Swallow Characteristics in Patients W
Oculopharyngeal Muscular Dystrophy
Phyllis M. Palmer
Amy T. Neel
University of New Mexico, Albuquerque
Gwyneth Sprouls
University of New Mexico
Hospitals, Albuquerque
Leslie Morrison
University of New Mexico Health
Sciences Center, Albuquerque
Journal of Speech, Language, and Hearing Research •
Purpose: This prospective investigation evaluates oral weakness and its impact on
swallow function, weight, and quality of life in patients with oculopharyngeal
muscular dystrophy (OPMD).
Method: Intraoral pressure, swallow pressure, and endurance were measured using
an Iowa Oral Performance Instrument in participants with OPMD and matched
controls. Timed water swallow, weight, and quality of life were also assessed.
Results: Participants with OPMD were weaker than controls. Oral weakness impacted
strength, swallow pressure, swallow capacity, swallow volume, swallow time, and
quality of life. Tongue endurance was not affected by oral weakness.
Conclusion: This investigation provides further insight into the swallow function of
patients with myopathic disease. Muscle fiber loss leads to weakness, which results
in reductions in swallow function and quality of life. Weight and endurance are
not greatly altered.
KEY WORDS: OPMD, swallow pressure, pressure reserve, SWAL-QOL,
timed water swallow, IOPI
T here is a paucity of research on the swallow deficits in patients
with oculopharyngeal muscular dystrophy (OPMD), a rare myopathic
disease with a high incidence in Hispanic New Mexicans (Becher
et al., 2001). Although genetic in origin, symptoms of OPMD do not typ-
ically become evident until the fifth decade of life. The primary hallmark
of this disease is progressive degeneration of the muscles of the eyelids
and pharynx, with later onset of proximal limb weakness. Degeneration
of the pharyngeal muscles results in dysphagia. Limited investigations of
the oropharyngeal dysphagia associated with OPMD have primarily fo-
cused on retrospective reviews of medical records and videofluorographic
evaluations of swallowing (Palmer et al., 2006; Young & Durant-Jones,
1997). Although various pharyngeal swallow deficits were identified, the
most notable were pharyngeal residue and reduced upper esophageal
sphincter (UES) opening.
The deficits associated with OPMD are not limited to the pharyngeal
structures. Recent investigations have evaluated the impact of OPMD
on oral structure and function. Using MRI and computerized tomography,
King, Lee, and Davis (2005) noted dystrophic changes (i.e., muscle fiber
loss with fatty infiltrates) in the tongue muscles of patients with OPMD.
This muscle wasting was evident despite no clinical change in tongue bulk.
Although impacting overall strength, the reduced number of muscle fibers
in the tongue does not greatly reduce speech intelligibility (Neel, Palmer,
Sprouls, & Morrison, 2006). A slowed speech rate may help compensate for
the reductions in muscle strength.
Vo l . 5 3 • 1 5 6 7 –1 5 7 8 • D ece mb er 2 0 1 0 • D Am eri ca n S p eech -L a ng u a ge -H ea ri n g A ss o ci at i o n 1567
 Although articulation and swallowing are both sub-
maximal tasks, the strength required for swallowing ex-
ceeds that needed for articulation. Tongue strength can
be evaluated by measuring maximal intraoral isometric
pressure as well as pressure during swallow. The impact
of weakness may be reflected in reduced pressure re-
serve, which has been defined as the difference between
themaximalisometricpressureandtheswallowpres-
sure (Nicosia et al., 2000). As the tongue plays an essen-
tial role in the swallow process, it is likely that reduced
strength impacts oropharyngeal function for swallowing
and/or the muscle endurance required to maintain func-
tion throughout a mealtime. Alterations to bolus flow
and swallow timing can be expected.
Although reductions in intraoral strength may lead
to dysphagia, the effect on weight is not clear. In healthy
aging individuals, weight tends to increase during mid-
dle age and decrease with advanced age (Borkan, Hults,
Gerzof, Robbins, & Silbert, 1983; Dey, Rothenberg, Sundh,
Bosaeus, & Steen, 1999). Clinically, dysphagia is associ-
ated with weight loss. Therefore, we expect that advanced
disease results in weight loss.
In the present investigation, we evaluated oral weak-
ness and its effect on function in patients with OPMD.
However, our goal was more than the mere description
