Longitudinal Study of Upper Extremity Reachable Workspace in Fascioscapulohumeral Muscular Dystrophy

Neuromuscul Disord, 2019 · DOI: 10.1016/j.nmd.2019.05.006 · Published: July 1, 2019

Neurology Rehabilitation Research Methodology & Design

Simple Explanation

This study examines how the reachable workspace, a measure of arm reach using motion sensors, changes over time in people with FSHD. The study found that reachable workspace declines over time, especially in reaching above the shoulder. Using wrist weights made the decline in reachable workspace more noticeable. The reachable workspace measurement can distinguish between mildly, moderately, and severely affected individuals with FSHD.

Study Duration

5 Years

Participants

18 subjects with FSHD (7 women, 11 men)

Evidence Level

Level III, Longitudinal Study

Key Findings

1
Reachable workspace declined −1.63%/year in total (p=0.144), with most decline in above-the-shoulder quadrants.
2
Reachable workspace declined more with 500g wrist weights: −1.82%/year (p=0.039).
3
The reachable workspace outcome distinguished subgroups in FSHD: mildly-, severely-, and moderately-affected.

Research Summary

This study tracked changes in reachable workspace upwards for 5-years in 18 FSHD subjects. Reachable workspace declined more significantly if the subjects were challenged with 500g wrist weights The study demonstrates utility for measuring declines in upper quadrant reachability, and provides enrichment/stratification of FSHD populations most likely to show treatment effects in clinical trials.

Practical Implications

Clinical Trial Enrichment

Reachable workspace can identify FSHD subgroups most likely to show treatment effects.

Outcome Measure Sensitivity

Reachable workspace is more sensitive than existing ordinal measures for tracking upper extremity decline.

Intervention Design

Challenging patients with wrist weights enhances the detection of subtle weaknesses and decline rates.

Study Limitations

1
Small sample size of 18 individuals from a regional neuromuscular clinic.
2
Limited number of patients with specific baseline total RSA values between 0.7 to 0.8 and above.
3
Reachable workspace outcome measure is limited to assessment of upper extremity proximal range of motion.

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