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DYSPHAGIA ASSESSMENT AND TREATMENT PLANNING: A TEAM APPROACH
Vela, M. F., Richter, J. E., Wachsberger, D.,
Connor, J., & Rice, T. W. (2004). Complexities of managing achalasia at a tertiary referral center: Use of pneumatic
dilatation, heller myotomy, and botulinum toxin injection. American Journal of
Gastroenterology, 99(6), 1029–1036. Scopus. https://doi.or g/10.1111/j.1572- 0241
.2004.30199.x
Wetscher, G. J., Glaser, K., Wieschemeyer,
T., Gadenstaetter, M., Prommegger, R., &
Profanter, C. (1997). Tailored antireflux
surgery for gastroesophageal reflux disease: Effectiveness and risk of postoperative dysphagia. World Journal of Surgery,
21(6), 605–610. https://doi.org/10.1007/
s002689900280
Winter, H. S., Madara, J. L., Stafford, R. J.,
Grand, R. J., Quinlan, J. E., & Goldman,
H. (1982). Intraepithelial eosinophils:
A new diagnostic criterion for reflux
esophagitis. Gastroenterology, 83(4), 818–
823.
Xiao, Y., Kahrilas, P. J., Nicodème, F., Lin,
Z., Roman, S., & Pandolfino, J. E. (2014).
Lack of correlation between HRM metrics and symptoms during the manometric protocol. American Journal of Gastro-
enterology, 109(4), 521. https://doi.org/
.1038/ajg.2014.13
Yadlapati, R., Kahrilas, P. J., Fox, M. R.,
Bredenoord, A. J., Gyawali, C. P., Roman,
S., . . . Pandolfino, J. E. (2021). Esophageal motility disorders on high resolution manometry: Chicago Classification
version 4.0. Neurogastroenterology and
Motility, 33(1), e14058. https://doi.org/
10 .1111/nmo.14058
10
Neurogenic Dysphagia
https://t.me/medicina_free
Jacqui E. Allen
Neurogenic disorders are among the
most common etiologies responsible
for swallowing dysfunction. A heterogeneous group of pathologies must be
considered that span acute-onset central disorders such as stroke; progressive, insidious disorders such as amyotrophic lateral sclerosis (ALS); and
peripheral neuromyogenic dysfunction as seen in inflammatory myositis
(Table 16–1). The effects of neuromuscular dysfunction may also manifest in
a variety of ways, including end-organ
weakness or failure, incoordination
of gestures, or total failure of central
patterning. The underlying etiology
determines which aspect of deglutition is most prominently affected and
therefore which therapies may be
effective in rehabilitating swallow or
preventing complications of dysphagia. More than three quarters of cases
of oropharyngeal dysphagia are due
to neurological disorders (Diniz et al.,
2009; White et al., 2008). Estimates of
prevalence of swallow complaints are
65% in acute stroke, at least 50% in
Parkinson’s disease (PD) and rises as
duration of disease increases, around
30% in multiple sclerosis (MS), 30% to
100% in motor neuron disease (MND),
and somewhere between 13% and 60%
in dementia (Panebianco et al., 2020).
Aspiration pneumonia, a consequence
of swallow impairment, is a common
cause of mortality in all neurogenic diseases. To offer the best diagnostic and
treatment options to patients, we must
understand basic pathophysiological
mechanisms in these disorders and the
most vulnerable portions of deglutition
in each case.
This chapter will briefly present the
most common neuromyogenic disorders related to dysphagia, characteristics of swallow impairments, and videofluoroscopic findings specific to each.
A brief discussion regarding optimum
assessment of the patient (crucial in
making the correct diagnosis and formulating a management strategy) is
included (Miles & Allen, 2015).
