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Tracking motor impairments in the progression of Huntington's disease

Long, Jeffery D., Paulsen, Jane S., Marder, Karen, Zhang, Ying, Kim, Ji-In, Mills, James A., PREDICT-HD Huntington’s Study Group, Rosser, Anne ORCID:, Price, Kathy and Hunt, Sarah 2014. Tracking motor impairments in the progression of Huntington's disease. Movement Disorders 29 (3) , pp. 311-319. 10.1002/mds.25657

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The Unified Huntington's Disease Rating Scale is used to characterize motor impairments and establish motor diagnosis. Little is known about the timing of diagnostic confidence level categories and the trajectory of motor impairments during the prodromal phase. Goals of this study were to estimate the timing of categories, model the prodromal trajectory of motor impairments, estimate the rate of motor impairment change by category, and provide required sample size estimates for a test of efficacy in clinical trials. In total, 1010 gene-expanded participants from the Neurobiological Predictors of Huntington's Disease (PREDICT-HD) trial were analyzed. Accelerated failure time models were used to predict the timing of categories. Linear mixed effects regression was used to model the longitudinal motor trajectories. Age and length of gene expansion were incorporated into all models. The timing of categories varied significantly by gene expansion, with faster progression associated with greater expansion. For the median expansion, the third diagnostic confidence level category was estimated to have a first occurrence 1.5 years before diagnosis, and the second and first categories were estimated to occur 6.75 years and 19.75 years before diagnosis, respectively. Motor impairments displayed a nonlinear prodromal course. The motor impairment rate of change increased as the diagnostic confidence level increased, with added acceleration for higher progression scores. Motor items can detect changes in motor impairments before diagnosis. Given a sufficiently high progression score, there is evidence that the diagnostic confidence level can be used for prodromal staging. Implications for Huntington's disease research and the planning of clinical trials of efficacy are discussed.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Subjects: R Medicine > R Medicine (General)
Additional Information: Anne Rosser, Kathy Price and Sarah Hunt are Researchers of the PREDICT-HD Huntington’s Study Group
Publisher: John Wiley & Sons
ISSN: 0885-3185
Date of Acceptance: 31 July 2013
Last Modified: 31 Oct 2022 09:02

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