Evaluation of the cognitive-motor performance of adults with Duchenne Muscular Dystrophy in a hand-related task

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.

Characterization of Forearm Muscle Activation in Duchenne Muscular Dystrophy via High-Density Electromyography: A Case Study on the Implications for Myoelectric Control

“…This may provide another indication (together with variability in maximum activation) of how the progress of the disease affects motor control, since DP1 is the least affected participant. The decrease in dimensionality may be partially attributed to the increased level of co-contractions between agonist and/or antagonist muscle groups that we observed in the DMD participants when performing the tasks and further supported by a recent hand motor performance study in people with DMD (48). Co-contractions may be elicited by the effort of the participants to stabilize their wrist during the tasks, but further work is needed to explore this hypothesis.…”

Motor Compensation Strategies for Reduced Upper Limb Function Among Individuals With Duchenne Muscular Dystrophy

“…Effective rehabilitation of the upper limbs can improve hand functioning among people with DMD; but, to best achieve this goal, the patients’ qualitative motor performances must be understood (Nizamis et al., 2020). In clinical practice, these data provide relevant information about motor behavior of patients with DMD.…”

Dp71 and intellectual disability in Indonesian patients with Duchenne muscular dystrophy

“…Duchenne muscular dystrophy (DMD) is a severe neuromuscular disorder that affects skeletal muscles in 1:5,000 births [ 1 , 2 ]. DMD is an X-linked recessive progressive muscular disease due to mutations in the DMD gene, which causes the absence or damage of dystrophin [ 2 , 3 ]. Dystrophin serves as a link between the internal cytoskeleton and extracellular matrix proteins, which stabilize muscle contractions.…”