Effects of multidisciplinary therapy on physical function in Huntington's disease.

OBJECTIVE: The primary objective of this trial was to evaluate the effects of outpatient multidisciplinary therapy, compared to usual care, on measures of physical function and muscle strength in patients with manifest Huntington's disease (HD). METHODS: Twenty-two patients with clinically verified HD were randomized to receive 36 weeks of outpatient multidisciplinary therapy or usual care. Outpatient multidisciplinary therapy comprised 9 months of supervised exercise, cognitive therapy and self-directed home-based exercise. Usual care consisted of standard medical care. Patients were assessed at 0 and 36 weeks by blinded assessors. The primary outcome was changed in mobility as measured by the 10-m Timed Walk Test. Secondary outcome measures included changes in manual dexterity (Timed Nut and Bolt Test), balance (Berg Balance Scale), cardiorespiratory endurance (6-Minute Walk Test) and upper and lower extremity muscle strength (isokinetic and isometric muscle strength and 10 Repetition Sit-to-Stand Tests). RESULTS: Patients receiving outpatient multidisciplinary therapy demonstrated significantly enhanced manual dexterity (P < 0.05) and lower extremity muscle strength (P < 0.05) than patients receiving usual care. No significant differences in mobility, balance, cardiorespiratory endurance and upper extremity strength outcomes were observed between groups after the intervention period. There were no adverse events associated with multidisciplinary therapy. CONCLUSION: Our findings suggest that outpatient multidisciplinary therapy has positive effects on manual dexterity and muscle strength, but no meaningful effects on mobility, balance, cardiorespiratory endurance and upper extremity muscle strength in patients with HD. Larger randomized controlled trials are needed to confirm these preliminary findings.

Torque variability of the plantar flexors in people with Huntington's disease.

BACKGROUND: Torque steadiness can be impaired in people with Huntington's disease (HD) and worsen with disease advancement. However, existing studies have several methodological oversights. Studies have used absolute torque targets, which do not account for differences in maximal torque capacity between people. Furthermore, despite its known influence on torque steadiness, previous studies in HD have not controlled for visual feedback. This study evaluated torque variability at relative intensities with and without visual feedback between people with prodromal HD and healthy controls. METHODS: Twenty-four people with prodromal HD and twenty-seven age- and sex-matched healthy controls were recruited for this study. Torque variability was evaluated, with and without visual feedback, in the right plantar flexors at 10% and 30% of each participant's maximum voluntary isometric contraction (MVIC). Measures of disease burden included the CAG age product, diagnostic confidence level and Unified Huntington's Disease Rating Scale - Total Motor Score. RESULTS: Significant differences in torque variability were observed, though not in overall MVIC, between people with prodromal HD and healthy controls. Significantly higher torque fluctuations were observed for both groups when visual feedback was removed. No associations were observed between torque variability and disease burden in people with prodromal HD. Torque variability measurements showed higher reliability in healthy controls. CONCLUSIONS: People with prodromal HD exhibited greater torque variability than healthy controls. Torque variability worsened for both groups when visual feedback was removed. These findings support further investigation into the utilisation of torque variability measurements as markers of disease progression in people with prodromal HD.