of a specific disease. As a myopathic disease (Bouchard,
Brais, Brunet, Gould, & Rouleau, 1997; Brais, 2003), in-
vestigations of patients with OPMD provide an estimate
of the consequences of motor damage (and, therefore,
weakness) on swallow function. We hypothesize that the
reductioninthenumberofmusclefibersresultsinoral
weakness, yieldingreduced swallow pressures and swal-
low reserve. These reduced pressures are expected to im-
pact the flow of the bolus and result in increased swallow
time and reduced swallow capacity. Despite reduced swal-
low function, muscle endurance may be maintained, as it
is measured using a percentage of maximal performance.
However, in a more advanced stage of the disease, we
would anticipate that endurance would also decline. Given
the expected decline in swallow function, poor weight
maintenance and reduced quality of life are likely.
The purpose of the present investigation wastwofold:
(a) to describe the oral deficitsassociated with OPMD and
link those deficits to swallow function, weight, and qual-
ity of life and (b) to provide insight into the oral motor def-
icits associated with oral motor decline, including not
only disease processes but also healthy aging itself.
Method
Participants
Eleven Hispanic individuals with OPMD (eight
women and three men) and nine Hispanic controls (five
women and four men) participated in this investigation.
1568 Journal of Speech, Language, and Hearing Research •
Participants with OPMD were recruited through the
OPMD Clinic at University Hospital, Albuquerque, New
Mexico. Control participants were matched for the age
range of the participants with OPMD. Control partici-
pants denied any history of swallow problems. Common
medical problems, such as diabetes, did not exclude par-
ticipants. Table 1 displaysthe age, gender, height, weight,
and other medical conditions for each participant. Fourof
the 11 participants with OPMDunderwent medical treat-
ment for their pharyngeal swallow deficits prior to data
collection. Three received a botulinum toxin (botox) in-
jection to the cricopharyngeus muscle, and one received
dilatation. For those four participants, the time from the
medical treatment to the data collection is indicated in
Table 1. As these treatments are aimed at altering pha-
ryngeal performance and not oral performance, these
treatments did not exclude participation.
Data Collection and Analysis
The data presented here are part of a larger study
evaluating the voice, speech, and swallow character-
istics in patients with OPMD. Collectively, these data
were obtained over a 2-to 3-hr period, dependingon the
participant’s speed and need for breaks. The present in-
vestigation focused on the results for swallow parameters
only. Swallow function was evaluated by (a) measuring
intraoral pressure during isometric and swallow tasks,
(b) measuring tongue endurance, (c) performing a timed
water swallow, (d) asking participants to complete a
swallow-related quality-of -life questionnaire, and (e) mea-
suring height and weight in order to calculate body mass
index and weight percentile.
Intraoral pressure. Tongue-to-palate strength was
measured during maximal isometric and swallow tasks
using the Iowa Oral Performance Instrument (IOPI; IOPI
Northwest Co., Carnation, WA). IOPI data were digi-
tized at a sampling frequencyof 1000 Hz and stored on a
computer using a digital acquisition system (WINDAQ;
DATAQ Instruments Inc., Akron, OH). Regardless of task,
the oral pressure bulb was centered on the midpoint of
the tongue body such that the end of the bulb did not ex-
tend past the mandibular teeth. During the first place-
ment, a marker wasused to draw a line at the place where
the IOPI tube aligned with the exterior border of the
lips. This mark was used to ensure consistent placement
throughout the testing protocol.
For maximal tongue-to-palate pressure, participants
were instructed to press the IOPI bulb as hard as possible.
Each participant performed this task four times—twice
early in the data collection session and twice at the end.
Maximal pressure was recorded as the greatest value
across the four trials.
For all participants, intraoral pressure was measured
during saliva swallows. Each participant was instructed
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 Table 1. Description of participants with OPMD and control participants.

