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Balance impairment in pediatric charcot-marie-tooth disease.

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.
Muscle Nerve; 60(3): 242-249, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31026080


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.


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.


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 R2 = 0.28). The visual dependence of balance increased with age.


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.
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