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小脑共济失调患者咳嗽技能训练单节段的可行性研究

Single-Session Feasibility of Cough Skill Training in Cerebellar Ataxia.

作者信息

Borders James C, Kuo Sheng-Han, Troche Michelle S

机构信息

Laboratory for the Study of Upper Airway Dysfunction, Department of Biobehavioral Sciences, Teachers College, Columbia University, New York, NY, USA.

Department of Neurology, Columbia University, New York, NY, USA.

出版信息

Cerebellum. 2025 Jul 11;24(5):129. doi: 10.1007/s12311-025-01884-1.

Abstract

Cerebellar ataxia (CA) is a heterogenous neurodegenerative disease affecting multiple neural structures. Individuals with CA exhibit difficulty coordinating voluntary movements and have a high prevalence of cough (dystussia) and swallowing (dysphagia) dysfunction. Although skill-based approaches to cough rehabilitation are efficacious for other neurogenerative diseases, the feasibility in CA remains unexplored. Seven people with genetically confirmed CA (6 female, 1 male) completed baseline voluntary cough assessments, followed by a single session of cough skill training (CST). Participants were instructed to cough with sufficient intensity to hit a target line set 25% above baseline maximum peak expiratory flow rate (PEFR). Metrics of feasibility included the percentage of trials completed, no adverse events, and duration of the treatment session. Bayesian multilevel models examined changes in three cough airflow outcomes: PEFR, cough expired volume (CEV), and cough inspired volume (CIV). All participants completed every trial of CST in less than one hour without any adverse events. PEFR improved by 0.77 L/s (95% CI: 0.37, 1.09) on single and 0.49 L/s (95% CI: 0.17, 0.76) on sequential voluntary cough. CEV increased by 0.36 L (95% CI: 0.11, 0.76) on single and 0.19 L (95% CI: 0.02, 0.46) on sequential voluntary cough. CIV showed no significant change. Individuals with CA demonstrated the ability to upregulate voluntary cough during a single session of CST within a manageable amount of time. These findings highlight the potential for modifiable cough outcomes in this population and support further research on the efficacy of CST in CA.

摘要

小脑共济失调(CA)是一种影响多个神经结构的异质性神经退行性疾病。患有CA的个体在协调自主运动方面存在困难,并且咳嗽(咳嗽障碍)和吞咽(吞咽困难)功能障碍的发生率很高。尽管基于技能的咳嗽康复方法对其他神经退行性疾病有效,但在CA中的可行性仍未得到探索。七名经基因确诊为CA的患者(6名女性,1名男性)完成了基线自主咳嗽评估,随后进行了一次咳嗽技能训练(CST)。参与者被指示以足够的强度咳嗽,以达到比基线最大呼气流量峰值(PEFR)高25%设定的目标线。可行性指标包括完成试验的百分比、无不良事件以及治疗 session 的持续时间。贝叶斯多级模型检查了三种咳嗽气流结果的变化:PEFR、咳嗽呼出量(CEV)和咳嗽吸入量(CIV)。所有参与者在不到一小时内完成了CST的每项试验,没有任何不良事件。单次自主咳嗽时PEFR提高了0.77 L/s(95%CI:0.37,1.09),连续自主咳嗽时提高了0.49 L/s(95%CI:0.17,0.76)。单次自主咳嗽时CEV增加了0.36 L(95%CI:0.11,0.76),连续自主咳嗽时增加了0.19 L(95%CI:0.02,0.46)。CIV没有显著变化。患有CA的个体在一次CST session中,在可管理的时间内表现出上调自主咳嗽的能力。这些发现突出了该人群中可改变咳嗽结果的潜力,并支持进一步研究CST在CA中的疗效。

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