment in early-to-moderate manifest HD, combining Total Functional Capacity (TFC), Total Motor Score, Symbol Digit Modalities Test and Stroop Word Reading measures into a single metric. To aid cUHDRS interpretation, it is important to understand how it relates to typical functional status.

To enhance understanding of cUHDRS scoring by linking to established measures of meaningful daily function and independence in individuals with early-to-moderate manifest 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 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, mean HD stage, Independence Scale (IS) score, mean Functional Assessment (FA) score and number of FA items achievable by ≥50% of individuals were calculated.

cUHDRS score groupings ranged between 2–18; only six individuals were cUHDRS=2, therefore this grouping was excluded from subsequent analyses, leaving a range of 3–18 (N=3,484). In the 14–18 score groupings, ≥50% achieved all 25 FA items and had a mean IS of 95. As cUHDRS scores declined, the number of FA items achievable and level of independence declined. In the cUHDRS=3 grouping, 12 FA items were achievable by ≥50%; with mean IS=65. A figure was created, incorporating these results, to aid interpretation of cUHDRS scores, by linking scores to measures of functional ability.

cUHDRS scores reflect differing levels of function in individuals with early-to-moderate manifest HD. The cUHDRS can better differentiate between individuals with Stage I 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.

Authors/Disclosures
Jeffrey D. Long
PRESENTER
Jeffrey Long has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Triplet. Jeffrey Long has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Vacinnex. Jeffrey Long has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Wave. Jeffrey Long has received personal compensation in the range of $0-$499 for serving as a Consultant for PTC. Jeffrey Long has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Jeffrey Long has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Jeffrey Long has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for uniQure. Jeffrey Long has received research support from NIH DSMB.
Dylan Trundell Dylan Trundell has received personal compensation for serving as an employee of Roche Products Ltd. Dylan Trundell has received stock or an ownership interest from Roche Products Ltd.
Giuseppe Palermo Giuseppe Palermo has received personal compensation for serving as an employee of F. Hoffman-La RocheLtd..
Blair Leavitt, MD (University of British Columbia) Dr. Leavitt has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Incisive Genetics. Dr. Leavitt has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Leavitt has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Leavitt has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for PTC Therapeutics. Dr. Leavitt has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Design TX. Dr. Leavitt has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ReMix Tx. Dr. Leavitt has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sintetica. Dr. Leavitt has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Triplet Tx. Dr. Leavitt has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Takeda. Dr. Leavitt has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Incisive Genetics. Dr. Leavitt has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Journal of Huntington's Disease. Dr. Leavitt has stock in Incisive Genetics. Dr. Leavitt has stock in SRNAlytics. The institution of Dr. Leavitt has received research support from CIHR. The institution of Dr. Leavitt has received research support from HSC. The institution of Dr. Leavitt has received research support from Brain Canada. The institution of Dr. Leavitt has received research support from NMIN. Dr. Leavitt has received intellectual property interests from a discovery or technology relating to health care.
Scott Schobel Scott Schobel has received personal compensation for serving as an employee of F. Hoffman-La Roche Ltd.
Sarah J. Tabrizi, MD, PhD Sarah J. Tabrizi, MD, PhD has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for F. Hoffmann-La Roche Ltd. Sarah J. Tabrizi, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech.