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Publication Detail
Association Between Body Mass Index and Disability in Children With Charcot-Marie-Tooth Disease.
  • Publication Type:
    Journal article
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  • Authors:
    Donlevy GA, Garnett SP, Cornett KMD, McKay MJ, Baldwin JN, Shy RR, Yum SW, Estilow T, Moroni I, Foscan M, Pagliano E, Pareyson D, Laura M, Bhandari T, Muntoni F, Reilly MM, Finkel RS, Sowden JE, Eichinger KJ, Herrmann DN, Shy ME, Burns J, Menezes MP
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  • Addresses:
    University of Sydney, Faculty of Medicine and Health, Sydney, Australia gabrielle.donlevy@health.nsw.gov.au.


This study examined the association between body mass index (BMI) and disability in children with Charcot-Marie-Tooth disease (CMT).


We conducted a cross-sectional analysis of 477 patients with CMT aged 3-20 years from the Inherited Neuropathy Consortium, and 316 age-and-sex matched healthy children from the 1000 Norms Project. BMI was categorised according to the International Obesity Task Force (IOTF) criteria, and BMI categorisation was compared with healthy children. IOTF categories (adult equivalent BMI cut points) were: severely underweight (BMI<17kg/m2); underweight (BMI≥17 to <18.5kg/m2); healthy weight (BMI≥18.5 to <25kg/m2); overweight (BMI ≥25 to <30kg/m2); obese (BMI ≥30kg/m2). Scores on the 0-44 point CMT Pediatric Scale (CMTPedS), a well-validated measure of disability, were examined in relation to BMI.


There was a higher proportion of children with CMT categorised as severely underweight (5.7%vs0.3%), underweight (10.3%vs5.1%), and obese (7.3%vs3.8%) (p<0.05). Fewer children with CMT were categorised as healthy weight (61.8%vs74.4%) (p<0.05), and the proportion of overweight (14.9%vs16.5%) between groups was similar. CMTPedS scores (mean ± SD) for weight categories were: severely underweight (27±9), underweight (20±8), healthy weight (17±9), overweight (17±9) obese (22 ±10). Compared to healthy weight children with CMT, being severely underweight was associated with being more disabled (p<0.001), as was being obese (p=0.015).


The proportion of underweight and obese children with CMT is higher compared to age-and sex-matched healthy children. Children with CMT who are underweight or obese are associated with greater disability than compared children with CMT of healthy weight.
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