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

PURPOSE: Neuromuscular disorders are characterised by muscle weakness that limits upper extremity mobility, but can be alleviated with dynamic arm support devices. Current research highlights the importance and difficulties of evidence-based recommendations for device development. We aim to provide research recommendations primarily concerning upper extremity body functions, and secondarily activity and participation, environmental and personal factors. METHODS: Evidence was synthesised from literature, ongoing studies, and expert opinions and tabulated within a framework based on a combination of the International Classification of Functioning, Disability and Health (ICF) model and contextual constructs. RESULTS: Current literature mostly investigated the motor capacity of muscle function, joint mobility, and upper body functionality, and a few studies also addressed the impact on activity and participation. In addition, experts considered knowledge on device utilisation in the daily environment and characterising the beneficiaries better as important. Knowledge gaps showed that ICF model components and contextual constructs should be better integrated and more actively included in future research. CONCLUSIONS: It is recommended to, first, integrate multiple ICF model components and contextual constructs within one study design. Second, include the influence of environmental and personal factors when developing and deploying a device. Third, include short-term and long-term measurements to monitor adaptations over time. Finally, include user satisfaction as guidance to evaluate the device effectiveness.IMPLICATIONS ON REHABILITATIONSynthesized evidence will support future research and development of dynamic arm supports.Tabulated evidence stresses the importance of integrating ICF model components and contextual constructs to fill the knowledge gaps.Presented knowledge gaps and proposed steps guide the set up of future studies on dynamic arm supports.

A gait paradigm reveals different patterns of abnormal cerebellar motor learning in primary focal dystonias.

Accumulating evidence points to a role of the cerebellum in the pathophysiology of primary dystonia. The aim of this study was to investigate whether the abnormalities of cerebellar motor learning in primary dystonia are solely detectable in more pure forms of cerebellum-dependent associative motor learning paradigms, or whether these are also present in other motor learning paradigms that rely heavily on the cerebellum but in addition require a more widespread sensorimotor network. Twenty-six patients with various forms of focal dystonia and 10 age-matched healthy controls participated in a motor learning paradigm on a split-belt treadmill. By using reflective markers, three-dimensional kinematics were recorded using a 6-camera motion analysis system. Adaptation walking parameters were analyzed offline, comparing the different dystonia groups and healthy controls. Patients with blepharospasm and writer's cramp were significantly impaired on various adaptation walking parameters. Whereas results of cervical dystonia patients did not differ from healthy controls in terms of adaptation walking parameters, differences in parameters of normal gait were found. We have here demonstrated abnormal sensorimotor adaptation with the split-belt paradigm in patients with blepharospasm and writer's cramp. This reinforces the current concept of cerebellar dysfunction in primary dystonia, and that this extends beyond more pure forms of cerebellum-dependent associative motor learning paradigms. However, the finding of normal adaptation in cervical dystonia patients indicates that the pattern of cerebellar dysfunction may be slightly different for the various forms of primary focal dystonia, suggesting that actual cerebellar pathology may not be a primary driving force in dystonia.