Muscle Structure, Function and Gait in dHMN

Plain-language summary

Distal Hereditary Motor Neuropathy (dHMN) is a rare inherited neuromuscular disorder. It is characterised by distal weakness. The condition usually manifests in the second decade of life and progresses slowly. Though patients usually have a normal lifespan it is a disabling condition and most eventually need aids to walk. In order to improve walking quality in patient with dHMN, research is needed to understand the impairments that lead to altered gait patterns, and to develop interventions to correct walking gait conservatively. In this proposed trial our goal is to explore the relationships between muscle structure, function and gait patterns for people with Distal Hereditary Motor Neuropathy. Over 12 months, muscle changes in dHMN are going to be observed in terms of structure and function using MRI, myometry and 3D motion analysis. In addition, the effect of a 16 weeks exercises program on muscle structure and function in dHMN is going to be measured by the same observational methods. To address walking gait directly in dHMN, gait patterns with and without wearing carbon fibre ankle foot orthoses (AFO)will be measured using 3D motion analysis.

Who can participate

What they're measuring

Trial details