Work

A pilot study using actigraphy to examine activity performance and its relationship with activity capacity in adults with facioscapulohumeral muscular dystrophy

Year: 2025

Type: article

Abstract: BackgroundWeakness associated with facioscapulohumeral muscular dystrophy (FSHD) impacts daily activities. Impact is often measured using standardized clinical assessments, documenting activity capacity, and only captures a snapshot of function. Wearable sensors may enable assessments of real-world activity performance.ObjectiveTo examine activity performance (actigraphy) and its relationship with capacity (clinical measures of strength and function) in adults with FSHD.MethodsRemote assessments were piloted in a subgroup from Motor Outcomes to Validate Evaluations in FSHD. Participants wore waist-worn activity monitors for 7 days. Activity metrics included moderate-to-vigorous intensity physical activity (MVPA), time in activity levels, and step counts. Descriptive statistics summarized activity, Mann Whitney U tests compared groups, and correlation analyses examined relationships between activity performance and capacity.ResultsThirteen subjects wore the device for a median of 7 days. Most time was sedentary, with minimal vigorous activity. Participants spent a median of 23.1 min daily (IQR: 23.8) and 161.7 min weekly (IQR: 157.9) in MVPA. Median daily step count was 4245 (IQR: 2892), with a median maximum step count of 20 (IQR: 7) within a 10-second epoch. Correlations were found between total MVPA and 10mw/r (ρ=-0.720, p = 0.006) and TUG-comfortable (ρ=-0.720, p = 0.006), and between maximum step count and several functional measures.ConclusionThis pilot study provides insights into activity performance and its relationship with capacity in a small cohort of adults with FSHD. Total MVPA and maximum step count appear most informative for evaluating activity performance; larger studies are needed to confirm findings and assess psychometric properties of these metrics.

Source: Journal of Neuromuscular Diseases

DOI: 10.1177/22143602251389076