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Background Evidence of best practice for long term physical activity Huntington’s disease (HD) is lacking, due in part, to inherent challenges in the delivery and evaluation of such life-style interventions. Aim To investigate the feasibility of a nested randomized controlled trial (RCT) of physical activity into Enroll-HD, an established HD cohort study. Methods We conducted a ‘Trial within a Cohort’ (TWiC) evaluation of an exercise intervention compared to usual activity in individuals with early-mid stage HD. All participants completed their usual Enroll-HD assessments and PACE-HD assessments, which included measures of fitness (predicted maximal oxygen uptake) and self-reported and quantitative measures of physical activity. We explored the use of propensity score weighting to compare the individuals in the intervention arm of the RCT to those in the cohort. Results Of the 274 participants screened, 204 met the inclusion criteria and of those, 54 (26.5%) declined to participate and 34 (16.7%) were not contactable. Recruitment targets were only narrowly missed; 59/60 (98.3%) for the cohort and 57/60 (93.5%) for the RCT. Retention rates at 12 months were ~ 85 in both groups. Percentage (%) data completeness for outcomes measures at baseline ranged from 42.3-100% and at 12 month follow up from 19.2–85.2 %. Imbalances in pretreatment confounders for the cohort and the RCT could not to be addressed via propensity score weighting likely due to small sample sizes. Conclusion A targeted recruitment strategy was instrumental in achieving target recruitment; retention at 12 months was excellent. Pre-specified criteria for minimum dataset data completion (both PACE-HD and linked Enroll-HD datasets) were met. The TWiC design, with linkage to Enroll-HD, is feasible for long-term physical activity evaluation in HD provided sample size requirements can be achieved. *PACE-HD & Enroll-HD site principal investigators Teresa Montojo. Neurology Department, Fundacion Jimenez Diaz, Madrid, Spain. Jesus Miguel Ruiz Idiago. Neuropsychiatry Unit, Hospital Mare de Deu de la Merce, Barcelona, Spain. Department of Psychiatry and Forensic Medicine, Universitat Autonoma de Barcelona, Spain. Julie Hershberg. University of Southern California, Division of Biokinesiology and Physical Therapy. Re+active physical therapy & wellness, Los Angeles, CA, USA. Yvette Bordelon. Department of Neurology, University of California, Los Angeles, CA, USA. Karen Marder, Columbia University Irving Medical Center, New York, NY, USA. Lori Quinn, Teachers College, Columbia University, New York, NY, USA. Ralf Reilmann. George-Huntington-Institute and Institute for Clinical Radiology, University of Munster, Munster, Germany. Kathrin Reetz. University Hospital Aachen, Germany. Bernhard Landwehrmeyer. University Hospital Ulm, Germany.

Background and aims Across the Huntington’s Disease (HD) communities, there has been some success in delivering exercise interventions in HD over the short term. In order to move the field forward we now need studies with longer term follow-up to elucidate the potential effect of exercise and physical activity on disease progression. The efficient use of existing data and consideration of patient preferences is critical for evaluation of non-drug interventions where recruitment and retention are challenging. Methods In PACE-HD we utilise a Trial-within-a-Cohort design involving six Enroll-HD sites across Europe and USA. This design involves longitudinal evaluation of physical fitness and activity in a cohort of people with manifest HD (n=120) and a nested randomized trial (RCT) at 3 sites (n=60) of 12-months exercise compared to usual care. The intervention incorporates a physical activity coaching program with goal setting, an associated workbook, and provision of exercise equipment and FitBit devices for self-monitoring of exercise. Assessments include fitness testing and the use of wearable technologies to capture and quantify dose (frequency, duration, intensity) of physical activity in a large HD cohort. Participants in the RCT allocated to usual activity and those from the observational cohort will provide reference data (n=90) from their annual Enroll-HD assessments that we will utilise to evaluate exercise effects in those randomized to supported exercise (n=30). Outcomes Should the design prove to be feasible, it will pave the way for robust evaluation of exercise in HD supported by the existing Enroll cohort.

