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Gait patterns of children and adolescents with Charcot-Marie-Tooth disease

机译：具有Charcot-Marie-Tooth疾病的儿童和青少年的步态模式

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Gait abnormalities reported in childhood Charcot-Marie-Tooth disease (CMT) include foot-drop, reduced ankle power at push-off and increased knee and hip flexion for swing clearance ('steppage-gait'). The purpose of this study was to describe the gait patterns of 60 children aged 6-17 years with CMT (CMTall) and distinguish differences based on functional weakness using the CMT Pediatric Scale (CMTPedS). Data were captured using Vicon Nexus system and compared to 50 healthy norms. Data were subdivided into three groups denoting increasing severity of dorsiflexion and plantarflexion weakness from the CMTPedS: no difficulty heel or toe walking (CMTND), difficulty heel walking (CMTDH), difficulty toe and heel walking (CMTDTH). Compared to healthy norms, CMTall demonstrated significantly worse gait profile score, reduced ankle dorsiflexion during swing (foot-drop), reduced ankle dorsiflexor moment in loading response and reduced external thigh-foot angle. Contrary to previous studies there were no signs of reduced ankle power or compensation through 'steppage gait' in this mild-moderately affected population. Instead, CMTall demonstrated reduced internal hip rotation and reduced hip abductor moment. When data were sub-grouped and compared to healthy norms, three different gait patterns at the ankle emerged: CMTND had a near-normal gait pattern, CMTDH presented with foot-drop, and CMTDTH had increased peak dorsiflexion and reduced ankle power generation. Several distinct and abnormal gait patterns were identified in children with CMT, with increasing gait abnormalities in more functionally severe cases. Classifying gait patterns based on disease severity might be a valuable tool in clinical decision making, assessing disease progression and phenotype-genotype correlation studies.
机译：儿童时期Charcot-Marie-Tooth疾病（CMT）报告的步态异常包括脚粪，在推迟下减少脚踝功率，增加膝关节和臀部屈曲，用于摆动间隙（'STEMPAGE-GAIT'）。本研究的目的是用CMT（CMTALL）描述60例6-17岁儿童的步态模式，并使用CMT儿科（CMTPES）基于功能弱点来区分差异。使用VICON Nexus系统捕获数据，并与50个健康规范相比。数据被细分为三组，表示来自CMT的背包和Plantarflexion弱点的越来越严重：脚跟或脚趾走路（CMTND），困难鞋跟步行（CMTDH），难以脚跟和脚跟步行（CMTDTH）。与健康规范相比，CMTALL展示了显着更差的步态型材评分，在摆动（脚下降）期间减少了脚踝背屈，减少了脚踝背屈时刻，加载响应和缩小外部大腿角。与之前的研究相反，通过在这种轻度中等受影响的人口中，通过“继发性步态”，没有减少脚踝动力或补偿的迹象。相反，CMTALL证明了内部髋部旋转和降低了髋关节旋转力矩。当数据被分组并与健康规范进行比较时，踝关节的三种不同的步态图案出现：CMTND具有近似正常的步态图案，CMTDH具有脚粪，CMTDTH增加了峰值背积峰值和降低的脚踝发电。在CMT的儿童中鉴定了几种明显的和异常的步态模式，随着在更功能严重的情况下的步态异常增加。基于疾病严重程度进行分类步态模式可能是临床决策中的有价值的工具，评估疾病进展和表型基因型相关性研究。

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