396

based on neuropathology such as abnormalities

of the tau protein untenable, and the neuropathology data available in some instances are incompatible with traditional clinical diagnosis as when
progressive supranuclear palsy (PSP) defined
pathophysiologically is diagnosed clinically as
PD. Consensus conferences [1] have improved
the correlation of clinical and pathophysiological
diagnosis but have left some clinicians (and neuropathologists) unsure about traditional clinical
syndromes. What remains is a traditional hybrid
approach to the ordering of the disease content
mixing clinical diagnoses such as PD and
Parkinson plus syndromes with sections based on
the primary pathophysiology such as dystonia
and ataxia. This approach affords the most common progressive neurologic diseases supplemented by brief, presentation of rarer conditions
such as Wilsons disease, GuillanBarre, and
post-polio syndrome. The purpose of this chapter
is to provide a comprehensive review of the effect
of progressive neurologic disease on swallowing
dysfunction and briefly discuss management
options.

J.C. Rosenbek and M.S. Troche

18.5% and 100% [36] with silent aspiration

occurring in at least one-third of patients [7]. Not
surprisingly, many PD patients report no swallowing impairment presumably due to their
frequent lack of insight about their neurologic
changes [8].

Pathophysiology
The etiology of swallowing dysfunction in persons with PD has not been well defined. Changes
have been attributed to the cardinal symptoms of
PD from dopaminergic pathway abnormality:
rigidity, hypokinesia, and tremor [9]. Rigidity
and bradykinesia have been implicated specifically
as responsible for difficulty chewing and drooling. Eadie and Tyrer [10] and Ertekin et al. [11]
hypothesized that the hypokinetic, reduced rate
of spontaneous swallowing movements, and the
slowness of segmented but coordinated sequential movements (p 948), may be the most
significant cause of swallowing dysfunction in
PD. Lastly, swallowing dysfunction in PD has
also been attributed to the involvement of the dorsal motor nucleus of the vagus nerve and of
Lewy bodies in the myenteric plexus of the
esophagus [12] (p 730).

Parkinsons Disease
Definition
Parkinsons disease (PD) has long been considered
an illness caused by dopamine depletion in the
substantia nigra that affects only motor function,
but the current conceptualization of PD acknowledges that it affects distributed neuroanatomical
regions, disrupting multiple motor and nonmotor systems [2]. The cardinal symptoms
include bradykinesia, rigidity, resting tremor, and
postural instability. In addition to basal gangliaspecific changes, it is now recognized that the PD
process begins in the dorsal motor nucleus of the
vagal nerve and, from there, proceeds upward
until it arrives at the cerebral cortex [2].
Prevalence/Incidence of Swallowing
Dysfunction
The incidence of dysphagia in persons with PD
established by careful clinical and instrumental
examination has been reported to be between

Diagnosis
Widespread impairment in PD results in swallowing deficits of every stage of swallowing, and
therefore, in addition to a complete history,
videofluoroscopy seems to be the best method
for assessing both motor and sensory involvement of the entirety of the swallowing mechanism. Dysfunction is commonly seen in oral
manipulation of the bolus including lingual
pumping, labial bolus leakage, lingual tremor,
slowed or limited mandibular function, piecemeal deglutition, pre-swallow spill, delayed
swallow triggering, and post-swallow residue
[4, 11, 1320]. Changes to the pharyngeal phase
of swallow include slow pharyngeal transit,
abnormal/delayed contraction of the pharyngeal
wall, coating of the pharyngeal walls with bolus
material, deficient epiglottic positioning,
decreased epiglottic range of motion, stasis in the
vallecula and pyriform sinuses, slow laryngeal
elevation and excursion, penetration, aspiration,

27

Progressive Neurologic Disease and Dysphagia...

and upper esophageal sphincter (UES) discoordination [4, 8, 1321]. Other associated impairments include vocal fold bowing, drooling, and
difficulty swallowing saliva in up to 78% of persons with PD, and deficits in swallowrespiratory
relationships as evidenced by more swallowing
during inhalation and swallowing at low tidal
volume [2224]. Further complicating the management of persons with PD and dysphagia are
the associated gastrointestinal symptoms which
often accompany PD [25]. These in conjunction
with oropharyngeal dysphagia can result in
reduced oral intake.

Complications
The risk of death secondary to pneumonia in PD
is six times greater than in those without PD [26],
with aspiration pneumonia being the leading
cause of death [2731]. This risk is probably a
consequence of chronic immobilization and
swallowing impairment, particularly in later
stages of the disease [32].
Management
Levodopa (l-Dopa), the gold standard for the
treatment of PD related symptoms, has not been
found to be efficacious for the treatment of dysphagia in PD [5, 15, 16, 33], nor does l-Dopa
improve/influence respiratoryswallow relationships [23]. A study testing the effects of various
compensatory strategies (i.e., chin tuck, nectar and
honey-thickened liquids) on occurrence of pneumonia in persons with dementia and PD found
significantly higher incidence of pneumonia in
persons given honey-thickened liquids versus
nectar-thickened liquids [34]. Additionally, a
recent randomized clinical trial found 4 weeks
of treatment with an expiratory muscle training
device resulted in significantly improved swallowing safety (i.e., reductions in penetration/aspiration) and improved cough effectiveness [35].
Other smaller scale non-randomized studies have
identified LSVT [36], verbal cueing [24], traditional swallowing exercises (e.g., Mendelsohn,
range of motion) [37], and bolus modification
[38] as possible treatment modalities for dysphagia in PD. Surgical management with cricopharyngeal sphincterotomy and myotomy for

397

cricopharyngeal dysfunction have been discussed

in the literature with good results on selected
patients [15, 39].

