The Easy Handgrip Test as a Tool for Assessing Motor Fatigability in Children With Charcot-Marie-Tooth Disease Type 1A

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System Pub Date : 2025-10-03 DOI:10.1111/jns.70061

Ester da Silva Estevam, Emanuela Juvenal Martins, Camila Scarpino Barboza Franco, Karoliny Lisandra Teixeira Cruz, Tenysson Will de Lemos, Pedro José Tomaselli, Wilson Marques Junior, Ana Claudia Mattiello-Sverzut

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Abstract

Background and Aims

Charcot-Marie-Tooth (CMT), the most common inherited neuromuscular disorder, causes progressive, symmetrical muscle weakness, often affecting distal extremities. Motor fatigability, characterized by a decline in maximal muscle force, is common in neuromuscular disorders but remains underexplored in children with CMT. This study aimed to evaluate the feasibility of a motor fatigability test using a bulb dynamometer in children and adolescents with CMT1A, assess test–retest reliability, and compare handgrip pressure, electromyographic parameters, perceived effort, and time to exhaustion between CMT1A and typically developing peers.

Methods

This observational cross-sectional study included 107 children (aged 8–16 years; 19 with CMT1A and 88 typically developing). Participants performed a handgrip motor fatigability test using a bulb dynamometer, involving repetitive maximum voluntary isometric contractions (MVICs) at one-second intervals until exhaustion, with simultaneous electromyographic monitoring. Test–retest reliability was assessed using Bland–Altman plots and the Intraclass Correlation Coefficient (ICC). For comparisons between groups and test phases, ANCOVA and linear mixed-effects models were adjusted for sex, age, height, and weight.

Results

A hundred and four children completed the test. The protocol was feasible, with both groups reaching significant exhaustion in 4 min and showing a significant decline in handgrip pressure (p < 0.05). The decline in median power frequency observed during the fatigue test indicated reduced neural activation in both groups. The ICC was 0.824, indicating good test–retest reliability.

Interpretation

The protocol shows promise for monitoring disease progression and treatment effects in children with CMT1A, and may serve as a functional marker. Future studies should include other CMT types.

Abstract Image

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简易握力试验作为评估1A型腓骨肌萎缩症儿童运动疲劳的工具

背景与目的：腓骨肌萎缩症（charco - marie - tooth， CMT）是最常见的遗传性神经肌肉疾病，可导致进行性、对称性肌肉无力，常累及远端肢体。运动疲劳，以最大肌肉力量下降为特征，在神经肌肉疾病中很常见，但在CMT患儿中仍未得到充分研究。本研究旨在评估在患有CMT1A的儿童和青少年中使用灯泡测力仪进行运动疲劳测试的可行性，评估测试-重测可靠性，并比较CMT1A和正常发展的同龄人之间的握力、肌电图参数、感知努力和疲劳时间。方法：本观察性横断面研究纳入107名儿童（8-16岁，19名患有CMT1A， 88名发育正常）。参与者使用灯泡测力仪进行了手部运动疲劳测试，包括每隔一秒重复最大自主等距收缩（mvic），直到精疲力竭，同时进行肌电监测。采用Bland-Altman图和类内相关系数（ICC）评估重测信度。对于组间和试验阶段的比较，采用ANCOVA和线性混合效应模型对性别、年龄、身高和体重进行调整。结果：104名儿童完成了测试。该方案是可行的，两组在4分钟内都达到了明显的疲劳，并且手部压力显着下降(p)解释：该方案有望监测CMT1A患儿的疾病进展和治疗效果，并可能作为功能标记物。未来的研究应包括其他CMT类型。

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

Journal of the Peripheral Nervous System 医学-临床神经学

CiteScore

6.10

自引率

7.90%

发文量

审稿时长

>12 weeks

期刊介绍： The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders. The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies. Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials. The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.