The Effectiveness of Wearable Upper Limb Assistive Devices in Degenerative Neuromuscular Diseases: A Systematic Review and Meta-Analysis

Abstract

Background: This systematic review summarizes the current evidence about the effectiveness of wearable assistive technologies for upper limbs support during activities of daily living for individuals with neuromuscular diseases. Methods: Fourteen studies have been included in the meta-analysis, involving 184 participants. All included studies compared patients ability to perform functional tasks with and without assistive devices. Results: An overall effect size of 1.06 (95% CI = 0.76-1.36, p < 0.00001) was obtained, demonstrating that upper limbs assistive devices significantly improve the performance in activities of daily living in people with neuromuscular diseases. A significant interaction between studies evaluating functional improvement with externally-assessed outcome measures or self-perceived outcome measures has been detected. In particular, the effect size of the subgroup considering self-perceived scales was 1.38 (95% CI = 1.08-1.68), while the effect size of the other group was 0.77 (95% CI = 0.41-1.11), meaning that patients' perceived functional gain is often higher than the functional gain detectable through clinical scales. Conclusion: Overall, the quality of the evidence ranged from low to moderate, due to low number of studies and participants, limitations in the selection of participants and in the blindness of outcome assessors, and risk of publication bias. Significance: A large magnitude effect and a clear dose-response gradient were found, therefore, a strong recommendation, in favor of the use of assistive devices could be suggested.

Recommendations for studies on dynamic arm support devices in people with neuromuscular disorders: a scoping review with expert-based discussion

“…Our analysis shows that muscle activity and joint mobility are affected by load reduction, but their relation has not been investigated nor linked to ADL performance. Gandolla et al, deducted similar conclusions from their focus on activity and participation [34]. Therefore, we recommend to investigate the relation between the two ICF components in a biomechanical framework under various levels of load reduction during functional tasks to optimise effort reduction and mobility improvements during common ADLs.…”

“…The perceived benefits, which vary between users, can even decrease so much over time that the user decides to stop using the device completely. Similarly, a recent systematic review on the short-term benefits of wearable devices found that as the disability level changes the device benefits change as well [34].…”

“…Lastly, experts and literature agree that user satisfaction, such as perceived benefits and comfort, should be taken as guidance to evaluate the device effectiveness. Cruz et al, and Gandolla et al, also recently promoted the use of objective and subjective measures as both measures provide equally important evidence of the functional status of the user [25,34]. Therefore, device requirements should align with the needs and goals of the user and additionally aim to relieve symptoms of pain, stiffness, and fatigue.…”

Daily Life Benefits and Usage Characteristics of Dynamic Arm Supports in Subjects with Neuromuscular Disorders

“…However, they are also prone to bias, e.g., self-reported duration of DAS use was found to depend heavily on users’ expectations of, and reliance on, the device [ 6 , 9 ]. Similar bias was also present in studies evaluating the functional improvement where a patient’s perceived gain was higher than the gain detectable through clinical scales [ 22 ]. The low level of specificity and the bias in self-reports makes it difficult to distil the most important aspects of DAS usage [ 21 ].…”

Test-retest reliability of the Performance of Upper Limb (PUL) module for muscular dystrophy patients

“…In the last years, the PUL module has been widely applied in DMD clinical trials [ 12 , 17 – 20 ], but it has been rarely used in other dystrophies [ 21 ]. A previous study evaluated PUL 1.2 module in a group of 322 DMD boys and adults [ 10 ], showing that this scale is an excellent tool to assess both ambulant and non-ambulant DMD patients’ upper limb functionality.…”