Yunusova Yana, Green Jordan R, Wang Jun, Pattee Gary, Zinman Lorne
Department of Speech-Language Pathology, University of Toronto.
J Vis Exp. 2011 Feb 21(48):2422. doi: 10.3791/2422.
Improved methods for assessing bulbar impairment are necessary for expediting diagnosis of bulbar dysfunction in ALS, for predicting disease progression across speech subsystems, and for addressing the critical need for sensitive outcome measures for ongoing experimental treatment trials. To address this need, we are obtaining longitudinal profiles of bulbar impairment in 100 individuals based on a comprehensive instrumentation-based assessment that yield objective measures. Using instrumental approaches to quantify speech-related behaviors is very important in a field that has primarily relied on subjective, auditory-perceptual forms of speech assessment(1). Our assessment protocol measures performance across all of the speech subsystems, which include respiratory, phonatory (laryngeal), resonatory (velopharyngeal), and articulatory. The articulatory subsystem is divided into the facial components (jaw and lip), and the tongue. Prior research has suggested that each speech subsystem responds differently to neurological diseases such as ALS. The current protocol is designed to test the performance of each speech subsystem as independently from other subsystems as possible. The speech subsystems are evaluated in the context of more global changes to speech performance. These speech system level variables include speaking rate and intelligibility of speech. The protocol requires specialized instrumentation, and commercial and custom software. The respiratory, phonatory, and resonatory subsystems are evaluated using pressure-flow (aerodynamic) and acoustic methods. The articulatory subsystem is assessed using 3D motion tracking techniques. The objective measures that are used to quantify bulbar impairment have been well established in the speech literature and show sensitivity to changes in bulbar function with disease progression. The result of the assessment is a comprehensive, across-subsystem performance profile for each participant. The profile, when compared to the same measures obtained from healthy controls, is used for diagnostic purposes. Currently, we are testing the sensitivity and specificity of these measures for diagnosis of ALS and for predicting the rate of disease progression. In the long term, the more refined endophenotype of bulbar ALS derived from this work is expected to strengthen future efforts to identify the genetic loci of ALS and improve diagnostic and treatment specificity of the disease as a whole. The objective assessment that is demonstrated in this video may be used to assess a broad range of speech motor impairments, including those related to stroke, traumatic brain injury, multiple sclerosis, and Parkinson disease.
改进评估延髓功能障碍的方法对于加快肌萎缩侧索硬化症（ALS）中延髓功能障碍的诊断、预测跨言语子系统的疾病进展以及满足正在进行的实验性治疗试验对敏感结局指标的迫切需求至关重要。为满足这一需求，我们正在基于全面的仪器评估获取100名个体的延髓功能障碍纵向概况，该评估可产生客观指标。在一个主要依赖主观听觉感知形式的言语评估的领域中，使用仪器方法量化与言语相关的行为非常重要(1)。我们的评估方案测量所有言语子系统的表现，这些子系统包括呼吸、发声（喉部）、共鸣（腭咽）和发音。发音子系统分为面部部分（下颌和嘴唇）和舌头。先前的研究表明，每个言语子系统对诸如ALS等神经系统疾病的反应不同。当前的方案旨在尽可能独立于其他子系统来测试每个言语子系统的表现。言语子系统是在言语表现的更全面变化的背景下进行评估的。这些言语系统水平变量包括说话速度和言语清晰度。该方案需要专门的仪器以及商业和定制软件。呼吸、发声和共鸣子系统使用压力-流量（空气动力学）和声学方法进行评估。发音子系统使用三维运动跟踪技术进行评估。用于量化延髓功能障碍的客观指标在言语文献中已得到充分确立，并显示出对延髓功能随疾病进展变化的敏感性。评估结果是每个参与者的全面跨子系统表现概况。与从健康对照中获得的相同指标相比，该概况用于诊断目的。目前，我们正在测试这些指标对ALS诊断和预测疾病进展速度的敏感性和特异性。从长远来看，这项工作得出的更精细的延髓性ALS内表型有望加强未来识别ALS基因位点的努力，并提高该疾病整体的诊断和治疗特异性。本视频中展示的客观评估可用于评估广泛的言语运动障碍，包括与中风、创伤性脑损伤、多发性硬化症和帕金森病相关的障碍。
