Протокол Комплексная оценка Бульбарная дисфункции в Боковой амиотрофический склероз (ALS)
Summary
Цель оценки физиологических механизмов, которые поддерживают речи необходимы для мониторинга началом заболевания и прогрессирования у пациентов с БАС и количественно эффект лечения в клинических испытаниях. В этом видео мы представляем комплексный, приборов протокол на основе количественной производительности речи двигателя в клинических популяциях.
Abstract
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 assessment1. 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.
Protocol
Discussion
Здесь мы показали всеобъемлющего протокола для оценки бульбарный (речь) дисфункции в ALS. Данные, полученные из этого протокола используются для более глубокого понимания того, как ALS влияет речи. Эти данные также используются для определения наиболее чувствительные меры прогрессирования заболевания. Хотя этот протокол в настоящее время используется для исследований, результаты этого исследования будут использованы для разработки более экономичных и клинически возможные подходы к количественному бульбарной участия.
Disclosures
The authors have nothing to disclose.
Acknowledgements
Эта работа получила поддержку со стороны Национального института здравоохранения, Национальный институт по глухоты и других расстройств связи, Грант R01DCO09890-02, Канадского фонда для инноваций (CFI-LOF # 15704), и Connaught Фонда, Университета Торонто. Авторы хотели бы поблагодарить Синтия Дидион, Мили Kuruvilla, Криста Руди, и Лори Synhorst за помощь в сборе данных и анализе, и Кара Ульман для создания видео клипов.
Анимация выступили Синий Издание Tree ( http://www.bluetreepublishing.com/ )
СПА и SMASH программное обеспечение Matlab основе и можно получить, обратившись в Иордании Зеленый jgreen4@unl.edu.
Посетите наши лаборатории:
Бульбарная Функция лаборатории (Саннибрук медицинских наук центр в Торонто, Канада):
http://www.sunnybrook.ca/research/?page=sri_groups_bulb_home
Речь производственная лаборатория (Университет штата Небраска Линкольне):
http://spl.unl.edu
Materials
- Equipment / Software
- Phonatory Aerodynamic System (PAS), KayPENTAX, Lincoln Park, NJ, USA
- Compact flash recorder (E.g., PMD660),
- Professional quality microphone,
- SPL meter, Extech Instruments
- MDVP, KAYPentax
- Nasometer, Model 6400, KAYPentax
- Eagle Digital System, Motion Analysis Corp.
- WAVE, Northern Digital Inc, Canada
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