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DYSPHAGIA ASSESSMENT AND TREATMENT PLANNING: A TEAM APPROACH
Table 16 –1. Etiology of Neurogenic Dysphagia
CENTRAL PERIPHERAL
Nondegenerative Degenerative Neuromuscular Myopathy
Cerebrovascular
Accident
Brain Injury
Medication
Neoplasm
Congenital
cerebral palsy,
syringobulbia,
Arnold-Chiari
malformation
—
Dementia
Alzheimer’s
disease,
multi-infarct
Parkinson’s
disease
Multiple sclerosis
Huntington’s
disease
Supranuclear
palsy
—
ASSESSMENT
The test battery approach in a multidisciplinary environment offers the best
diagnostic accuracy and will provide
the most useful information regarding
diagnosis, prognosis, interventions,
compensatory strategies, and dietary
safety. Multiple methods are available
to assess the dysphagic patient, and
each provides complementary information that can be crucial to treatment
planning in a team setting (Rugiu,
2007). Many of these strategies are discussed in this book.
A thorough clinical evaluation by
a trained swallowing professional
such as a speech pathologist is the
first step. While this is invaluable in
identifying struggling patients and
developing rapport, multiple studies
have demonstrated that patients with
Myasthenia gravis
Polio, Post-polio
syndrome
Amyotrophic
lateral sclerosis
Neuropathy Iatrogenic
Guillain-Barré
syndrome
Muscular
dystrophies:
Oculopharyngeal
muscular
dystrophy (OPMD),
Myotonic (MD),
Duchenne (DMD)
Polymyositis,
Dermatomyositis,
Inclusion body
myositis
Post surgery or
radiotherapy of
the head and
neck
neuromyogenic conditions frequently
present with silent aspiration and cannot, by definition, be identified by
bedside evaluation (Diniz et al., 2009;
González-Fernández et al., 2008; Kang
et al., 2011; Ramsey et al., 2003; Rugiu,
2007; Smithard, 2016; White et al., 2008).
Sensitivity of the bedside examination
ranges from 40% to 80% and specificity ranges from 59% to 91% (GonzálezFernández et al., 2008; Miles et al.,
2013; Ramsey et al., 2003; Rugiu, 2007;
Smithard, 2016). Cough reflex testing has been studied as an adjunct to
bedside examinations, with the aim of
improving detection of silent aspirators
(Miles et al., 2013). It may assist in identifying subjects requiring a comprehensive instrumental examination. Additional adjunctive measures have been
proposed such as measuring oxygen
saturations or cervical auscultation, so
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far without significant improvement in
the rate of detection of silent aspirators
(Marian et al., 2017; Smithard, 2016).
Instrumental examination is invaluable for detecting silent aspiration and
most commonly consists of a dynamic
videofluoroscopic swallowing study
(VFSS), also called a dynamic swallow
study (DSS). The dynamic swallow
study (Chapters 6–8) provides objective
and quantitative data that can elaborate
the nature and severity of a swallowing
problem, provide insights into prognosis in individual patients, and be used
to develop remedial treatment programs. It has been considered the “gold
standard” in instrumental assessment
(Carbo et al., 2021; González-Fernández
et al., 2008; Kang et al., 2011; Ney et al.,
2009; Rugiu, 2007). However, this radiographic study is two-dimensional and
requires exposure to ionizing radiation
(approximately that of two cervical
spine x-rays), and specialized equipment and personnel are not always
available to the assessing clinician.
Consequently, the use and timing of the
DSS must be considered with respect to
minimizing both diagnostic costs and
x-ray exposure, particularly in patient
populations that experience frequent
and significant change, sometimes
rapid and for the better, sometimes prolonged and for the worse.
An instrumental flexible endoscopic
evaluation of swallowing (FEES; Chapter 4) may also identify aspiration,
provide distinct laryngopharyngeal
anatomical information that may complement the DSS, be performed at the
bedside (useful in those patients unable
to be transported to a radiology suite),
and avoid the consequent risks of radiation exposure (most pertinent in young
or multiply injured patients). Informa-
tion from FEES is complementary to
videofluoroscopic study information,
and both studies may be useful in any
given patient (González-Fernández
etal., 2008; Rugiu, 2007).
