Provisional Best Practices Guidelines for the Evaluation of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis
Muscle & Nerve
Pattee, G. L., Plowman, E. K., et al. (2019).
Muscle & Nerve, 59(5), 531-536.
This is a consensus-based guideline of clinical recommendations in the areas of speech, swallowing, and alternative and augmentative communication (AAC) for use with individuals with amyotrophic lateral sclerosis (ALS).
Northeast Amyotrophic Lateral Sclerosis Consortium, Bulbar Committee
<p>Individuals with diagnosis of ALS should receive a speech evaluation at the initial visit. Assessment should include:</p>
<ul>
<li>a patient self-report questionnaire;</li>
<li>an oral structure and motor exam; and</li>
<li>a spontaneous speech and short reading passage sample to measure dysarthria severity, speaking rate, and involvement of one or more of the four speech subsystems (respiratory, phonatory, articulatory, and resonatory).</li>
</ul>
<p>All patients who present with an oral motor exam atypical for bulbar ALS (i.e., asymmetrical tongue or pharyngeal weakness, oral/throat pain, raspy voice, or swallow difficulties preceding speech impairment) should be referred for an otolaryngology evaluation to rule out alternative causes. Should the clinical exam identify bulbar dysfunction (i.e., findings reveal tongue weakness, dysarthria with weak cough, and/or compromised respiratory support) then an SLP referral is warranted for further comprehensive assessment.</p>
<p>A swallow screen is recommended for individuals with ALS. Initial swallowing evaluation should include objective testing in each of the following domains:</p>
<ul>
<li>patient reported outcomes; </li>
<li>dietary intake; </li>
<li>pulmonary function and airway defense physiologic capacity;</li>
<li>bulbar function; and </li>
<li>a dysphagia/aspiration screen. </li>
</ul>
<p>Individuals presenting with markers of dysfunction (e.g., failed screening measure) should receive a comprehensive swallowing evaluation, which may also include an instrumental assessment of swallowing function.</p>
<p>Due to the degenerative nature of speech in individuals with ALS, an AAC evaluation is recommended at the time of diagnosis, regardless of whether a speech impairment exists. Assessment should consider:</p>
<ul>
<li>communication participation across a variety of settings and situations;</li>
<li>voice banking;</li>
<li>use of low-tech communication strategies; and</li>
<li>identification of additional communication supports as needed.</li>
</ul>
