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使用安静站立和反应性姿势控制评估脊髓和延髓性肌萎缩中的感觉和运动功能

Evaluation of Sensory and Motor Function in Spinal and Bulbar Muscular Atrophy Using Quiet Stance and Reactive Postural Control.

作者信息

Shrader Joseph A, Sansare Ashwini, Niemic Allison C, Jiménez-Silva Rafael, Woolstenhulme Joshua G, Joe Galen O, Jacobs Uma, Kokkinis Angela, Fischbeck Kenneth, Grunseich Chris, Zampieri Cris

机构信息

Rehabilitation Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA.

Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA.

出版信息

Neurol Int. 2025 May 22;17(6):79. doi: 10.3390/neurolint17060079.

Abstract

INTRODUCTION

Spinal and bulbar muscular atrophy (SBMA) is an X-linked neuromuscular disorder characterized by progressive muscle weakness, along with muscle cramps, tremors, and sensory neuropathy. Previous research has shown that patients with SBMA have difficulty with dynamic balance and sensory postural control during quiet stance. There have been no reports on automatic postural reactions in SBMA.

OBJECTIVES

In this study, we aimed (1) to augment previous findings of sensory postural control, (2) to investigate automatic postural reactions in SBMA, and (3) to explore the relationship between strength and balance.

DESIGN

A cross-sectional design was used for the analysis.

PARTICIPANTS

The participants were fifty male individuals with a confirmed diagnosis of SBMA.

OUTCOME MEASURES

Balance testing included the NeuroCom modified Clinical Test of Sensory Interaction on Balance (mCTSIB), which measures sway velocity during quiet stance, and the NeuroCom Motor Control Test (MCT), which measures the latency and strength of postural reactions following sudden perturbations. Strength testing included maximal voluntary isometric contractions measured via fixed-frame dynamometry.

RESULTS

Forty-seven out of fifty participants were able to complete the mCTSIB test, but only thirty-eight completed the MCT test. Patients who were unable to complete the MCT were significantly weaker in all lower extremity muscles compared to those who were able to complete testing. Compared to normative data, participants showed significantly higher sway velocity during quiet stance across all conditions of the mCTSIB, except when standing on foam with eyes open. They also exhibited significantly slower postural reactions in response to sudden shifts of the force plate on the MCT. Plantarflexor weakness was significantly correlated with poor postural control on the mCTSIB and MCT.

CONCLUSIONS

This study confirms previously reported abnormalities of sensory postural control in SBMA and highlights patients' heavy reliance on visual inputs for postural control. Additionally, this study shows that automatic postural corrections are slower than normal in SBMA and provides a unique approach for measuring the combined sensory and motor components of the disease. Both the sensory and automatic balance abnormalities were found to be associated with plantarflexor weakness and may contribute to a higher risk of falls under challenging situations. Therefore, addressing this weakness may be an important step toward fall prevention in this population.

摘要

引言

脊髓延髓性肌萎缩症(SBMA)是一种X连锁神经肌肉疾病,其特征为进行性肌肉无力,伴有肌肉痉挛、震颤和感觉神经病变。先前的研究表明,SBMA患者在安静站立时动态平衡和感觉姿势控制存在困难。目前尚无关于SBMA患者自动姿势反应的报道。

目的

在本研究中,我们旨在(1)补充先前关于感觉姿势控制的研究结果,(2)调查SBMA患者的自动姿势反应,以及(3)探索力量与平衡之间的关系。

设计

采用横断面设计进行分析。

参与者

参与者为50名确诊为SBMA的男性个体。

结果测量

平衡测试包括NeuroCom改良的平衡感觉交互临床测试(mCTSIB),该测试测量安静站立时的摇摆速度,以及NeuroCom运动控制测试(MCT),该测试测量突然扰动后姿势反应的潜伏期和强度。力量测试包括通过固定框架测力计测量的最大自主等长收缩。

结果

50名参与者中有47名能够完成mCTSIB测试,但只有38名完成了MCT测试。与能够完成测试的患者相比,无法完成MCT的患者所有下肢肌肉明显较弱。与标准数据相比,除了睁眼站在泡沫上时,参与者在mCTSIB的所有条件下安静站立时的摇摆速度均显著更高。他们在MCT上对测力台突然移动的姿势反应也明显较慢。跖屈肌无力与mCTSIB和MCT上的姿势控制不佳显著相关。

结论

本研究证实了先前报道的SBMA患者感觉姿势控制异常,并强调患者在姿势控制方面严重依赖视觉输入。此外,本研究表明SBMA患者的自动姿势矫正比正常人慢,并提供了一种独特的方法来测量该疾病的感觉和运动综合成分。感觉和自动平衡异常均与跖屈肌无力有关,可能导致在具有挑战性的情况下跌倒风险更高。因此,解决这种肌无力可能是该人群预防跌倒的重要一步。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/610d/12196303/20495e9da08d/neurolint-17-00079-g001.jpg

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