High-resolution pharyngoesophageal manometry is an expanding diagnostic tool that may also be employed
for biofeedback purposes. It measures
the pressures along the aerodigestive
tract, from velum to stomach and, if
combined with impedence recording,
can also demonstrate where the bolus
is situated within the pharyngoesophageal lumen, through alterations in resistance (Panebianco, 2020).
Electromyography can be employed
to delineate specific muscle activity
from differing areas involved with swallowing, including masticatory muscles,
tongue, floor of mouth, anterior cervical area, and the upper esophageal
sphincter (Panebianco, 2020). A battery
of diagnostic strategies that permit the
most efficacious collection of information in a practical and safe way should
be the aim when assessing swallowing
problems in neurogenic patients.
CENTRAL NERVOUS
SYSTEM DISORDERS
A selection of common neuropathic
conditions affecting deglutition will be
discussed.
Cerebrovascular
Accident (CVA)
Dysphagia is found in 29% to 80% of
those presenting with an acute stroke
(Diniz et al., 2009; Garon et al., 2009;
González-Fernández et al., 2008; Kägi
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DYSPHAGIA ASSESSMENT AND TREATMENT PLANNING: A TEAM APPROACH
et al., 2016; Kang et al., 2011; Kumar
et al., 2010; Ney et al., 2009; Rugiu,
2007; Simons & Hamdy, 2017; Smithard,
2016). In many cases (50%–90%), rapid
improvement may be expected over
the short to medium term, but persistent dysphagia is seen in approximately 20% to 50% of patients (Diniz
etal., 2009; Garon et al., 2009; GonzálezFernández et al., 2008; Kägi et al., 2016;
Rugiu, 2007; Smithard, 2016; White
et al., 2008). More than half of those
with acute stroke develop aspiration
and 35% develop aspiration pneumonia (Ney et al., 2009; Panebiano et al.,
2020). Even a year later, 15% to 20% of
post-CVA patients develop pneumonia
and almost half of CVA patients are
malnourished (Ney et al., 2009).
Swallowing function has bilateral
cortical representation with interconnecting crossed pathways at the level
of the brainstem (González-Fernández
et al., 2008; Panebianco et al., 2020;
White et al., 2008). Medullary or pontine strokes result in severe dysphagia
due to both sensory and motor disruption (Flowers et al., 2017; Jones et al.,
2020). Injury involving the right primary sensory cortex, right insula, left
and right motor cortices, and midbrain
structures including the internal capsule, thalamus, basal ganglia, and cerebral peduncles may also affect deglutition and are increasingly delineated by
a magnetic resonance imaging (MRI)
scan (Dehaghani et al., 2016; Flowers
et al., 2017). Dysphagia is therefore a
prominent feature of brainstem and both
right and left cortical insults (Jones et al.,
2020; Kumar et al., 2010; Ney et al., 2009;
Paliwal et al., 2009) and affects the oral
and oropharyngeal phases of deglutition
(Donner et al., 1985; Rugiu, 2007).
Sensory deficits (particularly if they
affect the pharynx) also lead to significant dysfunction and may be a cause
of silent aspiration. Therefore, injury at
many sites can result in swallowing difficulties. Furthermore, older adults who
typically suffer from stroke may harbor preexisting swallow impairments
or physiological decline in swallow
reserve (presbyphagia), which is then
unmasked or exacerbated following
another insult such as stroke (Smithard,
2016). As the picture changes over time,
frequent reassessment is crucial (Kägi
et al., 2016; Smithard, 2016).
Dysphagia after stroke is associated with development of aspiration
pneumonia (3- to 11-fold increased
risk) (Altman et al., 2010; Feng et al.,
2019; González-Fernández et al., 2008;
Kumar et al., 2010; Ney et al., 2009;
Rugiu, 2007; Smithard, 2016; White
etal., 2008). Silent aspiration is common after stroke (2%–66%) and may
manifest only as recurrent pulmonary
complications (González-Fernández
et al., 2008; Ney et al., 2009; Ramsey et
al., 2003, 2005; Rugiu, 2007). Aspiration
pneumonia is the most common cause
of rehospitalization in acute stroke
patients and contributes to more than
50% of post-CVA deaths in the first
30days (Ney et al., 2009). In fact, aspiration pneumonia is the leading cause of
death poststroke, and reduced oxygenation resulting from pneumonia may
exacerbate neurological injury and slow
recovery (Kang et al., 2011; Vilardell
etal., 2017; White et al., 2008). In those
with dysphagia following stroke, there
is an adjusted hazard ratio of 1.84 for
death within 5 years, compared to those
without dysphagia (Feng et al., 2019).
