Evolution of foot manifestations in children with Charcot-Marie-Tooth disease
© Burns et al; licensee BioMed Central Ltd. 2008
Published: 26 September 2008
Charcot-Marie-Tooth disease (CMT) is the most common genetic nerve disorder. The most prevalent form, CMT1A, is characterised by demyelinating neuropathy with progressive foot and ankle weakness, contractures and deformity. The wide range of foot/ankle manifestations in CMT1A complicates the assessment, diagnosis and therapy. We aimed to characterise foot and ankle strength, flexibility, morphology and symptoms in children with CMT1A.
81 children aged 2–16 y with CMT1A were objectively assessed for strength (dorsiflexion, plantarflexion, inversion, eversion) with hand-held dynamometry , ankle dorsiflexion flexibility using the weight bearing lunge  and foot morphology with the Foot Posture Index (FPI) . We also looked for difficulties in heel or tip-toe walking, foot drop during gait, and questioned about foot/ankle pain, cramps, ankle instability, trips and falls during walking.
Ankle dorsiflexion ranged from 7–41° (mean 25°, SD 7). Compared to normal, ankle flexibility was lower in children at all ages with CMT1A. Interestingly, there were no significant correlations between ankle flexibility and age, height or body weight.
Compared to norms, foot morphology in preschool children with CMT1A did not differ, but from the age of 5 years, deviated from normal towards a more cavoid appearance.
Difficulty heel walking occurred in 82% of children, 4% had difficulty tip-toe walking and 4% exhibited foot drop. 27% reported foot pain, 36% reported cramps, 72% described ankle instability, 63% reported frequent trips and 47% falls. Foot pain, cramps, toe-walking and foot drop worsened with age (p < 0.05).
Children with CMT1A experience foot weakness, contracture and deformity from an early age. These manifestations are expected to impact negatively on daily function. Early intervention targeting the foot and ankle may prevent long-term disability in CMT1A.
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