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Optimising mobility outcome measures in Huntington's disease
Monica Busse1, Lori Quinn1, Hanan Khalil2
1School of Healthcare Sciences, Cardiff University, Cardiff, UK.
Insights
This study optimized mobility measures for Huntington's disease (HD). Shortened Berg Balance Scale (BBS) and Tinetti Mobility Test (TMT) effectively assess unique HD gait and balance impairments, aiding future clinical trials.
Area of Science:
- Neurology
- Movement Disorders
- Clinical Assessment
Background:
- Existing mobility measures for Huntington's disease (HD) were often developed for other neurological conditions.
- This limits their specificity and effectiveness in capturing HD-related functional impairments.
Purpose of the Study:
- To evaluate individual item performance of common mobility scales in HD.
- To optimize these scales for better application in Huntington's disease populations.
Main Methods:
- Utilized data from 75 individuals with HD (pre-manifest and manifest).
- Assessed participants using multiple mobility tests including the Six-Minute Walk Test, Timed "Up & Go" Test (TUG), Berg Balance Scale (BBS), and Tinetti Mobility Test (TMT).
- Employed regression analysis and item response theory for scale shortening and psychometric evaluation.
Main Results:
- The Timed "Up & Go" Test (TUG), Berg Balance Scale (BBS), and Tinetti Mobility Test (TMT) demonstrated good disease-specific mobility measurement.
- The Physical Performance Test (PPT) effectively measured functional performance.
- Shortened versions of the BBS and TMT were developed, showing reliability and ability to discriminate between manifest and pre-manifest HD.
Conclusions:
- The Physical Performance Test (PPT) is suitable for general function assessment in HD.
- Shortened BBS and TMT scales accurately capture unique gait and balance deficits in HD.
- These optimized scales, along with TUG, are valuable for future Huntington's disease clinical trials.
Background:
Many of the performance-based mobility measures that are currently used in Huntington's disease (HD) were developed for assessment in other neurological conditions such as stroke.
Objective:
We aimed to assess the individual item-response of commonly used performance-based mobility measures, with a view to optimizing the scales for specific application in Huntington's Disease (HD).
Method:
Data from a larger multicentre, observational study were used. Seventy-five people with HD (11 pre-manifest & 64 manifest) were assessed on the Six-Minute Walk Test, 10-Meter Walk Test, Timed "Up & Go" Test (TUG), Berg Balance Scale (BBS), Physical Performance Test (PPT), Four Square Step Test, and Tinetti Mobility Test (TMT). The Unified Huntington's Disease Rating Scale (UHDRS) Total Motor Score, Functional Assessment Scale and Total Functional Capacity scores were recorded, alongside cognitive measures. Standard regression analysis was used to assess predictive validity. Individual item responses were investigated using a sequence of approaches to allow for gradual removal of items and the subsequent creation of shortened versions. Psychometric properties (reliability and discriminant ability) of the shortened scales were assessed.
Results:
TUG (β 0.46, CI 0.20-3.47), BBS (β -0.35, CI -2.10-0.14), and TMT (β -0.45, CI -3.14-0.64) were good disease-specific mobility measures. PPT was the best measure of functional performance (β 0.42, CI 0.00-0.43 for TFC & β 0.57 CI 0.15-0.81 for FAS). Shortened versions of BBS and TMT were developed based on item analysis. The resultant BBS and TMT shortened scales were reliable for use in manifest HD. ROC analysis showed that shortened scales were able to discriminate between manifest and pre-manifest disease states.
Discussion:
Our data suggests that the PPT is appropriate as a general measure of function in individuals with HD, and we have identified shortened versions of the BBS and TMT that measure the unique gait and balance impairments in HD. These scales, alongside the TUG, may therefore be important measures to consider in future clinical trials.

