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Management of Dysphagia in Individuals With Amyotrophic

Lateral Sclerosis
Beau Bedore
Minneapolis VA Health Care System
Spinal Cord Injury & Disorder Center
Minneapolis, MN
Disclosure: Beau Bedore has no financial or nonfinancial relationships related to the content of
this article.
This article presents a comprehensive approach to the evaluation and treatment of
dysphagia in individuals with amyotrophic lateral sclerosis (ALS). The author considers
the role of therapeutic exercise in ALS in the context of dysphagia rehabilitation.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving
the motor neurons of both the brain and spinal cord (Yorkston, Miller, & Strand, 2004). The
disease is classified into three types (Kawai et al., 2003) according to the region of onset of
initial symptoms (Ball, Beukelman, & Bardach, 2007): bulbar (brainstem nerves), spinal
(nerves of the spinal cord), and mixed (a combination of brainstem and spinal nerves). Ball et
al. (2007) describe the principle symptoms in the early stage that distinguish the three types:
The bulbar type is the most rapidly progressive form of ALS and involves the speech and
swallowing musculature controlled by the cranial nerves. The spinal type progresses more
slowly than the bulbar form and involves the upper and lower extremities controlled by the
spinal nerves. Finally, the mixed type includes symptoms of both bulbar and spinal forms of
the disease and progresses at a rate that is determined by the predominant bulbar or spinal
symptoms. Classification of type is an important consideration for the evaluation and
treatment of dysphagia in individuals with ALS because of the variability in the clinical
courses; the time of onset of dysphagia will differ greatly among these three types (Kawai et al.,
2003).
Clinical staging of ALS is another important consideration and is used to describe the
presentation and progression of symptoms. The ALS Severity Scale (ALSSS) is an ordinal
staging system used to rate function in four areas: speech, swallowing, lower extremity, and
upper extremity (Hillel et al., 1989). Each of the four areas has ten possible rating scores
(subscales) based on the progressive decline of function in one specific area. Using this scale,
clinicians can describe bulbar function by adding the scores for the Speech Subscale and
Swallowing Subscale, and describe spinal function by adding the scores for the Lower
Extremity Subscale and Upper Extremity Subscale. Because the ALSSS is ordinal, the
progression of symptoms is built into the scale. Hillel et al. (1989) propose that the ALSSS
“provides a succinct numerical indication of a patient’s functional status and is useful for
indicating a ‘stage’ of disease” (p. 149). Yorkston et al. (2004) describe a five-stage model for
swallowing management in ALS based on the headings of the Swallowing Subscale:
• Stage 1: Normal Eating Habits (includes “Normal Swallowing”—a score of 10, and
“Nominal Abnormality”—a score of 9)
• Stage 2: Early Eating Problems (includes “Minor Swallowing Problems”—a score of
8, and “Prolonged Time or Smaller Bite Size”—a score of 7)

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 • Stage 3: Dietary Consistency Changes (includes “Soft Diet”—a score of 6, and
“Liquefied Diet”—a score of 5)
• Stage 4: Needs Tube Feeding (includes “Supplemental Tube Feedings”—a score of 4,
and “Tube Feeding With Occasional PO Nutrition”—a score of 3)
• Stage 5: Nothing by Mouth (includes “Secretions Managed With
Aspirator/Medication”—a score of 2, and “Aspiration of Secretions”—a score of 1)
This staging model provides a valuable framework for clinical decision-making in the
management of dysphagia. Although the rate of symptom progression in ALS is variable, the
sequence of symptom appearance follows a predictable course as swallowing function declines
with disease progression. Thus, it is necessary to understand the sequence of symptom
appearance and anticipate the predictable changes in swallowing function that characterize a
transition from one stage of the disease to the next.
