小儿遗传性运动感觉神经病（CMT）的平衡障碍。
Balance impairment in pediatric charcot-marie-tooth disease.
机构信息
Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
The University of Sydney & Children's Hospital at Westmead, Sydney, Australia.
出版信息
Muscle Nerve. 2019 Sep;60(3):242-249. doi: 10.1002/mus.26500. Epub 2019 May 15.
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 = 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.
简介
平衡障碍可导致成年 Charcot-Marie-Tooth 病（CMT）患者步态功能障碍、跌倒和生活质量下降，但在儿科 CMT 中很少被检测到。
方法
对 520 名 CMT 患儿进行了 CMT 儿科量表（CMTPedS）评估。研究了平衡功能（Bruininks-Oseretsky 运动能力测试 [BOT-2]）与感觉运动和步态障碍之间的关系。
结果
42.3%的参与者报告日常出行/跌倒。平衡（BOT-2）因 CMT 亚型而异，4 岁儿童中有 42%存在平衡障碍，且随年龄增长而下降（P<0.001）。振动觉（P<0.001）、针刺觉（P<0.004）、踝关节背屈肌力（P<0.001）和足对线（P<0.004）与 BOT-2 平衡相关（调整后的 R=0.28）。平衡对视觉的依赖随年龄增长而增加。
讨论
CMT 患儿从很小的年龄就开始出现平衡障碍。儿科 CMT 需要进行平衡干预研究，应考虑感觉运动障碍的程度、足对线不良和视觉依赖。肌肉神经，2019 年。
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