Surgical correction of foot deformities in Charcot-Marie-Tooth-disease: effects on personal goals and gait capacity

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-10-01 DOI:10.1016/j.nmd.2025.106213

Heleen van der Wielen , Jorik Nonnekes , Michelle van Mierlo , Christian M.A. Donken , Jan Willem K. Louwerens , Noël L.W. Keijsers

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引用次数: 0

Abstract

Charcot Marie Tooth disease (CMT) is the most common group of inherited muscular disorders, resulting in foot deformities. Foot and ankle surgical procedures are frequently performed to improve function in persons with CMT. Understanding the patients’ personal goals is crucial for effective counselling. However, there is limited evidence regarding the extent to which personal goals are achieved through surgery, as well as the impact of surgery on balance and gait capacity. A prospective cohort study was conducted in 28 persons with CMT who underwent surgery. A wide range of personal goals was identified using the Canadian Occupational Performance Measure (COPM), with the most common goal being 'less problems during walking on uneven ground with shoes’. Wilcoxon signed-rank revealed a significant improvement in mean COPM scores after surgery for both performance (median difference: 3.0, range: -2 to 8; p < 0.001) and satisfaction (3.5, -2 to 9; p ≤ 0.001). Mini-BESTest (balance capacity) also showed significant improvement (17.0 to 20.3 points, p < 0.001), whereas gait (gait velocity, gait width, and single support time) remained unchanged. This study highlights the variability in personal goals, with an overlap on individual and group levels. After surgery, balance capacity improved, both objectively and subjectively.

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charcot - marie - tooth病足部畸形的手术矫正：对个人目标和步态能力的影响

Charcot Marie Tooth disease （CMT）是最常见的遗传性肌肉疾病，导致足部畸形。足部和踝关节手术通常用于改善CMT患者的功能。了解病人的个人目标是有效咨询的关键。然而，关于通过手术实现个人目标的程度，以及手术对平衡和步态能力的影响，证据有限。一项前瞻性队列研究对28名接受手术的CMT患者进行了研究。加拿大职业表现评估（COPM）确定了广泛的个人目标，其中最常见的目标是“穿鞋在不平坦的地面上行走时减少问题”。Wilcoxon sign -rank显示，手术后的平均COPM评分在表现（中位差：3.0，范围：-2至8；p < 0.001）和满意度（3.5，-2至9；p≤0.001）两方面均有显著改善。mini - best（平衡能力）也有显著改善（17.0至20.3分，p < 0.001），而步态（步态速度、步态宽度和单次支撑时间）保持不变。这项研究强调了个人目标的可变性，在个人和群体层面上存在重叠。手术后，平衡能力在客观上和主观上都有所改善。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neuromuscular Disorders 医学-临床神经学

CiteScore

4.60

自引率

3.60%

发文量

543

审稿时长

53 days

期刊介绍： This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.