Physical activity of children and adolescents with Charcot-Marie-Tooth neuropathies: A cross-sectional case-controlled study.

Rachel A Kennedy,Kristy Rose,Jennifer L Mcginley,Kade L Paterson,Joshua Burns,Monique M Ryan,Kate Carroll,Matti Douglas Allen

doi:10.1371/journal.pone.0209628

Abstract

BackgroundDisability related to the progressive and degenerative neuropathies known collectively as Charcot-Marie-Tooth disease (CMT) affects gait and function, increasing with age and influencing physical activity in adults with CMT. The relationship between CMT-related disability, ambulatory function and physical activity in children and adolescents with CMT is unknown.MethodA cross-sectional case-controlled study of physical activity in 50 children with CMT and age- and gender-matched typically developing (TD) controls [mean age 12.5 (SD 3.9) years]. A 7-day recall questionnaire assessed physical activity; CMT-related disability and gait-related function were measured to explore factors associated with physical activity.ResultsChildren with CMT were less active than TD controls (estimated weekly moderate to vigorous physical activity CMT 283.6 (SD 211.6) minutes, TD 315.8 (SD 204.0) minutes; p < 0.001). The children with CMT had moderate disability [CMT Pediatric Scale mean score 17 (SD 8) /44] and reduced ambulatory capacity in a six-minute walk test [CMT 507.7 (SD 137.3) metres, TD 643.3 (74.6) metres; p < 0.001]. Physical activity correlated with greater disability (ρ = -0.56, p < 0.001) and normalised six-minute walk distance (ρ = 0.74, p < 0.001).ConclusionsCMT-related disability affects physical activity and gait-related function in children and adolescents with CMT compared to TD peers. Reduced physical activity adversely affects function across the timespan of childhood and adolescence into adulthood in people with CMT.

Highlights

Physical activities involving walking, running and jumping are often limited by progressive muscle weakness in individuals affected by the inherited peripheral neuropathies collectively known as Charcot-Marie-Tooth disease (CMT) [1, 2]
Children with CMT were less active than typically developing (TD) controls (estimated weekly moderate to vigorous physical activity CMT 283.6 (SD 211.6) minutes, TD 315.8 (SD 204.0) minutes; p < 0.001)
Physical activity correlated with greater disability (ρ = -0.56, p < 0.001) and normalised six-minute walk distance (ρ = 0.74, p < 0.001)

Summary

Introduction

Physical activities involving walking, running and jumping are often limited by progressive muscle weakness in individuals affected by the inherited peripheral neuropathies collectively known as Charcot-Marie-Tooth disease (CMT) [1, 2]. Reduced physical activity is associated with negative health outcomes irrespective of disability, with a compounding effect in adulthood [3,4,5]. For children and adolescents (“children”) with CMT, the negative health consequences of reduced physical activity are likely to compound CMT-related disability. It is possible that children with CMT are less active than their typically developing peers due to CMTrelated disability and are at risk of negative health outcomes related to inactivity. Disability related to the progressive and degenerative neuropathies known collectively as Charcot-Marie-Tooth disease (CMT) affects gait and function, increasing with age and influencing physical activity in adults with CMT. The relationship between CMT-related disability, ambulatory function and physical activity in children and adolescents with CMT is unknown

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