Computerised Dynamic Posturography in Premanifest and Manifest individuals with Huntington\u2019s Disease

Alvaro Reyes,Mel Ziman,Jody Corey Bloom,Paul E Gilbert,Wei-Peng Teo,Danielle Bartlett,Luis D Medina,Travis Cruickshank,Pauline Zaenker,Roger W Simmons,Danielle Salomonczyk,Eva Pirogovsky-Turk

doi:10.1038/s41598-018-32924-y

Abstract

Evidence from small-scale studies indicates that impairments in postural stability are an early and disabling feature of Huntington’s disease (HD) and may be a useful clinical endpoint for disease modifying trials. Larger studies are needed to confirm these preliminary findings and the suitability of postural stability outcomes as clinical endpoints. Static and dynamic postural stability were evaluated in 54 premanifest HD, 36 manifest HD and 45 healthy individuals using the Sensory Organization Test (SOT) and Limits of Stability (LOS) test. Manifest HD displayed significantly lower scores on all SOT conditions and on the SOT composite score and had more falls than healthy and premanifest HD (p < 0.05). Premanifest and manifest HD demonstrated significantly lower endpoint excursion (p < 0.001), maximum excursion (p ≤ 0.001), and directional control (p ≤ 0.004) values than healthy individuals on the LOS test. Deficits in LOS were found to manifest on the left side of premanifest HD. Significant but low associations were observed between UHDRS-TMS, disease burden score, diagnostic confidence level, SOT conditions and SOT composite score. We confirm here that individuals with premanifest and manifest HD display significant impairments in static and dynamic postural stability. Dynamic posturography assessments should be considered as clinical endpoints for future disease modifying trials.

Highlights

Evidence from small-scale studies indicates that impairments in postural stability are an early and disabling feature of Huntington’s disease (HD) and may be a useful clinical endpoint for disease modifying trials
Significant impairments in static and dynamic postural stability and a greater number of falls were detected in individuals with premanifest and manifest HD compared to healthy age- and gender-matched controls
Previous work by our team has shown that individuals with manifest HD, but not premanifest HD, have significant impairments in postural control at their limits of stability4,12

Summary

Introduction

Evidence from small-scale studies indicates that impairments in postural stability are an early and disabling feature of Huntington’s disease (HD) and may be a useful clinical endpoint for disease modifying trials. Studies examining changes in postural stability in individuals with HD have typically used clinical tests and scales such as the Timed Up and Go Test, Functional Reach Test, Tinetti Mobility Test, mini Balance Evaluation System Test, Berg Balance Scale and Activities-specific Balance Confidence Scale. Studies examining changes in postural stability in individuals with HD have typically used clinical tests and scales such as the Timed Up and Go Test, Functional Reach Test, Tinetti Mobility Test, mini Balance Evaluation System Test, Berg Balance Scale and Activities-specific Balance Confidence Scale1,2,5–7 These studies have reported an increased risk of falls, reduced balance confidence, a widened base of support and impairments in postural transitioning and single- and double-legged stance in individuals with manifest HD1,6,7. These measures provide little insight into the pathological mechanisms underpinning changes in postural stability in individuals with HD1–4,8,9

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