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Front Neurol. 2019 Feb 19;10:106. doi: 10.3389/fneur.2019.00106. eCollection 2019.

Clinical Measures of Bulbar Dysfunction in ALS.

Author information

1
Department of Speech Language Pathology, University of Toronto, Toronto, ON, Canada.
2
Hurvitz Brain Sciences Research Program, Sunnybrook Research Institute, Toronto, ON, Canada.
3
Volcal Tract Visualization Lab, Toronto Rehabilitation Institute, University Health Network, Toronto, ON, Canada.
4
Swallowing Systems Core, Department of Speech, Language, and Hearing Sciences, University of Florida, Gainesville, FL, United States.
5
Department of Communication Sciences and Disorders, MGH Institute of Health Professions, Boston, MA, United States.
6
Speech and Hearing Biosciences and Technology Program, Harvard University, Cambridge, MA, United States.
7
Division of Neurology, Department of Medicine, University of Toronto and University Health Network, Toronto, ON, Canada.
8
Institute of Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada.
9
Computational Neuroimaging Group, Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland.

Abstract

Bulbar impairment represents a hallmark feature of Amyotrophic Lateral Sclerosis (ALS) that significantly impacts survival and quality of life. Speech and swallowing dysfunction are key contributors to the clinical heterogeneity of ALS and require well-timed and carefully coordinated interventions. The accurate clinical, radiological and electrophysiological assessment of bulbar dysfunction in ALS is one of the most multidisciplinary aspects of ALS care, requiring expert input from speech-language pathologists (SLPs), neurologists, otolaryngologists, augmentative alternative communication (AAC) specialists, dieticians, and electrophysiologists-each with their own evaluation strategies and assessment tools. The need to systematically evaluate the comparative advantages and drawbacks of various bulbar assessment instruments and to develop integrated assessment protocols is increasingly recognized. In this review, we provide a comprehensive appraisal of the most commonly utilized clinical tools for assessing and monitoring bulbar dysfunction in ALS based on the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) evaluation framework. Despite a plethora of assessment tools, considerable geographical differences exist in bulbar assessment practices and individual instruments exhibit considerable limitations. The gaps identified in the literature offer unique opportunities for the optimization of existing and development of new tools both for clinical and research applications. The multicenter validation and standardization of these instruments will be essential for guideline development and best practice recommendations.

KEYWORDS:

Bulbar ALS; COSMIN; amyotrophic lateral sclerosis; dysarthria; dysphagia; outcome assessment (Health Care)

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