CMT 1A in children: Evolution of foot and ankle manifestations

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Muscle Nerve. 2009 Jan 14;39(2):158-166.

Evolution of foot and ankle manifestations in children with CMT1A.

Burns J, MM, Ouvrier RA.

Discipline of Paediatrics and Child Health, Faculty of Medicine,

University of Sydney/Institute for Neuromuscular Research, Children's

Hospital at Westmead, Locked Bag 4001, Westmead, Sydney, NSW 2145

Australia.

We studied the timing and progression of foot and ankle changes in 81

children with genetically confirmed Charcot-Marie-Tooth disease type

1A (CMT1A) and determined their impact on motor function and walking

ability.

Foot deformity, weakness, pain, cramps, and instability were a common

feature of CMT1A. Foot structure evolved toward pes cavus from early

childhood to adolescence, although a subgroup with normal and planus

feet remained. Foot strength increased with age, although compared to

age-equivalent norms it declined from 4 years.

Factors associated with evolving foot deformity included muscle

weakness/imbalance, restricted ankle flexibility, and joint

hypermobility.

Regression modeling identified dorsiflexion weakness, global foot

weakness, and difficulty toe-walking as independent predictors of

motor dysfunction, while pes cavus and difficulty heel-walking were

predictors of poor walking ability.

Foot problems are present from the earliest stages of the disease and

can have a negative impact on function. Early foot and ankle

intervention may prevent long-term disability and morbidity in CMT1A.