Because stroke also results in depres-
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sion of the immune system, aspiration
occurring post-CVA may be less well
tolerated, increasing the rate of aspiration pneumonia. It is estimated that
5% to 15% of community-acquired
pneumonia is caused by aspiration
(White et al., 2008). Silent aspiration (by
definition) is not detected by bedside
evaluation and therefore a high index
of suspicion is required to avoid missing the diagnosis. Independent factors
associated with greater risk of poor
swallowing after stroke include older
age, malnutrition at admission, larger
lesion volume, brainstem involvement,
corticobulbar involvement, white matter involvement, presence of dysarthria or dysphagia, cognitive impairment, and reduction in functional scale
scores (e.g., Rankin Scale, Barthel index
or National Institutes of Health Stroke
Scale) (Jones et al., 2020).
Due to the high prevalence of dysphagia in stroke patients, screening for
swallowing problems should be undertaken in all patients, even those with
no overt signs of dysphagia. Initially, a
bedside assessment by a speech pathologist or trained clinician should be performed. To improve sensitivity to swallow impairment, addition of cervical
auscultation, measures of oxygen saturation, or cough reflex testing has been
proposed (Jones et al., 2020; Marian
etal., 2017; Miles et al., 2013; Smithard,
2016; Vilardell et al., 2017). None of
these methods is sensitive enough to
reliably detect silent aspiration. In most
cases involving hemispheric or brainstem sites, an instrumental evaluation
is indicated due to the high rate of silent
aspiration (González-Fernández et al.,
2008; Jones et al., 2020; Ney et al., 2009;
Ramsey et al., 2003, 2005; Rugiu, 2007).
Patients presenting with additional
risk factors for aspiration (based on
site of lesion, comorbidities, age, pulmonary health, head and neck cancer,
dementia, cervical spine abnormalities) should be identified and should
also undergo instrumental evaluation
regardless of findings of bedside or
clinical evaluations.
Instrumental examination may consist of videofluoroscopy (DSS), endoscopy (FEES), or both. DSS and FEES
both demonstrate high sensitivity and
specificity in identifying aspiration
(>85%) (Ramsey et al., 2005; Rugiu,
2007). The DSS will also demonstrate
pharyngoesophageal and esophageal
phase abnormalities. Fluoroscopic studies help delineate the mechanism of
dysphagia, allow assessment of compensatory maneuvers, and contribute
to planning of safe swallow strategies.
Visualization of the vocal folds is not
possible with DSS, though mobility
may be appreciated with a voicing task
performed in the anterior-posterior
view. FEES permits ready examination of the vocal folds and may allow
assessment of asymmetry (e.g., unilateral weakness, sensory change, or
obstruction) and will assist in tailoring
compensatory strategies.
High-resolution pharyngeal manometry (HRPM) provides information
regarding pharyngeal pressure generation, and those with poststroke dysphagia demonstrate decreased pharyngeal
propulsive pressures and abnormal
upper esophageal sphincter nadir pressures. In addition, HRPM has identified
pharyngeal missequencing (simultaneous pharyngeal pressure generation)
as characteristic of poststroke swallow
behavior (Jones et al., 2020). Therefore,
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DYSPHAGIA ASSESSMENT AND TREATMENT PLANNING: A TEAM APPROACH
information obtained from these studies is complementary, and often one or
more are required to devise the best
treatment strategy.
Clinical factors that suggest the
need for evaluation with a DSS include
(a) aspiration pneumonia; (b) cough,
“wet voice,” and “wet lung” following swallowing; and (c) inability to
maintain oral hydration and nutrition.
