Abstract Details

Title How Well Does the Composite Unified Huntington’s Disease Rating Scale (cUHDRS) Reflect Disease Progression in Huntington’s disease (HD)?

Topic Movement Disorders

Presentation(s) Movement Disorders Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 041

Objective

To examine the usefulness of the cUHDRS in reflecting disease progression in a well-characterized group of mild to moderate HD subjects followed at one academic HD center.

Background

The cUHDRS is a composite endpoint developed to evaluate disease progression in early-to-moderate manifest HD. It is comprised of four assessments: the UHDRS Total Functional Capacity (TFC), the UHDRS Total Motor Score (TMS), Symbol Digit Modalities Test (SDMT) and Stroop Word Reading (SWR).

Design/Methods We used Pearson product-moment correlation to examine the association between cUHDRS scores and various UHDRS and non-UHDRS clinical measures in 84 subjects with mild-moderate HD. The clinical measures included: TFC, TMS, UHDRS Independence Scale (IS), SDMT, SWR, MMSE, MoCA, the Computerized Test of Information Processing (CTiP) which is comprised of three reaction time subtests that successively increase in task complexity, Total Body Sway (TBS) as captured by a computerized balance assessment device (BTrackS^TM), and total score on the Huntington’s Disease Behavioral Questionnaire (HD-BQ).

Results We found that, not surprisingly, there were strong-very strong correlations between the cUHDRS and assessments on which it is based, including the TFC (r=.769), TMS (r=-.830), SDM (r=.851) and SWRT (r=.807). For other cognitive measures, however, such as the MMSE (r=.614), MoCA (r=.603), CTiP Choice Reaction Time (r=-.517), and CTiP Semantic Search Reaction Time (r=-.590), correlations were less robust. Correlations between the cUHDRS and another assessment of daily functioning, the UHDRS Independence Scale, were strong (r=.735). There was a moderate correlation with balance as appraised by TBS (r=.627) but a poor correlation with behavior as assessed by the HD-BQ (r=-.226).

Conclusions

The cUHDRS appears to reflect clinical progression reasonably well, particularly with regard to measures on which it is based; however, it also correlates well with independent assessments, which provides support for its usefulness as a primary outcome measure in clinical trials of mild to moderate HD.

Authors/Disclosures
Jody Corey-Bloom, MD, PhD, F�鶹��ýӳ�� (UCSD Neurosciences) PRESENTER	Dr. Corey-Bloom has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UniQure. Dr. Corey-Bloom has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva Pharmaceuticaks. Dr. Corey-Bloom has received personal compensation in the range of $10,000-$49,999 for serving as a Co-Director, HD-Net with Huntington Study Group.
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Brenton A. Wright, MD	Dr. Wright has nothing to disclose.
Paul Gilbert, PhD (SDSU-UCSD)	Dr. Gilbert has nothing to disclose.

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