Magnetic resonance imaging of the anterior compartment of the lower leg is a biomarker for weakness, disability, and impaired gait in childhood Charcot–Marie–Tooth disease. Issue 2 (19th December 2018)

• Record Type: Journal Article
• Authors: Cornett, Kayla M. D.; Wojciechowski, Elizabeth; Sman, Amy D.; Walker, Terri; Menezes, Manoj P.; Bray, Paula; Halaki, Mark; Burns, Joshua
• Other Names: Sman D. investigator.; Cornett Kayla M. D. investigator.; Wojciechowski Elizabeth investigator.; Menezes Manoj P. investigator.; Mandarakas Melissa R. investigator.; Rose Kristy J. investigator.; Bray Paula investigator.; Baldwin Jennifer investigator.; McKay Marnee J. investigator.; Refshauge Kathryn M. investigator.; Raymond Jacqueline investigator.; Walker Terri investigator.; Sampaio Hugo investigator.; Mudge Anita investigator.; Purcell Leanne investigator.; Miller Clare investigator.; Gray Kelly investigator.; Harman Meghan investigator.; Gabrael Natalie investigator.; Ouvrier Robert A. investigator.; Farrar Michelle investigator.; Sman Amy investigator.
• Is Part Of: Muscle & nerve. Volume 59:Issue 2(2019)
• Page Start: 213
• Page End: 217
• Publication Date: 2018-12-19
• Subjects: 3‐dimensional gait analysis -- Charcot–Marie–Tooth disease -- MRI -- musculoskeletal -- pediatric; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.26352
• Languages: English
• ISSNs: 0148-639X

Abstract

ABSTRACT: Introduction : Biomarkers of disease severity in Charcot–Marie–Tooth disease (CMT) are required to evaluate early responses to treatment. In this study we used magnetic resonance imaging (MRI) to evaluate the relationship between muscle volume and intramuscular fat accumulation with weakness, disability, and impaired gait in affected children and adolescents. Methods : Fifty‐five participants underwent MRI of the anterior compartment of the lower leg. Muscle and fat volumes were calculated. Strength was measured using hand‐held dynamometry, disability using the CMT Pediatric Scale, and 3‐dimensional gait analysis using an 8‐camera Vicon Nexus motion capture system. Results : Lower muscle volume was significantly associated with reduced dorsiflexion strength, increased disability, impaired gait profile score, and foot drop. Intramuscular fat accumulation was associated with reduced dorsiflexion strength and impaired gait profile score. Discussion : The MRI protocol described was feasible, reliable, and sensitive to the magnitude of weakness, disability, and walking difficulties in children with CMT. Muscle Nerve 59 :213–217, 2019