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Review Article

Bulbar and speech motor assessment in ALS: Challenges and future directions

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Pages 494-500 | Received 13 Dec 2012, Accepted 15 Jun 2013, Published online: 30 Jul 2013
 

Abstract

Bulbar motor deterioration due to amyotrophic lateral sclerosis (ALS) leads to the eventual impairment of speech and swallowing functions. Despite these devastating consequences, no standardized diagnostic procedure for assessing bulbar dysfunction in ALS exists and adequate objective markers of bulbar deterioration have not been identified. In this paper, we consider objective measures of speech motor function, which show promise for forming the basis of a comprehensive, quantitative bulbar motor assessment in ALS. These measures are based on the assessment of four speech subsystems: respiratory, phonatory, articulatory, and resonatory. The goal of this research is to design a non-invasive, comprehensive bulbar motor assessment instrument intended for early detection, monitoring of disease progression, and clinical trial application. Preliminary data from an ongoing study of bulbar motor decline are presented, which demonstrate the potential clinical efficacy of the speech subsystem approach.

Acknowledgements

The authors would like to thank Antje Mefferd, Cynthia Didion, Kimber Green, Michelle Falikowski, Vincci Tau, Daniele Thomas, Casey Willett, Toni Hoffer, Aaron Pattee, and Emily Foutch for their assistance with this project. They also thank Michael Jackson for illustrating .

Declaration of interest: This research was funded by an NIH-NIDCD grant, Bernice Ramsey Discovery Grant from ALS Canada, and the Barkley Trust at the University of Nebraska- Lincoln. The authors do not have financial and/or business in any company that may be affected by the research reported in this manuscript. The authors alone are responsible for the content and writing of the paper.