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).

Method: We used Pearson product-moment correlation to examine the association between cUHDRS scores and various UHDRS and non-UHDRS clinical measures in 83 subjects with mild-moderate HD.  The clinical measures included the TFC, the TMS, the UHDRS Independence Scale (IS), SDMT, SWR, Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (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=.713), TMS (r=-.805), SDM (r=.857) and SWRT (r=.777).  For other cognitive measures, however, such as the MMSE (r=.510), MoCA (r=.543), CTiP Choice Reaction Time (r=-.483), and CTiP Semantic Search Reaction Time (r=-.608), correlations were less robust.  Correlations between the cUHDRS and another assessment of daily functioning, the UHDRS Independence Scale, were strong (r=.671). There was a strong correlation with balance as appraised by TBS (r=.627) but a poor correlation with behavior as assessed by the HD-BQ (r=-.173).

Conclusion: 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.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/how-well-does-the-composite-unified-huntingtons-disease-rating-scale-cuhdrs-reflect-disease-progression-in-huntingtons-disease-hd/