. 2021 Oct 26;97(17):e1727-e1736.

doi: 10.1212/WNL.0000000000012725. Epub 2021 Sep 7.

Association Between Body Mass Index and Disability in Children With Charcot-Marie-Tooth Disease

Gabrielle A Donlevy¹, Sarah P Garnett², Kayla M D Cornett², Marnee J McKay², Jennifer N Baldwin², Rosemary R Shy², Sabrina W Yum², Timothy Estilow², Isabella Moroni², Maria Foscan², Emanuela Pagliano², Davide Pareyson², Matilde Laura², Trupti Bhandari², Francesco Muntoni², Mary M Reilly², Richard S Finkel², Janet E Sowden², Katy J Eichinger², David N Herrmann², Michael E Shy², Joshua Burns², Manoj P Menezes²

Affiliations

¹ From the University of Sydney (G.A.D., S.P.G., M.P.M.), Faculty of Medicine and Health; Children's Hospital at Westmead (G.A.D., S.P.G., K.M.D.C., J.B., M.P.M.); University of Sydney (K.M.D.C., M.J.M., J.B.), School of Health Sciences; Faculty of Health and Medicine (J.N.B.), University of Newcastle, Australia; Departments of Pediatrics (R.R.S.) and Neurology (M.E.S.), Carver College of Medicine, University of Iowa, Iowa City; Division of Neurology (S.W.Y.) and Department of Occupational Therapy (T.E.), Children's Hospital of Philadelphia; Department of Neurology (S.W.Y., T.E.), Perelman School of Medicine, University of Pennsylvania, Philadelphia; Fondazione IRCCS Istituto Neurologico Carlo Besta (I.M., M.F., E.P., D.P.), Milan, Italy; Centre for Neuromuscular Diseases (M.L., M.M.R.), University College London, Queen Square; University College London Institute of Child Health & Great Ormond Street Hospital (T.B., F.M.), London, England; Translational Neurosciences (Pediatrics) (R.S.F.), St. Jude Children's Research Hospital, Memphis, TN; and Department of Neurology (J.E.S., K.J.E., D.N.H.), University of Rochester, NY. gabrielle.donlevy@health.nsw.gov.au.
² From the University of Sydney (G.A.D., S.P.G., M.P.M.), Faculty of Medicine and Health; Children's Hospital at Westmead (G.A.D., S.P.G., K.M.D.C., J.B., M.P.M.); University of Sydney (K.M.D.C., M.J.M., J.B.), School of Health Sciences; Faculty of Health and Medicine (J.N.B.), University of Newcastle, Australia; Departments of Pediatrics (R.R.S.) and Neurology (M.E.S.), Carver College of Medicine, University of Iowa, Iowa City; Division of Neurology (S.W.Y.) and Department of Occupational Therapy (T.E.), Children's Hospital of Philadelphia; Department of Neurology (S.W.Y., T.E.), Perelman School of Medicine, University of Pennsylvania, Philadelphia; Fondazione IRCCS Istituto Neurologico Carlo Besta (I.M., M.F., E.P., D.P.), Milan, Italy; Centre for Neuromuscular Diseases (M.L., M.M.R.), University College London, Queen Square; University College London Institute of Child Health & Great Ormond Street Hospital (T.B., F.M.), London, England; Translational Neurosciences (Pediatrics) (R.S.F.), St. Jude Children's Research Hospital, Memphis, TN; and Department of Neurology (J.E.S., K.J.E., D.N.H.), University of Rochester, NY.

PMID: 34493614
PMCID: PMC8605613
DOI: 10.1212/WNL.0000000000012725

Association Between Body Mass Index and Disability in Children With Charcot-Marie-Tooth Disease

Gabrielle A Donlevy et al. Neurology. 2021.

. 2021 Oct 26;97(17):e1727-e1736.

doi: 10.1212/WNL.0000000000012725. Epub 2021 Sep 7.

