Clinical Measures of Bulbar Dysfunction in ALS

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.

Diagnostic utility of the amyotrophic lateral sclerosis Functional Rating Scale—Revised to detect pharyngeal dysphagia in individuals with amyotrophic lateral sclerosis

“…In addition to PROMs, voluntary cough function is noted to significantly differ in individuals with ALS compared to healthy age and gender matched controls, contributing to the impaired ability to effectively expel tracheal aspirate and manage secretions in this population [26]. Given that peak expiratory flow is noted to be reduced by 50% in ALS patients with unsafe swallowing [27], voluntary cough peak expiratory flow (commonly known as peak cough flow testing) has been suggested as a screen to index one's physiologic airway defense capacity [28,29]. Future work is needed to identify additional sensitive clinical markers in order to develop and validate a pragmatic and accurate dysphagia screening tool for use in ALS clinics [8,9,29].…”

Early detection and tracking of bulbar changes in ALS via frequent and remote speech analysis

“…The standard ways of assessing bulbar dysfunction are the ALS functional rating scale-revised (ALSFRS-R) and, less commonly, the Center for Neurologic Study Bulbar Function Scale (CNS-BFS) 8 . Both instruments, however, lack sensitivity to early bulbar changes 9 . Several studies have found that speech features, such as jitter, shimmer, articulatory rate, speaking rate, and pause rate, are affected in ALS 10 , 11 , and that these can be measured from remotely-collected speech samples 12 , 13 .…”

The Effect of a Half-Day Training Program for Students in Multiple Healthcare Disciplines on Communication Support for People with Amyotrophic Lateral Sclerosis using Pre- / Post-tests

“…Amyotrophic lateral sclerosis (ALS) is one of the cruelest neurodegenerative diseases. Upper and lower motor neuron dysfunction causes progressive loss of voluntary muscle activity, including bulbar impairment (dysarthria and dysphagia) [1,2]. According to the latest data, 9,636 ALS patients in Japan were registered in 2017 [3].…”