Elsevier

Neuromuscular Disorders

Volume 19, Issue 12, December 2009, Pages 818-821
Neuromuscular Disorders

Case report
Feasibility of foot and ankle strength training in childhood Charcot-Marie-Tooth disease

https://doi.org/10.1016/j.nmd.2009.09.007Get rights and content

Abstract

Weakness of ankle dorsiflexion is the cardinal manifestation of CMT. We investigated if a 12-week progressive resistance dorsiflexion strengthening program was feasible, safe and beneficial in a 15-year-old girl with an axonal form of CMT. Training load was based on a dose-escalating percentage of one-repetition maximum, completed on three non-consecutive days each week. Outcomes included dynamometric foot strength, motor function and instrumented walking ability. At 12-weeks, dorsiflexion strength improved 56–72% and plantarflexion strength by 15–20%. Standing long jump increased by 16%, while balance and endurance did not. Walking ability improved for speed, cadence, step time and stride length. Compliance was high and there were no adverse events. This case suggests progressive strength training might be a feasible intervention to help foot weakness and disability in childhood CMT.

Introduction

Charcot-Marie-Tooth disease (CMT), the most common genetic nerve disorder, is characterised by progressive length-dependent weakness and atrophy of the distal muscles of the limbs. In particular, weakness of ankle dorsiflexion is the cardinal manifestation of CMT and contributes to foot deformity, ankle contracture, poor motor function and walking difficulty in affected children and adults [1].

Strength training is a commonly used intervention for muscle weakness and atrophy in a range of patient populations aimed at improving capacity to undertake functional tasks [2]. In people with CMT though, few studies have investigated the effect of lower limb training on strength and function, and although moderate increases in strength and function have been found [3], [4], [5], these interventions have been limited to training of the proximal leg muscles. In contrast, there are no reports of strength training for distal leg muscles in children or adults with CMT. Yet these muscles are affected considerably more in CMT due to the length-dependent neuropathic weakness [6]. Given that foot strength is closely related to functional ability in healthy children [7] and that in paediatric CMT, foot and ankle weakness are closely related to problems with motor function and walking ability [1], increasing strength of the distal leg muscles might result in important gains in function. It has also been suggested though, that muscles in CMT may be weakened when exercised and that excessive activity should be avoided [8]. Therefore, we sought to investigate if a 12-week progressive resistance training program designed to strengthen ankle dorsiflexors was feasible, safe and beneficial in a child with CMT.

Section snippets

Case report

A 15-year-old girl (height 1.70 m, weight 64 kg) with a hereditary autosomal recessive axonal form of CMT volunteered to participate in this study. On genetic testing, she was negative for a mitofusin 2 mutation and her 20-year-old brother who was also affected, but more severely, was negative for CMT1A, CMTX, Hereditary Neuropathy with Liability to Pressure Palsies and Friedreich’s ataxia. Her nerve conduction studies produced mixed sensory and motor neuropathy responses. MRI of the brain and

Discussion

This study showed that high intensity progressive resistance training can strengthen ankle dorsiflexors and generally improve the strength of affected foot and ankle muscles in a child with CMT. These findings are consistent with strength training of other muscles groups in adults with CMT [4], [5]. There were also improvements in some functional tests, but not all. Given that foot and ankle strength is closely related to functional ability in children [7], larger or prolonged gains in strength

Acknowledgements

This study was supported in part by grants from The University of Sydney Research and Development Scheme (#K2701 U3332) and the National Health and Medical Research Council of Australia (NHMRC #336705). We thank Paul de Sensi for photography.

References (11)

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