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October 26, 2021; 97 (17) Research Article

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

Gabrielle A. Donlevy, View ORCID ProfileSarah P. Garnett, Kayla M.D. Cornett, Marnee J. McKay, Jennifer N. Baldwin, Rosemary R. Shy, View ORCID ProfileSabrina W. Yum, View ORCID ProfileTimothy Estilow, View ORCID ProfileIsabella Moroni, Maria Foscan, Emanuela Pagliano, Davide Pareyson, Matilde Laura, Trupti Bhandari, View ORCID ProfileFrancesco Muntoni, Mary M. Reilly, View ORCID ProfileRichard S. Finkel, Janet E. Sowden, Katy J. Eichinger, David N. Herrmann, Michael E. Shy, View ORCID ProfileJoshua Burns, View ORCID ProfileManoj P. Menezes
First published September 7, 2021, DOI: https://doi.org/10.1212/WNL.0000000000012725
Gabrielle A. Donlevy
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.
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Sarah P. Garnett
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.
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Kayla M.D. Cornett
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.
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Marnee J. McKay
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.
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Jennifer N. Baldwin
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.
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Rosemary R. Shy
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.
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Sabrina W. Yum
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.
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Timothy Estilow
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.
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Isabella Moroni
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.
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Maria Foscan
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.
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Emanuela Pagliano
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.
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Davide Pareyson
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.
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Matilde Laura
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.
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Trupti Bhandari
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.
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Francesco Muntoni
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.
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Mary M. Reilly
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.
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Richard S. Finkel
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.
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Janet E. Sowden
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.
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Katy J. Eichinger
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.
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David N. Herrmann
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.
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Michael E. Shy
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.
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Joshua Burns
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.
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Manoj P. Menezes
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.
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Association Between Body Mass Index and Disability in Children With Charcot-Marie-Tooth Disease
Gabrielle A. Donlevy, Sarah P. Garnett, Kayla M.D. Cornett, Marnee J. McKay, Jennifer N. Baldwin, Rosemary R. Shy, Sabrina W. Yum, Timothy Estilow, Isabella Moroni, Maria Foscan, Emanuela Pagliano, Davide Pareyson, Matilde Laura, Trupti Bhandari, Francesco Muntoni, Mary M. Reilly, Richard S. Finkel, Janet E. Sowden, Katy J. Eichinger, David N. Herrmann, Michael E. Shy, Joshua Burns, Manoj P. Menezes
Neurology Oct 2021, 97 (17) e1727-e1736; DOI: 10.1212/WNL.0000000000012725

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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.

Glossary

BMI=
body mass index;
CDC=
Centers for Disease Control and Prevention;
CMT=
Charcot-Marie-Tooth disease;
CMTPedS=
CMT Pediatric Scale;
IOTF=
International Obesity Task Force;
9-HPT=
Nine-Hole Peg Test;
6MWT=
6-minute walk test;
WHO=
World Health Organization; Nine-Hole Peg Test (9-HPT)

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • ↵* These authors contributed equally to this work as senior authors.

  • Received February 15, 2021.
  • Accepted in final form August 16, 2021.
  • © 2021 American Academy of Neurology
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