Dysphagia is a common and serious symptom for individuals with ALS (Leder, Novella,
& Patwa, 2004) and is caused by degeneration of cranial nerves V, VII, IX, X, XI, and XII. Since
function for both speech and swallowing rely on the same muscles, swallowing difficulties
generally parallel speech difficulties (Yorkston et al., 2004). Evaluation of dysphagia in
individuals with ALS is based on both clinical and instrumental assessment. The Clinical
Examination for ALS (Yorkston et al., 2004) is a standardized assessment tool that organizes
the clinical evaluation into three general areas: history, physical examination, and assessment
of speech and swallowing function. Taking the individual’s history involves gathering
information about the nature and course of the disorder, and how the disorder is being
managed. Given the variability in the clinical courses among the three types of ALS, it is
important for clinicians to identify the initial symptoms that led to the diagnosis, as well as the
presence or absence of dysphagia symptoms, which characterize the bulbar form of the
disease, including coughing, choking, or throat clearing with eating; weight loss; resistance to
eating or going out for meals; pain or discomfort while eating; loss of pleasure with eating;
extra effort required when swallowing liquids, solids, or pills; sensation of food sticking in the
throat; and stress associated with swallowing. A discussion of advance directives for medical
decisions (i.e., establishment of a living will; identification of a medical or health-care power of
attorney [POA]; request for a do not resuscitate [DNR] order or a do not intubate [DNI] order) is
also a relevant aspect of taking the individual’s history given the terminal prognosis for
patients with ALS. This discussion pertains to the patient’s current health-care management
and preferences regarding end-of-life care. Yorkston et al. (2004) organize the physical
examination into groups according to components of the speech and swallowing mechanisms:
Group 1: Tongue and lips; Group 2: Palate, pharyngeal constrictors, and muscles of
mastication; Group 3: Buccal/frontal/orbital muscles, sternocleidomastoid/trapezius muscle,
and the vocal folds; and Group 4: Muscles of respiration. During the physical examination, the
clinician documents impairment or physiologic abnormalities observed in any of the four
groups (which would indicate cranial nerve involvement and identify early bulbar signs).
Assessment of speech and swallowing function is the final area to be evaluated. This phase of
the clinical examination is focused on the individual’s ability to maintain adequate nutrition
and hydration, handle oral secretions, and tolerate food and liquid without evidence of overt
signs of aspiration. The clinician observes and documents difficulty managing saliva, oral stage
and pharyngeal stage problems (e.g., leakage from mouth, difficulty chewing, difficulty
manipulating the bolus, pocketing of food, difficulty initiating a swallow, nasal regurgitation,
coughing after swallow, voice change after swallow), and general complications related to
feeding and oral intake.
The clinical exam may also include the Amyotrophic Lateral Sclerosis Functional Rating
Scale (ALSFRS; Brooks et al., 1996), a validated questionnaire-based scale that measures
physical function in carrying out activities of daily living (ADLs) of individuals with ALS. The
original version of this scale covers 10 areas, including assessment of speech, salivation, and
swallowing. Speech-language pathologists are responsible for scoring these three areas.
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 Swallowing function on the ALSFRS is scored on a 5-point ordinal scale, which is adopted from
the Swallowing Subscale of the ALSSS. Respiratory function is also a critical concern in the
management of dysphagia for this population. Because respiration is momentarily interrupted
during the pharyngeal phase of swallowing, individuals with respiratory insufficiency may
experience fatigue with eating that is unrelated to oral or pharyngeal weakness (Yorkston et al.,
2004). In addition, increased respiratory rate and work of breathing can result in airway
compromise, leading to aspiration. The Revised ALSFRS (ALSFRS-R) incorporates additional
assessments of dyspnea (shortness of breath), orthopnea (shortness of breath that occurs while
lying down), and respiratory insufficiency (the need for ventilatory support; Cedarbaum et al.,
1999). The ALSFRS-R is a commonly used tool in specialized ALS centers and multidisciplinary
clinics and can be utilized as a screen for dysphagia, as well as an indicator of the need for
further evaluation and intervention (Kidney, Alexander, Corr, O’Toole, & Hardiman, 2004). It
also provides reliability across sites and is a frequently used outcome measure (Dal Bello-Haas
et al., 2007; Pinto, Swash, & Carvalho, 2012). Therefore, it is important for clinicians
evaluating and treating individuals with ALS to become familiar with this tool.