The DSS is of value in identifying the
presence of aspiration, and the effects
of various remedial strategies, even
with no quantitative assessment. Our
experience, however, suggests that the
extra time and expense associated with
obtaining objective displacement and
timing measures can contribute significantly to patient care. Our research has
demonstrated that a pharyngeal transit
time of greater than 5 seconds (normal
1.00 ± 0.15 seconds) is significantly
associated with aspiration pneumonia
risk in stroke patients, while a time of
less than 2 seconds has a low association (Johnson & McKenzie, 1993; Johnson et al., 1992).
Timing of Evaluations
Following a CVA, a patient’s symptoms
may undergo rapid change. Timing of
evaluations and frequent reassessment
are therefore critical. Early speech pathology (SP) assessment should follow
initial screening tests (usually within
3 days of admission), to evaluate the
patient’s ability to handle oral intake
and to provide a baseline for consistent follow-up assessment. Thereafter,
additional SP input may vary — however, repeated evaluations improve
patient outcomes. Reassessment and
education around rehabilitative strategies reduce the number of days with-
out oral intake (Nakazora et al., 2017).
In many cases of acute stroke, dysphagia improves rapidly. Factors that suggest a prolonged period of dysphagia
(>2 weeks) include failure to cleanly
swallow 50mL of water, modified Barthel Index <20, dysphasia, insular or
frontal cortex involvement, and Parramatta Hospitals’ Dysphagia Index
Score <70 (Vilardell et al., 2017; White
et al., 2008).
As the DSS exposes the patient to
radiation, one must be careful to utilize
the study at the appropriate time and
maximize the amount of information
obtained from each study (Chau et al.,
2009). Early fluoroscopic examination
may be necessary for identification
of silent aspiration and pharyngeal
residue or suspected cricopharyngeal
or esophageal problems. Subsequent
examinations may be scheduled after
a therapeutic trial, when significant
motor improvements have been noted
(or deterioration seen) or when sensory changes have occurred. FEES
examinations may be performed more
frequently if trained staff are available,
as this does not expose the patient to
radiation. FEES also offers the possibility of visual feedback to the patient,
which can be of great value in teaching
compensatory strategies, if a viewing
screen is utilized. Furthermore, biofeedback techniques utilizing manometry can be selected to “train” an individual to reach a target or implement
an action and focus on skill training as
opposed to simply increasing strength
(Jones etal., 2020).
DSS Findings
Stroke often affects the nucleus ambiguus in the brainstem, which provides
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motor output to the pharynx and
esophagus, resulting in pharyngoesophageal abnormalities. Pharyngeal
weakness, prolonged pharyngeal transit time, aspiration, cricopharyngeal
dysfunction, and esophageal dysmotility are common findings (Jones et al.,
2020; Rugiu, 2007). Tongue dysfunction may be noted if the hypoglossal
nucleus, situated more caudally in the
brainstem, has been involved. Cortical strokes may produce facial asymmetry and weakness that can result in
oral incompetence and poor oral bolus
control. This may manifest with significant oral and pharyngeal residue on
DSS, which increases the risk of aspiration (Johnson et al., 1992; Rugiu, 2007;
Schimmel et al., 2017). Johnson et al.
(1992) demonstrated that prolongation
of pharyngeal transit times over 1 second was associated with a significant
increase in airway penetration and
aspiration and with occurrence of aspiration pneumonia. This was also the
finding in Kang et al.’s (2011) study of
39 post-CVA patients. Loss of sensory
function within the oropharynx and
airway will also impair swallow by
reducing bolus awareness and airway
protection. Penetration and aspiration
may occur without a response (e.g.,
silent aspiration), or the cough response
may only occur once material reaches
the carina.
Summary
CVA is a leading cause of oropharyngeal and esophageal dysphagia.