Background Wearable technology can provide detailed information about physical activity (PA) in Huntington’s disease (HD). Most commercially available devices are however limited by the lack of disease specific validation and consideration of wear time. Aim To develop a methodology for accurate profiling of PA in HD. Methods We recruited 29 people with early stage HD (mean (SD) age 51.76 (10.27); 14 males) and 17 healthy controls (mean (SD) age 52.95 (10.76); 10 male) to a cross sectional multi-centre observational study. Participants used a wrist worn accelerometer for 7 days following the completion of a laboratory-based validation study. PA profiles (percentage time sedentary, low, moderate and high PA) were produced. Differences were assessed when accounting for wear time based on body temperature trends. Results HD mean (SD) device wear time over 7 days was 9783 mins (2192.84) (approx. 23.3 hours per day) and controls 7965 mins (1200.34) approx. 18.9 hours per day). When accounting for wear time HD participants were sedentary for 54% of the time and participated in light, moderate and vigorous PA for 27.2%, 17.4% and 0.43% of the time respectively. Control participants were sedentary for 61% of the time and participated in light, moderate and vigorous PA 26.6%, 11.2% and 1.2% of the time respectively. Conclusions Surprisingly HD participants were less sedentary than age matched controls. The impact of involuntary movements on the assessment of PA in HD needs to be explored. Critical to this is the importance of a wear time algorithm to identify true sedentary behaviour in comparison to non-wear.

Background This speaker presentation will interpret results of an individual patient data meta-analyses (IPD-MA) of a range of randomized controlled feasibility trials with the aim of exploring prospects for exercise and rehabilitation in Huntington’s Disease (HD). Methods Participants(n=121) in five randomised physical activity and/or exercise trials engaged in a combination of supervised and self-directed exercise and physical activity interventions with varied emphasis on aerobic, strength, endurance, flexibility and task training across interventions and duration ranged from eight to 16 weeks. The primary outcome in the combined dataset was the modified Unified Huntington’s Disease Rating Motor Score (mMS). There were a range of secondary outcomes. Primary pooled effect estimates were examined via a cross-classified linear mixed model. A random effects model was used to account for study heterogeneity. Results There was significant heterogeneity between the studies. Risk of bias was moderate in these open trials; however, the primary motor outcome was collected blind. A favourable motor outcome was associated with individual studies where supervision was an important component of the intervention. There were no effects on motor outcome in the pooled data. Of the pooled effects for secondary outcomes, self-reported physical activity was significantly improved. Conclusions Self-reported physical activity increased following participation in short duration supervised and self-directed exercise and physical activity interventions but this did not translate into motor benefits in HD. Longer duration intervention trials that incorporate supervised components meeting frequency, intensity, time and type (FITT) principles for exercise prescription are required. The importance of a defined intervention, appropriate supervision and related core outcome set (including patient reported outcomes) in HD cannot be understated.

Background Physical activity (PA) has great potential for symptom management and improved quality of life in Huntington’s disease (HD). There is a lack of underpinning theory informing existing complex PA interventions utilised in HD research. Qualitative exploration of PA in HD is critical to inform development of such theoretically informed interventions. Aim To explore how living with Huntington’s disease (HD) impacts on the experience of physical activity (PA) across the stages of the disease. Methods Eight focus groups were conducted across the UK. Participants included people with prodromal to late stage HD, caregivers (family members/formal), healthcare professionals. Data were analysed using a framework analysis method. An analytical coding framework was developed from the data and the self-regulation model (SRM) to assist analysis. Results Key themes include ‘the evolving representations of HD and physical activity’, ‘the varying social environment of the person with HD and the impact on physical activity’ and ‘achieving PA participation while coping with the nuances of HD’. The SRM facilitated understanding of PA experiences; self-regulation of activities becomes more collaborative with the caregiver as HD progresses. Conclusion A modified SRM which accounts for increased collaborative regulation of representations and PA participation as HD progresses over time is suggested. Social and familial context of individuals with HD is a key consideration for health care professionals supporting PA participation. Strategies such as using PA as a way of achieving control, and adjusting expectations can help people with HD continue to be active and adapt PA from high level to more functional activities with symptom progression. The findings provide a theoretical foundation to underpin development of a wide range of PA management and research interventions in HD and have been used in development of the currently trialled interventions (clinical trials.gov NCT03344601).