Instrumental assessment of dysphagia in individuals with ALS can involve both
videofluoroscopic evaluation of swallowing (a modified barium swallow study) and fiberoptic
endoscopic evaluation of swallowing (FEES). Yorkston and colleagues (2004) indicate that
videofluoroscopic evaluations “are used to evaluate swallowing in individuals with symptoms
that cannot be explained on the basis of the physical examination” (p. 31). In other words,
clinical swallowing evaluations for this population typically identify symptoms of dysphagia
that can be explained by the previously established diagnosis of ALS. If the clinical swallowing
evaluation reveals findings that do not match this constellation of symptoms, or the
individual’s complaints and history suggest multiple problems, videofluoroscopic evaluations
are recommended. For example, in individuals with spinal ALS, no abnormality is usually
observed at the esophageal phase of swallowing because smooth muscles are not damaged in
the disease (Kawai et al., 2003). However, the upper third of the esophagus is striated muscle
(controlled by the vagus nerve); consequently, individuals with bulbar ALS may experience
esophageal stage problems. Patients may exhibit or endorse symptoms consistent with
cricopharyngeal dysfunction, esophageal webs, diverticuli, esophageal dysmotility, or
gastroesophageal reflux. In such cases, Yorkston et al. (2004) recognize the benefit of
videofluoroscopic evaluation; however, they also remind clinicians that “because aspiration is
evident on the basis of the physical examination and history, radiographic studies generally
should not be used for the detection of aspiration alone” (p. 31). Despite the fact that
videofluoroscopic evaluation is objective and used widely, it may not be the most appropriate
technique to use for instrumental assessment of dysphagia in this population.
FEES is a viable alternative to videofluoroscopic assessment and has unique
advantages for evaluating swallowing function in individuals with ALS (Leder et al., 2004).
FEES allows for preswallow assessment of both pharyngeal and laryngeal anatomy and
physiology and provides evidence of pooled secretions in the pharynx, larynx, or trachea
(Murray, Langmore, Ginsberg, & Dostie, 1996). Moreover, FEES is an objective technique that
can be easily repeated. The procedure can be performed in the clinic or at bedside, permits the
use of regular food, and does not require a time limit—which allows clinicians to assess for
fatigue during meals. Another advantage is that FEES eliminates transportation and
positioning problems in the fluoroscopy suite and avoids radiation exposure and ingestion of
barium sulfate. For individuals with ALS, FEES succeeds “in assessing preswallow anatomy
and physiology, diagnosing pharyngeal dysphagia, and providing objective data for appropriate
therapeutic interventions to promote safer oral intake and maintain optimum quality of life”
(Leder et al., 2004, p. 180). The technique lends itself to repeat instrumental evaluations (serial
FEES) and is an effective way to monitor dysphagia symptoms throughout the course of the
disease. Ultimately, the choice to use videofluoroscopic evaluation or FEES is a clinical
decision and should be based upon the patient’s history and symptoms.

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 Treatment of dysphagia in ALS is primarily focused on managing symptoms based on
the five-stage model of disease progression (Yorkston et al., 2004). In the early stages of ALS,
when swallowing habits are normal, no intervention is needed. Management during this stage
involves education. As the individual develops occasional problems with eating and drinking,
intervention involves behavioral compensations (eating smaller, more frequent meals
throughout the day; crushing or taking pills in pudding or yogurt; taking smaller bites and
sips; minimizing distractions and conversation during meals; maintaining concentration while
eating and drinking), postural adjustments (chin-tuck posture, head rotation), and swallowing
maneuvers (effortful swallow, supraglottic swallow; Ashford et al., 2009). At this stage,
Yorkston et al. (2004) also recommend providing information on first aid for choking. The next
stage of progression is signaled by increased difficulty with eating and drinking. As the
swallowing problems worsen, the individual begins to need special meal preparation.
Intervention at this stage is focused on dietary consistency changes, including modification of
food texture and liquid viscosity. Further intervention during this stage is based on visual
biofeedback provided by FEES, which is useful “for both patient and family education and to
investigate individualized therapeutic strategies that, if successful, can be implemented
immediately” (Leder et al., 2004, p. 180).