Dysphagia may be short-lived, but in
approximately 50% of patients, it will
be prolonged >2 weeks. Dysphagia is
the primary risk factor for aspiration
pneumonia, which is the leading cause
of death after stroke. Aspiration may
be silent in up to two thirds of patients
after stroke. Instrumental evaluation
is therefore critical in assessing these
patients. DSS provides reliable information about the swallow from oral cavity
to stomach. It may be complemented by
information from other instrumental
evaluations. The DSS provides accurate quantitative data that can be used
to formulate treatment and rehabilitative strategies and demonstrate change
over time. Thoughtful performance of
DSS and careful poststudy analysis will
maximize the benefit obtained from fluoroscopic evaluation.
Parkinson’s Disease
Dysphagia is common in patients suffering from Parkinson’s disease (PD),
a progressive motor system disease
caused by failed central dopamine production (Merola et al., 2011). Histopathological studies demonstrate degeneration of pharyngeal motor nerves,
indicating that oropharyngeal dysphagia in PD occurs from direct involvement of motor supply to pharyngeal
musculature as well as lack of central
dopamine (Mu et al., 2013). Braak and
Del Tredici (2003) proposed that PD
develops from peripheral insults that
propagate centrally, and studies of axonal movement of α-synuclein support
this proposition. Other theories include
mitochondrial dysfunction resulting in
generation of damaging reactive oxygen species and inflammation in the
brain that is driven by cytotoxic T cells
and microglia (Moradi Vastegani et al.,
2023; Sohrabi et al., 2023).
Along with voice deterioration, dysphagia is often one of the presenting
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DYSPHAGIA ASSESSMENT AND TREATMENT PLANNING: A TEAM APPROACH
symptoms of PD. Altman et al. (2010)
demonstrated that admission to hospital with a symptom of dysphagia
was significantly associated with Parkinson’s disease (relative risk = 4.5)
compared with age- and sex-matched
patients admitted without dysphagia. Prevalence of dysphagia in PD is
estimated to be between 32% and 70%
but increases with disease severity and
duration, and it remains underreported
when compared with the objective
prevalence rate (80%) (Kalf et al., 2012;
Luchesi et al., 2015; Merola et al., 2011).
Despite a high incidence of dysphagia, PD sufferers may not complain of
swallow problems even with demonstrable objective evidence of dysfunction (Cosentino et al., 2022; Kalf et al.,
2012; Luchesi et al., 2015). Swallow dysfunction remains the leading cause of
mortality in PD patients secondary to
aspiration pneumonia (Cosentino et al.,
2022; Hobson & Meara, 2018; Kalf et al.,
2012; Kulisevsky et al., 2018; Nicaretta
et al., 2013; Videira & Castro-Caldas,
2018). Rodrigues et al. (2011) identified silent aspiration of saliva on FEES
exam in 10% (penetration in 29%) of PD
patients, with a reduction in laryngeal
sensitivity in 90% of tested individuals.
The rate may be greater during meals.
Aspiration pneumonia rates are threefold higher in PD subjects compared to
age-matched controls (Cosentino etal.,
2022).
In patients with PD, solid foods are
often more difficult to swallow than
liquids as the suprahyoid musculature
may be significantly affected (GonzálezFernández et al., 2008; Luchesi et al.,
2015). Difficulty swallowing pills, globus sensation, coughing during meals,
postswallow voice changes, and chest
infections may be early signs of swallow
impairment in the PD patient (Cosentino et al., 2022). PD patients aged over
63, with high levodopa doses and gait
difficulty or postural instability, demonstrate increased risk of developing
dysphagia (Cosentino et al., 2022).
The hypokinesia that characterizes
limb and truncal movements in PD
appears to slow initiation of the swallow sequence at the oral cavity and
then prolong laryngeal and esophageal
movements (González-Fernández et al.,
2008; Rugiu, 2007). A high prevalence of
esophageal dysfunction is seen in PD
patients as diagnosed by high-resolution manometry (Cosentino et al., 2022).
