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Observational Study
. 2017 Dec 12;89(24):2495-2502.
doi: 10.1212/WNL.0000000000004743. Epub 2017 Nov 15.

Motor, cognitive, and functional declines contribute to a single progressive factor in early HD

Scott A Schobel  1 , Giuseppe Palermo  2 , Peggy Auinger  2 , Jeffrey D Long  2 , Shiyang Ma  2 , Omar S Khwaja  2 , Dylan Trundell  2 , Merit Cudkowicz  2 , Steven Hersch  2 , Cristina Sampaio  2 , E Ray Dorsey  2 , Blair R Leavitt  2 , Karl D Kieburtz  2 , Jeffrey J Sevigny  2 , Douglas R Langbehn  2 , Sarah J Tabrizi  2 ; TRACK-HD, COHORT, CARE-HD, and 2CARE Huntington Study Group Investigators
Collaborators, Affiliations
Observational Study

Motor, cognitive, and functional declines contribute to a single progressive factor in early HD

Scott A Schobel et al. Neurology. .

Abstract

Objective: To identify an improved measure of clinical progression in early Huntington disease (HD) using data from prospective observational cohort studies and placebo group data from randomized double-blind clinical trials.

Methods: We studied Unified Huntington Disease Rating Scale (UHDRS) and non-UHDRS clinical measures and brain measures of progressive atrophy in 1,668 individuals with early HD followed up prospectively for up to 30 to 36 months of longitudinal clinical follow-up.

Results: The results demonstrated that a composite measure of motor, cognitive, and global functional decline best characterized clinical progression and was most strongly associated with brain measures of progressive corticostriatal atrophy.

Conclusions: Use of a composite motor, cognitive, and global functional clinical outcome measure in HD provides an improved measure of clinical progression more related to measures of progressive brain atrophy and provides an opportunity for enhanced clinical trial efficiency relative to currently used individual motor, cognitive, and functional outcome measures.

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Figures

Figure 1
Figure 1. Longitudinal S/N ratio of UHDRS and non-UHDRS clinical measures in early HD (stages I and II at baseline) across the motor, quantitative motor, cognitive, behavior, and global functional domains in the TRACK-HD sample
HD = Huntington disease; PBA = Problem Behaviors Assessment; SDMT = Symbol Digit Modality Test; S/N = single-to-noise; SWR = Stroop Word Reading Test; TFC = Total Functional Capacity; TMS = Total Motor Score; UHDRS = Unified Huntington Disease Rating Scale.
Figure 2
Figure 2. Longitudinal S/N ratio of individual UHDRS clinical measures (TFC, TMS, SDMT, SWR) and the cUHDRS across the 4 studies and time points
Care HD = Co-Enzyme Q10 and Remacemide: Evaluation in HD; Cohort = Cooperative Huntington's Disease Observational Research Trial; cUHDRS = composite Unified Huntington Disease Rating Scale; SDMT = Symbol Digit Modality Test; S/N = single-to-noise; SWR = Stroop Word Reading Test; TFC = Total Functional Capacity; TMS = Total Motor Score; 2Care = Coenzyme Q10 (CoQ) in Huntington Disease; UHDRS = Unified Huntington Disease Rating Scale.
Figure 3
Figure 3. Comparative baseline and longitudinal association of individual UHDRS clinical measures and the cUHDRS with measures of progressive corticostriatal atrophy in TRACK-HD
Pearson correlation coefficient 2-sided p values: *p < 0.05, **p < 0.01, ***p < 0.001. cUHDRS = composite Unified Huntington Disease Rating Scale; SDMT = Symbol Digit Modality Test; TFC = Total Functional Capacity; TMS = Total Motor Score; UHDRS = Unified Huntington Disease Rating Scale.
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References

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