Characterization and Related Factors of Postural Control in Adults With Late-Onset Pompe Disease: Cross-Sectional and Case Control Study.

IF 3.5 4区医学 Q1 ORTHOPEDICS

Physical Therapy Pub Date : 2025-05-19 DOI:10.1093/ptj/pzaf073

Théo Maulet, Thomas Cattagni, Sofiane Mahmoudi, Thomas Gandois, Fabien Dubois, Pascal Laforet, Céline Bonnyaud

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引用次数: 0

Abstract

Importance: Adults with late-onset Pompe disease (LOPD) experience lower limb weakness, balance disorders, and recurrent falls. Mechanisms underlying postural control (stability, orientation) and falls in LOPD remain poorly understood. Investigating these aspects is critical to guide care and rehabilitation.

Objective: This study compared postural control in participants with LOPD to controls, examining postural and muscular factors linked to falls and muscular contributions to postural alterations.

Design: This study was a cross-sectional, case-control study. Postural stability and orientation during stationary standing were evaluated using force platforms and 3D motion analysis under 2 visual conditions, while maximal muscle strength was measured with an isokinetic dynamometer.

Setting: This cross-sectional, case-control study was conducted at a teaching hospital, which was a reference center for neuromuscular disease.

Participants: This study involved 2 cohorts: participants with LOPD and control participants.

Main outcomes and measure: Postural stability (center of pressure: COP displacements, lower limb joint range of motion) and orientation (COP mean position, weight distribution, joint positions) parameters were assessed, alongside falls reported over 3 months and maximal lower limb muscles strength.

Results: Compared to 20 controls, 18 adults with LOPD showed impaired postural stability, especially medio-laterally with eyes closed (ES = 0.42-0.83), and an anterior shift of the COP without joint orientation changes. Falls were strongly associated with COP mean velocity (eyes open: ρ = 0.73; eyes closed: ρ = 0.74) and with hip abductor (ρ = - 0.77) and extensor strength (ρ = - 0.79). Hip extensor strength was most strongly associated with COP mean velocity (eyes closed: ρ = - 0.72; eyes open: ρ = - 0.69).

Conclusions: Adults with LOPD demonstrate impaired postural stability, especially in the medio-lateral plane with eyes closed, and an anteriorized upright orientation.

Relevance: Hip extensor and abductor strength, strongly linked to falls and stability, should be important to target in evaluations and rehabilitation and in studies on new therapies on LOPD.

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成人迟发性庞贝病的体位控制特征及相关因素：横断面和病例对照研究。

重要性：成人迟发性庞贝病（LOPD）会出现下肢无力、平衡障碍和复发性跌倒。LOPD的姿势控制（稳定性、方向）和跌倒的机制尚不清楚。调查这些方面对指导护理和康复至关重要。目的：本研究比较了LOPD参与者和对照组的姿势控制，检查与跌倒相关的姿势和肌肉因素以及肌肉对姿势改变的贡献。设计：本研究为横断面病例对照研究。在两种视觉条件下，使用力平台和三维运动分析评估静止站立时的姿势稳定性和方向，同时使用等速测力仪测量最大肌肉力量。背景：本横断面病例对照研究在一家教学医院进行，该医院是神经肌肉疾病的参考中心。参与者：本研究包括两个队列：LOPD参与者和对照组参与者。主要结果和测量：评估体位稳定性（压力中心：COP位移，下肢关节活动范围）和方向（COP平均位置，体重分布，关节位置）参数，以及3个月内报告的跌倒和最大下肢肌肉力量。结果：与20名对照组相比，18名成人LOPD患者体位稳定性受损，特别是闭眼时中外侧（ES = 0.42-0.83）， COP前移，但关节方向没有改变。跌倒与COP平均速度密切相关(睁眼：ρ = 0.73；闭眼：ρ = 0.74)，髋外展肌（ρ = - 0.77）和伸肌力量（ρ = - 0.79）。髋伸肌力量与COP平均速度最密切相关(闭眼：ρ = - 0.72；睁开眼睛：ρ = - 0.69)。结论：LOPD的成人表现为姿势稳定性受损，特别是在闭眼时的中外侧平面和前固定的直立方向。相关性：髋关节伸肌和外展肌力量与跌倒和稳定性密切相关，在LOPD的评估、康复和新疗法研究中应该是重要的目标。

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

Physical Therapy Multiple-

CiteScore

7.10

自引率

0.00%

发文量

187

审稿时长

4-8 weeks

期刊介绍： Physical Therapy (PTJ) engages and inspires an international readership on topics related to physical therapy. As the leading international journal for research in physical therapy and related fields, PTJ publishes innovative and highly relevant content for both clinicians and scientists and uses a variety of interactive approaches to communicate that content, with the expressed purpose of improving patient care. PTJ"s circulation in 2008 is more than 72,000. Its 2007 impact factor was 2.152. The mean time from submission to first decision is 58 days. Time from acceptance to publication online is less than or equal to 3 months and from acceptance to publication in print is less than or equal to 5 months.