The next stage of decline is marked by an inability to maintain adequate nutrition and
hydration despite dietary modifications and consumption of calorie-dense foods. At this point,
oral intake may no longer be safe or swallowing may require too much effort. Feeding tube
placement is then discussed as a possible intervention. This option is also considered in the
context of respiratory status, with recommendations for placement of a feeding tube before vital
capacity falls to 50% of predicted values (Hardiman, 2000). The final stage of dysphagia in ALS
is reached when the individual can no longer safely manage oral intake. In addition, special
consideration must be given to the most advanced individuals who cannot manage their own
secretions (Yorkston et al., 2004). Intervention at this stage often involves the use of a portable
suction machine or aspirator, as well as medications to manage saliva. A towel or bib can also
be used to absorb secretions. If necessary, radiation of the parotid and submandibular glands
can significantly reduce drooling (Andersen, Gronberg, Franzen, & Funegard, 2001). In some
cases, aspiration of secretions may require surgical management: Patients will often elect for
tracheostomy if the management of saliva requires tracheal suctioning in order to maintain
adequate respiratory function. Though tracheostomy does not prevent aspiration, it “does
provide excellent access for pulmonary toilet” (Yorkston et al., p. 64).
One of the most frequently asked questions by individuals with ALS is whether exercise
is beneficial. Historically, general strengthening and conditioning exercises have not been
recommended as the standard of care for persons with ALS. Early studies warn that persons
with ALS should conserve energy and avoid exercise other than the exercise inherent in
everyday ambulatory activities. Consequently, exercise has been thought to accelerate the
progression of skeletal muscle weakness, the cardinal sign and symptom of motor neuron
degeneration in ALS (Chen, Montes, & Mitsumoto, 2008). However, Lopes de Almeida, Silvestre,
Pinto, and de Carvalho (2012) point out that no controlled studies have documented that
overuse weakness actually occurs. In fact, there is a growing body of literature in physical
therapy to suggest that moderate exercise is not associated with adverse outcomes in
individuals with early-stage ALS (Chen et al., 2008). On the other hand, several systematic
reviews (Cup et al., 2007; Dal Bello-Haas, Florence, & Krivickas, 2008; Lui & Byl, 2009) of this
same body of literature conclude that there is insufficient evidence for the effectiveness of
muscle strengthening exercises for individuals with ALS. Dal Bello-Haas et al. (2008) caution
that the studies (Drory, Goltsman, Reznik, Mosek, & Korczyn, 2001; Dal Bello-Haas et al.,
2007) are too small to determine to what extent exercise for people with ALS “is beneficial or
harmful” (p. 8). Lui and Byl (2009) are more confident that “strengthening and cardiovascular
exercises may help maintain function and do not adversely affect disease progression in
persons with ALS”; notably, they acknowledge that “the current evidence is not sufficiently
detailed to recommend specific exercise guidelines or outline a specific exercise prescription for
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 persons with ALS” (p. 85). Regardless of these differences, both groups of investigators agree
that more research is needed.
In light of this discussion, it is easy to understand why rehabilitation of dysphagia in
ALS has traditionally been discouraged. Nevertheless, while there is insufficient evidence from
these studies to recommend specific exercise guidelines for clinical practice, researchers
remain interested in the potential use of exercise in ALS to prevent deconditioning and disuse
weakness beyond the amount caused by the disease itself (Dal Bello-Haas et al., 2008). This
idea is in line with the “use it and improve it” principle of neural plasticity (Kleim & Jones,
2008) and has been described in the context of swallowing and dysphagia rehabilitation by
Robbins et al. (2008). Researchers are optimistic that moderate exercise, when prescribed
appropriately, may be physically and psychologically important for individuals with ALS,
especially in the earlier stages of the disease and before significant muscular atrophy or
deconditioning occurs (Dal Bello-Haas et al., 2008). Still, the question remains, what is the
appropriate exercise prescription? At this time, there is no clear answer. There have not been
any published studies to date on ALS and the effect of swallowing exercises (e.g., lingual
exercise, Mendelsohn maneuver, head-lifting exercise [Shaker exercise], tongue-hold exercise
[Masako exercise]). But while rehabilitation of dysphagia in this population has always been
rejected as harmful, future research may prove otherwise. In the meantime, the primary aim of
dysphagia intervention for individuals with ALS is to reduce the risk of aspiration and improve
swallow function for safe and efficient oral intake (Ashford et al., 2009).
Comments/questions about this article? Visit our ASHA Community and join the discussion!

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