Authors

Affiliations

¹ From the University of Sydney (G.A.D., S.P.G., M.P.M.), Faculty of Medicine and Health; Children's Hospital at Westmead (G.A.D., S.P.G., K.M.D.C., J.B., M.P.M.); University of Sydney (K.M.D.C., M.J.M., J.B.), School of Health Sciences; Faculty of Health and Medicine (J.N.B.), University of Newcastle, Australia; Departments of Pediatrics (R.R.S.) and Neurology (M.E.S.), Carver College of Medicine, University of Iowa, Iowa City; Division of Neurology (S.W.Y.) and Department of Occupational Therapy (T.E.), Children's Hospital of Philadelphia; Department of Neurology (S.W.Y., T.E.), Perelman School of Medicine, University of Pennsylvania, Philadelphia; Fondazione IRCCS Istituto Neurologico Carlo Besta (I.M., M.F., E.P., D.P.), Milan, Italy; Centre for Neuromuscular Diseases (M.L., M.M.R.), University College London, Queen Square; University College London Institute of Child Health & Great Ormond Street Hospital (T.B., F.M.), London, England; Translational Neurosciences (Pediatrics) (R.S.F.), St. Jude Children's Research Hospital, Memphis, TN; and Department of Neurology (J.E.S., K.J.E., D.N.H.), University of Rochester, NY. gabrielle.donlevy@health.nsw.gov.au.
² From the University of Sydney (G.A.D., S.P.G., M.P.M.), Faculty of Medicine and Health; Children's Hospital at Westmead (G.A.D., S.P.G., K.M.D.C., J.B., M.P.M.); University of Sydney (K.M.D.C., M.J.M., J.B.), School of Health Sciences; Faculty of Health and Medicine (J.N.B.), University of Newcastle, Australia; Departments of Pediatrics (R.R.S.) and Neurology (M.E.S.), Carver College of Medicine, University of Iowa, Iowa City; Division of Neurology (S.W.Y.) and Department of Occupational Therapy (T.E.), Children's Hospital of Philadelphia; Department of Neurology (S.W.Y., T.E.), Perelman School of Medicine, University of Pennsylvania, Philadelphia; Fondazione IRCCS Istituto Neurologico Carlo Besta (I.M., M.F., E.P., D.P.), Milan, Italy; Centre for Neuromuscular Diseases (M.L., M.M.R.), University College London, Queen Square; University College London Institute of Child Health & Great Ormond Street Hospital (T.B., F.M.), London, England; Translational Neurosciences (Pediatrics) (R.S.F.), St. Jude Children's Research Hospital, Memphis, TN; and Department of Neurology (J.E.S., K.J.E., D.N.H.), University of Rochester, NY.

PMID: 34493614
PMCID: PMC8605613
DOI: 10.1212/WNL.0000000000012725

Abstract

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

Methods: We conducted a cross-sectional analysis of 477 patients with CMT who were 3 to 20 years of age from the Inherited Neuropathy Consortium and 316 age- and sex-matched healthy children from the 1,000 Norms Project. BMI was categorized according to the International Obesity Task Force (IOTF) criteria, and BMI categorization was compared with healthy children. IOTF categories (adult equivalent BMI cut points) were severely underweight (BMI <17 kg/m²), underweight (BMI ≥17-<18.5 kg/m²), healthy weight (BMI ≥18.5-<25 kg/m²), overweight (BMI ≥25-<30 kg/m²), and obese (BMI ≥30 kg/m²). Scores on the 0 to 44-point CMT Pediatric Scale (CMTPedS), a well-validated measure of disability, were examined in relation to BMI.

Results: There was a higher proportion of children with CMT categorized as severely underweight (5.7% vs 0.3%), underweight (10.3% vs 5.1%), and obese (7.3% vs 3.8%) (p < 0.05). Fewer children with CMT were categorized as healthy weight (61.8% vs 74.4%) (p < 0.05), and the proportion of overweight (14.9% vs 16.5%) between groups was similar. CMTPedS scores (mean ± SD) for weight categories were as follows: severely underweight 27 ± 9, underweight 20 ± 8, healthy weight 17 ± 9, overweight 17 ± 9, and obese 22 ± 10. Compared to children with a healthy weight with CMT, being severely underweight was associated with being more disabled (p < 0.001), as was being obese (p = 0.015).

