Home‐based multi‐sensory and proximal strengthening program to improve balance in Charcot–Marie–Tooth disease Type 1A: A proof of concept study

Author:

Dudziec Magdalena M.123,Lee Laurence E.12,Massey Charlotte2ORCID,Tropman David4,Skorupinska Mariola12,Laurá Matilde12,Reilly Mary M.12,Ramdharry Gita M.12ORCID

Affiliation:

1. Department of Neuromuscular Diseases University College London: Institute of Neurology London UK

2. Queen Square Centre for Neuromuscular Diseases National Hospital for Neurology and Neurosurgery, University College Hospitals, NHS Foundation Trust London UK

3. School of Rehabilitation Sciences, Faculty of Health, Social Care and Education Kingston University and St Georges University of London London UK

4. Purchasing and Stores Department The London Clinic London UK

Abstract

AbstractIntroduction/AimsPeople with Charcot–Marie–Tooth Disease (CMT) frequently report problems with balance, which lead to an increased risk of falls. Evidence is emerging of training interventions to improve balance for people with CMT, but to date all have relied on clinic‐based treatment and equipment. This proof‐of‐concept study explored whether a multi‐modal program of proprioceptive rehabilitation and strength training can be delivered at home, to improve balance performance in people with CMT Type 1A.MethodsFourteen participants with CMT Type 1A were recruited into this randomized, two‐arm study. Baseline assessments included measures of disease severity, posturography, physical function, and patient‐reported outcome measurements. All participants received one falls education session. Participants were randomized to either 12 weeks of balance training or 12 weeks of usual activities. The intervention comprised a home‐based, multi‐sensory balance training and proximal strengthening program, supported by three home visits from a physiotherapist.ResultsThirteen participants completed the study. The intervention was successfully implemented and well tolerated, with high participation levels. Functional measures of balance and walking showed strong effect sizes in favor of the training group. Posturography testing demonstrated moderate improvements in postural stability favoring the intervention group. Inconsistent changes were seen in lower limb strength measures.DiscussionThe intervention was feasible to implement and safe, with some evidence of improvement in balance performance. This supports future studies to expand this intervention to larger trials of pragmatic, home‐delivered programs through current community rehabilitation services and supported self‐management pathways.

Funder

Kingston University

University College London Hospitals Biomedical Research Centre

Medical Research Council Canada

Muscular Dystrophy Association

Publisher

Wiley

Subject

Physiology (medical),Cellular and Molecular Neuroscience,Neurology (clinical),Physiology

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