Balance impairment in pediatric charcot–marie–tooth disease. Issue 3 (15th May 2019)

• Record Type: Journal Article
• Authors: Estilow, Timothy; Glanzman, Allan M.; Burns, Joshua; Harrington, Ann; Cornett, Kayla; Menezes, Manoj P.; Shy, Rosemary; Moroni, Isabella; Pagliano, Emanuela; Pareyson, Davide; Bhandari, Trupti; Muntoni, Francesco; Laurá, Matilde; Reilly, Mary M.; Finkel, Richard S.; Eichinger, Kate J.; Herrmann, David N.; Troutman, Gregory; Bray, Paula; Halaki, Mark; Shy, Michael E.; Yum, Sabrina W.
• Is Part Of: Muscle & nerve. Volume 60:Issue 3(2019)
• Page Start: 242
• Page End: 249
• Publication Date: 2019-05-15
• Subjects: balance -- Charcot–Marie–Tooth -- children -- intervention -- sensorimotor impairment; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.26500
• Languages: English
• ISSNs: 0148-639X

Abstract

ABSTRACT: Introduction : Balance impairment contributes to gait dysfunction, falls, and reduced quality of life in adults with Charcot–Marie–Tooth disease (CMT) but has been minimally examined in pediatric CMT. Methods : The CMT Pediatric Scale (CMTPedS) was administered to 520 children with CMT. Associations between balance function (Bruininks–Oseretsky Test of Motor Proficiency [BOT‐2]) and sensorimotor and gait impairments were investigated. Results : Daily trips/falls were reported by 42.3% of participants. Balance (BOT‐2) varied by CMT subtype, was impaired in 42% of 4‐year‐olds, and declined with age ( P < 0.001). Vibration ( P < 0.001), pinprick ( P < 0.004), ankle dorsiflexion strength ( P < 0.001), and foot alignment ( P < 0.004) were associated with BOT‐2 balance (adjusted R 2 = 0.28). The visual dependence of balance increased with age. Discussion : Balance impairment occurs from a young age in children with CMT. Balance intervention studies are required in pediatric CMT and should consider the degree of sensorimotor impairment, foot malalignment, and visual dependence. Muscle Nerve, 2019