Kennedy Rachel, Carroll Kate, Paterson Kade L, Ryan Monique M, McGinley Jennifer L
Department of Neurology, The Royal Children's Hospital, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia; The University of Melbourne, Parkville, Victoria, Australia.
Department of Neurology, The Royal Children's Hospital, Parkville, Victoria, Australia; Murdoch Childrens Research Institute, Parkville, Victoria, Australia.
Neuromuscul Disord. 2017 Jul;27(7):658-666. doi: 10.1016/j.nmd.2017.04.005. Epub 2017 Apr 19.
Children and adolescents with Charcot-Marie-Tooth disease (CMT) report problems with gait. Little is known about gait changes over time in children with this degenerative disease. This prospective longitudinal study investigated change in spatio-temporal gait parameters and functional ambulation. Two assessments were conducted 12 months apart. Assessments included: barefoot gait on an electronic walkway; functional ambulation with a six minute walk test and the Functional Mobility Scale; and disease severity with the CMT Pediatric Scale. Gait and strength data were normalised to body anthropometrics. Twenty-seven children (mean age 12.2, SD 3.7 years) with various CMT subtypes (CMT1A 52%) were evaluated. Over 12 months, normalised variables of gait speed, stride length and six minute walk distance significantly decreased. The CMT Pediatric Scale increased 0.7 points, indicating marginal but not significant worsening of disease; notably calf strength decreased significantly over 12 months. Baseline maximum ankle dorsiflexion angle, gastrocnemius and hamstrings length, and calf strength correlated with decline in gait speed, with maximum ankle dorsiflexion angle as the strongest predictor. Deterioration over one year in normalised variables of gait speed, stride length and distance walked in six minutes are indicators of functional decline and disease progression in children and adolescents with CMT.
患有夏科-马里-图思病（CMT）的儿童和青少年存在步态问题。对于患有这种退行性疾病的儿童，其步态随时间的变化情况知之甚少。这项前瞻性纵向研究调查了时空步态参数和功能性步行的变化。相隔12个月进行了两次评估。评估内容包括：在电子步道上的赤脚步态；通过六分钟步行测试和功能性活动量表进行的功能性步行；以及使用CMT儿童量表评估疾病严重程度。步态和力量数据根据身体人体测量学进行了标准化。对27名患有各种CMT亚型（CMT1A占52%）的儿童（平均年龄12.2岁，标准差3.7岁）进行了评估。在12个月的时间里，步态速度、步幅长度和六分钟步行距离的标准化变量显著下降。CMT儿童量表增加了0.7分，表明疾病有轻微但不显著的恶化；值得注意的是，小腿力量在12个月内显著下降。基线时最大踝关节背屈角度、腓肠肌和腘绳肌长度以及小腿力量与步态速度的下降相关，其中最大踝关节背屈角度是最强的预测指标。CMT儿童和青少年在一年中步态速度、步幅长度和六分钟步行距离的标准化变量恶化是功能衰退和疾病进展的指标。