Parkinsonian Syndromes
Definition
Depending on the source, 12 or more conditions
are included among the parkinsonian syndromes
[40]. Multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) are discussed
in this section. Gilman and colleagues [41] define
MSA as a progressive neurodegenerative disease
of unknown etiology. The disease occurs sporadically and causes parkinsonism with cerebellar,
autonomic, urinary and pyramidal dysfunction in
many combinations [41]. Three syndromes are
traditionally identified: (1) MSA-P (also called
striatonigral degeneration or SND) in which
parkinsonian features predominate, (2) MSA-C
(also called OPCA) in which cerebellar features
predominate, and (3) MSA-A (called ShyDrager
syndrome, an increasingly infrequent label) in
which autonomic features predominate. Signs of
all three types appear in more than 25% of
patients with disease progression. See Higo [42]
for more details. PSP, also called Richardsons
disease, is a progressive neurologic disorder of
middle age often beginning with gait abnormality
and frequent falls. Litvan and colleagues [43]
outline criteria for diagnosis.
Prevalence and Incidence
The estimated prevalence for MSA, without
regard for subtypes, is 4.0 per 100,000 people
[44]. Ben-Shlomo et al. [45] report onset at an
average age of 54 years and a median survival of
6.2 years. Dysphagia occurs earlier and with
greater severity in MSA than in Parkinsons
disease. Shulman et al. [46] call dysphagia a
pervasive sign. Using videofluoroscopy, Higo
and colleagues [47] identified at least one sign of
dysphagia in approximately 75% of patients with
MSA referred for swallowing evaluation.
Prevalence of PSP is estimated at 3.9 per 100,000
persons in the United States [48]. Onset usually
occurs between ages of 55 and 70 and death for

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most occurs 57 years after diagnosis. Litvan

et al. [49] reported a variety of oral and pharyngeal stage symptoms in 26 of 27 patients with
PSP, including coughing and choking and signs
on videofluoroscopic swallowing examination
including delayed swallow initiation.

Pathophysiology
Pathophysiology is complex in MSA. Delayed
initiation of movement, slowed movement, and
rigidity are the most frequent parkinsonian signs.
Hypotonicity and dyscoordination are likely
with cerebellar involvement. Hypertonicity and
affective disorders and additional cognitive
changes impacting swallowing can also occur.
Rigidity, bradykinesia, spasticity, and dystonia
are common features of PSP and are often accompanied by general cognitive slowness and behavioral abnormalities [43].
Diagnosis
The definitive diagnosis of MSA requires
autopsy. The clinical diagnosis of PSP requires
the presence of gait instability, early falls, rigidity, bradykinesia, vertical gaze abnormality,
spastic dysarthria, profound dysphagia, and
frontal lobe abnormalities [49]. The swallowing
clinician is often working with a tentative diagnosis. The swallowing evaluation can accomplish the following: (1) establish the signs of
dysphagia and its severity, (2) support hypotheses about the usually complex underlying
pathophysiology as a focus of treatment, (3)
determine the probable quality of life and health
impact of the dysphagia, (4) identify potential
affective and cognitive influences on the swallowing and on treatment, (5) attend carefully to
laryngeal stridor secondary to vocal fold immobility which may be more common in MSA than
in other neurodegenerative diseases, and (6)
contribute to differential diagnosis by identifying the onset and features of the dysphagia. Both
oral and pharyngeal deficits are often observed.
According to Higo and colleagues [47] the most
common signs are delayed initiation of the swallow, reduced posterior tongue movement, and
disturbance of bolus holding (p 632).
Residuals in the pyriform sinuses and valleculae

J.C. Rosenbek and M.S. Troche

and reduced opening of the UES are among the

most frequent pharyngeal signs [50, 51].

Complications
Higos group [52] says dysphagia is the most
critical complication of MSA (p 647). Muller
et al. [53] report an average duration of 15 months
from a patients recognizing swallowing changes
to death. Twenty-four percent of Higo et al.s [47]
patients had a history of aspiration pneumonia.
Muller and colleagues [53] identify dysphagia as
a bad prognostic sign in PSP. Aspiration, dehydration, malnutrition, and prolonged eating
with attendant caregiver stress are among the
complications.
Management
Because medical and surgical management of
parkinsonian syndromes is often of limited usefulness for all but general symptom reduction,
dysphagia management is especially critical. If
the underlying pathophysiology is assumed to be
rigidity, hypotonia, and/or weakness then a
variety of the strengthening techniques may be
appropriate. If dyscoordination, delayed initiation, or slow movement predominates, the
skill building techniques may be appropriate.
Combinations will likely be necessary as will be
a concerted behavioral effort to improve cognitive deficits that include attention and general
responsiveness. While these efforts are ongoing,
compensations will also be useful.

Multiple Sclerosis
Definition
Multiple sclerosis (MS) is an inflammatory process in which the myelin surrounding the axons
of the brain and spinal cord is damaged leading
to demyelinating and lesions throughout the
neuroaxis [54]. There are four main types of MS:
(1) relapsing/remitting, (2) secondary progressive, (3) primary progressive, and (4) progressive
relapsing. Relapsing/remitting, the most prevalent of the subtypes, is characterized by acute
attacks of MS followed by periods of remission.
This is in contrast to secondary progressive MS