Abstract

Duchenne muscular Dystrophy (DMD) is a progressive degenerative muscle disease, affecting, among others, the upper extremities. Effective hand rehabilitation can improve the hand function of people with DMD. To reach this goal, we first need to gain more insight into the hand cognitive-motor performance of people with DMD. This is the first study employing a systematic analysis on multi-finger, cognitive-motor performance of people with DMD. For this purpose, we propose an active dynamic visuo-motor task. The task employed six visual stimuli, a subset of which was activated at each trial. The stimuli were activated with a frequency of 1, 2, 3 and 4 Hz. Eight healthy participants and three participants with DMD performed the task. Additionally, the healthy participants performed seven sessions, and we assessed the training effects. Task-related cognitive-motor performance was evaluated using information transfer rate (ITR) and perceived workload. Regarding ITR, healthy participants performed significantly better than DMD participants; however, this was more evident for trials involving more than three fingers. Workload showed no difference between the healthy and the DMD groups. Healthy participants significantly improved their performance during training. Our results suggest that hand rehabilitation of people with DMD should consider multi-finger dynamic training. However, additional research with more people with DMD is needed for further generalization of our conclusions.

Highlights

  • Duchenne muscular dystrophy (DMD) is an X chromosome-linked recessive neuromuscular disease, affecting mainly males

  • The provided information (PI) per trial refers to the amount of bits/sec that we provide the participant via the visual stimuli

  • For each trial and each DMD participant, we considered the null hypothesis that the information transfer rate (ITR) of the DMD participant was an observation from the ITRs of the healthy population

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Summary

Introduction

Duchenne muscular dystrophy (DMD) is an X chromosome-linked recessive neuromuscular disease, affecting mainly males. It is diagnosed in childhood, affecting approximately 1:5000 births [1]. In 2013, the population of people with DMD in The Netherlands was 420 [2]. Duchenne is caused by mutations in the dystrophin gene that encodes the protein dystrophin, causing its absence or defect [3]. People with DMD suffer from progressive muscle weakness.

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