Case reportFeasibility of foot and ankle strength training in childhood Charcot-Marie-Tooth disease
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.
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2017, The Lancet Child and Adolescent HealthCitation Excerpt :Children exercised the dorsiflexors of each foot. Participants trained with a purpose-built adjustable exercise cuff, similar to ankle weights commonly available in sports stores and those used during pilot testing;13 however, the cuff was redesigned in-house to prevent sliding during movement and increase load capacity (figure 1). Participants trained three times per week on non-consecutive days for 6 months (72 training sessions in total).
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2015, Pediatric Clinics of North AmericaCitation Excerpt :Analysis of disease progression from diagnosis using standardized techniques (eg, CMTPeds: a validated pediatric neuropathy scoring system, some timed tests, motion analysis techniques) in collaborative studies would help improve understanding of the specific factors affecting disability and prognosis for future ambulation status, and would allow clinicians to optimize interventions to preserve ambulation and improve the quality of life of children with CMT. Regular physical therapy should focus on strengthening, range of motion, and balance training in order to maintain the mobility of patients.77 Swimming and other pool-based therapies may be useful for maintaining axial strength and preventing scoliosis.
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