Discussion: The proportion of children with CMT who are underweight or obese is higher compared to age- and sex-matched healthy children. In children with CMT, being underweight or obese is associated with greater disability, when compared to children with CMT of healthy weight.

PubMed Disclaimer

Figures

**Figure 1. Mean CMTPedS Disability Scores (With Standard Error) According to BMI Categories**
Severely underweight (body mass index [BMI] <17 kg/m²), underweight (BMI ≥17 to <18.5 kg/m²), healthy weight (BMI ≥18.5–<25 kg/m²), overweight (BMI ≥25–<30 kg/m²), and obese (BMI ≥30 kg/m²) (with standard error). Horizontal bars represent significance between groups. CMTPedS = Charcot-Marie-Tooth Pediatric Scale. Significance represented by *p ≤ 0.05.

**Figure 2. CMTPedS Item Scores by BMI Categories for Hand Dexterity (Functional Dexterity Test, Nine-Hole Peg Test), Muscle Strength (Grip, Plantarflexion Dorsiflexion), and Power (Long Jump)**
Higher mean (SE) item scores indicate higher disability. CMTPedS = Charcot-Marie-Tooth Pediatric Scale. Horizontal bars are significant differences between body mass index (BMI) categories represented by *p ≤ 0.05.

**Figure 3. CMTPedS Items Scores by BMI Categories for Balance, Endurance (6-Minute Walk Test), Gait, and Sensation (Pinprick, Vibration)**
Higher mean (SE) item scores indicate higher disability. Horizontal bars are significant differences between body mass index (BMI) categories represented by *p ≤ 0.05. CMTPedS = Charcot-Marie-Tooth Pediatric Scale.

See this image and copyright information in PMC

Cited by

Association of Body Mass Index With Disease Progression in Children With Charcot-Marie-Tooth Disease.
Donlevy GA, Cornett KMD, Garnett SP, Shy R, Estilow T, Yum SW, Anderson K, Pareyson D, Moroni I, Muntoni F, Reilly MM, Finkel RS, Herrmann DN, Eichinger KJ, Shy ME, Burns J, Menezes MP. Donlevy GA, et al. Neurology. 2023 Aug 15;101(7):e717-e727. doi: 10.1212/WNL.0000000000207488. Epub 2023 Jun 28. Neurology. 2023. PMID: 37380432 Free PMC article.

References

1. Rossor AM, Polke JM, Houlden H, Reilly MM. Clinical implications of genetic advances in Charcot-Marie-Tooth disease. Nat Rev Neurol. 2013;9(10):562-571. - PubMed
1. Hoyle JC, Isfort MC, Roggenbuck J, Arnold WD. The genetics of Charcot-Marie-Tooth disease: current trends and future implications for diagnosis and management. Appl Clin Genet. 2015;8:235-243. - PMC - PubMed
1. Burns J, Ouvrier R, Estilow T, et al. . Validation of the Charcot-Marie-Tooth Disease Pediatric Scale as an outcome measure of disability. Ann Neurol. 2012;71(5):642-652. - PMC - PubMed
1. Patzko A, Shy M. Update on Charcot-Marie-Tooth disease. Curr Neurol Neurosci Rep. 2011;11(1):78-88. - PMC - PubMed
1. Pareyson D, Saveri P, Pisciotta C. New developments in Charcot-Marie-Tooth neuropathy and related diseases. Curr Opin Neurol. 2017;30(5):471-480. - PubMed

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions

LinkOut - more resources

Full Text Sources
Medical
- MedlinePlus Health Information

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

Association Between Body Mass Index and Disability in Children With Charcot-Marie-Tooth Disease

Affiliations

Association Between Body Mass Index and Disability in Children With Charcot-Marie-Tooth Disease

Authors

Affiliations

Abstract

Figures

Similar articles

Cited by

References

MeSH terms

LinkOut - more resources

Full Text Sources

Medical

Abstract

Figures

Similar articles

Cited by

References

MeSH terms

Related information

LinkOut - more resources

Full Text Sources

Medical