Sialorrhea may result and stasis and
residue may be seen on contrast studies
(Nicaretta et al., 2013). Electrophysiological studies of patients with PD have
demonstrated marked delay in triggering swallow, extremely prolonged pharyngeal swallow durations, and normal
cricopharyngeal muscle contractions
(Ertekin et al., 2002). Oropharyngeal
coordination may be impaired and
muscle rigidity may affect bolus transit
through the pharynx and into the pharyngoesophageal segment (PES), even
with a compliant sphincter (Nicaretta
et al., 2013).
A videofluoroscopic study of 34 patients with swallowing complaints and
Parkinson’s disease was conducted
using the objective measures of event
timing and structural displacement
described in Chapter 8 of this book.
That study identified a delay in airway
closure to be present in 62% of patients
and poor pharyngeal constriction to be
present in 30.4%. No evidence of prolonged pharyngeal transit times, poor
hyoid elevation, or cricopharyngeal
achalasia were identified (Ellerston
etal., 2016).
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Early screening or assessment of
swallow, particularly with instrumental tools, in those with PD is recommended, particularly as voice and
swallow symptoms respond poorly to
pharmacotherapies given for PD or to
deep brain stimulation.
Motor Neuron Diseases:
Amyotrophic Lateral
Sclerosis (ALS)
Motor neuron diseases (MNDs) encompass a range of disorders that are classified depending on the type of motor
neuron subset that is affected. ALS
involves both upper and lower motor
neurons and causes dysfunction at both
bulbar and spinal levels, and dysphagia is a common manifestation (Ertekin
et al., 2000; González-Fernández et al.,
2008; Ramroop & Cruz, 2022; Rugiu,
2007).
ALS is a progressive, neurodegenerative disease that destroys nerve
cells in the brain and spinal cord.
Bulbar dysfunction at presentation is
apparent in 20% of patients (Ramroop
& Cruz, 2022). Over time, dysphagia
becomes almost ubiquitous, leading
to aspiration pneumonia in approximately 15% of patients (Ertekin et al.,
2000; González-Fernández et al., 2008).
Solid food dysphagia is usually the
first manifestation of swallowing difficulty, but symptoms may eventually
be more global, with abnormal swallow patterning and rhythmicity, frequent pharyngeal residue, penetration,
and aspiration (Aydogdu et al., 2011).
Dysphagia affects quality of life in ALS
patients and is a significant source of
morbidity (Ertekin et al., 2000). Patients
may describe a globus sensation due
to poor pharyngeal transit and poor
trans-sphincteric flow due to upper
esophageal sphincter (UES) dysfunction (Ertekin et al., 2000; Rugiu, 2007).
Poor oral and tongue control (muscle
may fasciculate or atrophy), delayed
pharyngeal transit, and UES dysfunction are often present and well delineated by DSS (Aydogdu et al., 2011;
Ertekin et al., 2000). These investigators performed EMG studies in 43 ALS
patients and demonstrated prolonged
hyolaryngeal elevation, reduced and
uncoordinated UES opening, and
loss of voluntarily initiated swallow sequence (Aydogdu et al., 2011;
Ertekin et al., 2000). Electrodiagnostic
testing is key to confirming diagnosis,
and identifying motor neuron diseases
and subtypes (nerve conduction studies and EMG; Ramroop & Cruz, 2022)
(Table 16–2). To quantify impairment,
a recently developed questionnaire,
the DYALS (dysphagia in amyotrophic
lateral sclerosis), has been adapted
from the DYMUS questionnaire used
in multiple sclerosis (see below under
Multiple Sclerosis heading). This questionnaire correlates with EAT-10 scores
and indicates worse swallowing in bulbar ALS patients compared to spinal
ALS patients, as expected (Diamanti
etal., 2022).
In MND/ALS disorders, both structural and functional swallow abnormalities are present and contribute to
airway violation prior to, during, and
after swallow. Aspiration is often silent
and may be accompanied by cognitive dysfunction (Printza et al., 2021).
Tongue weakness is also a feature and
correlates with higher EAT-10 scores,
increased aspiration, more pharyngeal
secretions and decreased pharyngeal
efficiency (Printza et al., 2021). Because
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