Motion sensor‐acquired reachable workspace correlates with patient‐reported upper extremity activities of daily living (ADL) function in facioscapulohumeral dystrophy

Abstract

Background This study examines the correlation, and clinical meaningfulness, between reachable workspace outcome and reported activities of daily living (ADL) function of individuals with facioscapulohumeral dystrophy (FSHD). Methods Twenty‐one FSHD subjects with various disease severity (clinical severity scores 1‐4) underwent reachable workspace evaluation and completed the Quality of Life in Neurological Disorders (NeuroQoL) upper extremity questionnaire. Spearman and receiver operator curve analyses were performed. Results Moderate correlation was found between NeuroQoL scores and total (ρ = 0.7609; P < .01), and upper‐quadrants relative surface areas (RSAs) (ρ = 0.6969; P < .01). Five specific items (ie, shirt on, shirt off, use spoon, pull on pants, pick‐up clothes) demonstrated even higher correlations with total (ρ = 0.8397; P < .01) and above shoulder (ρ = 0.8082; P < .01) RSAs. A total RSA cuffoff value of 0.70 would achieve 100% sensitivity and 94% specificity (area under the curve = 0.975). Conclusions Reachable workspace values identify when individuals have difficulties performing ADLs at home. This information improves patient monitoring, and clinical decision making by enabling more timely recommendations for medications, assistive devices, or considerations for clinical trial enrollments.

Reachable workspace analysis is a potential measurement for impairment of the upper extremity in neuralgic amyotrophy

“…This rigidity limits the lateral translation and internal rotation of the scapula, which are needed to position the humerus across the body, possibly resulting in a decrease in AROM for the affected arm to the contralateral side 27 . This may indicate that patients with facioscapulohumeral dystrophy have less rigidity and primarily demonstrate weakness of (peri)scapular muscles 7,16 . The patients in this study did show lower strength compared with healthy controls, but strength values were well over Medical Research Council score 3 for the arm‐ and scapular‐pivoting musculature, and should not have affected the capability to move the arm for the duration of the protocol.…”

“…The sample size was based on a power analysis for the primary outcome measure of the NA‐CONTROL study (ie, functional capacity of the upper extremity as measured with the Shoulder Rating Questionnaire‐‐‐Dutch language version [SRQ‐DLV]) 6 . Previous RWS studies in patients with neuromuscular disorders had similar or smaller sample sizes 7,10,12,16 . A detailed description of recruitment procedures and a full list of inclusion and exclusion criteria can be found in the design paper of the NA‐CONTROL study 6 …”

“…As an alternative to goniometry, a sensor‐based reachable workspace (RWS) measure to evaluate the proximal upper extremity AROM and arm reachability has been developed 8–11 . The RWS allows simultaneous tracking of multiple joints of the upper extremity in a series of protocolized movements, which correlates with activities of daily living 7 . The system shows sufficient accuracy and robustness in tracking upper extremity movement when compared with more complex and expensive motion capture systems 8,9 .…”

Outcome Measures in Facioscapulohumeral Muscular Dystrophy Clinical Trials

“…Furthermore, the quantitative MVICT of shoulder abduction and elbow flexion moderately correlates with RWS [ 75 ]. Based on a more recent investigation, RWS moderately to strongly correlates with daily life activities at home (especially pick-up clothes, shirt on, shirt off, use spoon, and pull on pants) [ 76 ]. Notably, the recent randomized, double-blind, placebo-controlled (phase 2b) trial of Losmapimod (an inhibitor of p38α/β mitogen-activated protein kinases) on 80 FSHD patients found small but significant improvements of up to 1.5% in RWS surface area after 48-week treatment with Losmapimod compared to placebo ( Table 2 ) [ 27 ].…”

Current Trends and Challenges in Pediatric Access to Sensorless and Sensor-Based Upper Limb Exoskeletons

“…Indeed, it is known that children with limited exploration ability are at higher risk of developing cognitive, social, and motor impairments [6]. Moreover, weakness or impairments at the upper limb decrease autonomy in most activities of daily living (ADL), such as eating, bathing, getting dressed, and playing [7,8].…”