Symptom duration Medical Time Weight Height Other medical

Variable Sex Age (in years) a treatment since rx (kg) (cm) conditions

Participants with OPMD

S1 F 57 5 None N/A 67.5 148 Diabetes
S2 F 57 9 None N/A 50 155 Arthritis
S3 F 57 2 None N/A 79 164 None
S4 F 62 4 None N/A 78 156 None
S5 F 63 3 Botox 9 months 59 150 Diabetes, asthma
S6 F 66 10 Botox 3 months 73 165 None
S7 F 67 10 Dilatation 2 months 70 157.5 Diabetes, lumbar disc rupture
S8 F 73 10 None N/A 70.5 154 Arthritis, hypertension
S9 M 50 2 None N/A 80.5 177 Diabetes, hypertension
S10 M 59 8 Botox 5 years 75 175 Spinal stenosis
S11 M 61 10 None N/A 68.5 168.5 None

Control participants
C1 F 54 67 161 None
C2 F 61 78 154 Hypothyroid, glaucoma
C3 F 61 62 149.5 None
C4 F 64 51.5 158.5 Anxiety
C5 F 67 121 165.5 Asthma, arthritis
C6 M 52 110 183 None
C7 M 56 81 178 None
C8 M 58 109.5 170 None
C9 M 76 82.5 170 Coronary artery disease

Note. OPMD = oculopharyngeal muscular dystrophy; rx = treatment; F = female; M = male; N/A = not applicable.
a
Patient report of the duration of the dysphagia symptoms.

to drink 10 ml of water prior to data collection to reduce maximum pressure. Percentage of the maximal pressure
possible oral dryness. The bulb was then placed in the used to swallow was calculated by dividing the average
mouth, and the participant was asked to swallow. This saliva swallow pressure into the maximum isometric
procedure was performed fourtimes. Average saliva swal- pressure.
low pressure was calculated across the four trials. Tongue endurance and fatigue. Tongue endurance
After it became clear that participants with OPMD and fatigue were measured as an estimate of a partic-
could manage the task of swallowing with the pressure ipant ’s ability to safely complete a meal in a timely fash-
bulb in the oral cavity, and the data collection protocol ion. Maximal endurance was measured in two ways. The
did not cause fatigue, a water bolus was added. For seven first few participants were asked to hold 25% of their
participants with OPMD and seven control participants, maximal strength for15s. This time cap was used to limit
pressure was measured during two trials of 15-ml water
boluses. This bolus size was selected to reflect a challenge
Figure 1. Sample pressure waveform for a 15-ml water swallow.
to the oropharyngeal swallow system. Pressure during
water swallows was measured by placing the IOPI bulb on
the mid-tongue. Water was dispensed via a syringe. Par-
ticipants were asked to swallow the bolus in one swallow,
and swallow pressure was averaged across the two trials.
Peak pressure data were extracted from the wave-
form using a digital oscilloscope (WINDAQ; DATAQ In-
struments Inc., NJ). Figure 1 displays a sample waveform.
In a case in which multiple peaks were identified, the
highest peak was used. No other data points were ex-
tracted from the waveform. For all participants, pres-
sure reserve was calculated by subtracting the average
pressure generated across the saliva swallows from the

Pa l me r et a l . : Swallow in OPMD 1569

any muscle fiber damage that can occur in patients with
muscular dystrophy during excessive exercise. After three
participants with OPMD completed the endurance task
and reported no fatigue or discomfort, the instructions
were changed. Participants were then asked to hold 25%
of their maximal strength “for as long as possible.” Par-
ticipants were encouraged to terminate the trial at the
onset of struggle or fatigue. Each participant performed
two trials of the endurance task. Using the longer of the
two trials, endurance was analyzed using a technique sim-
ilar to that used in Solomon, Robbin, and Luschei (2000).
For 10 waveforms, endurance analysis was performed by
two raters. Interrater reliability wasconsideredacceptable
if the raters agreed within 2 s. Interrater agreement was
100%.
In addition to maximal endurance, fatigue was esti-
mated by having participants perform similar tasks at
the beginning and end of the data collection protocol. For
all participants, these tasks included maximal isometric
pressure and saliva swallow pressure. Endurance trials
were recorded early and late in the data collection ses-
sion for all participants whose data were not capped. Com-
puter recording errors occurred in five endurance trials.
Omitting participants with capped endurance data, and
those who had recording errors on the early- or late-
endurance trial, yielded seven participants with OPMD
and five controls. Performance between the early and
late trials was compared using descriptive statistics and
a paired t test. Fatigue was also rated by participants
using the Fatigue subscale of the Swallow Quality of
Life Instrument (SWAL-QOL; McHorney et al., 2000).
Timed water swallow. Using a technique similar to
that applied in Hughes and Wiles (1996), we instructed
participants to perform a timed water swallow; in this
technique, we asked participants to swallow 50 ml of
water“as quickly as is c omfortably possible” while timing
the participant and counting the number of swallows re-
quired to complete the task. Manual palpation was used
to count the number of swallows. The time was measured
from the moment the cup touched the lips until the last
swallow was completed. This procedure yielded (a) aver-
age volume per swallow, which was calculated bydividing
the number ofswallows into the total volume; (b) average
time per swallow, which was calculated by dividing the
number of swallows into the total time; and (c) swallow ca-
pacity, which was calculated by dividing the amount of
water by the amount of time. Phonation tasks were used
before and after the water swallow to note changes in vo-
cal quality, which may be suggestive of penetration, as-
piration, or pharyngeal residue. Presence or absence of
drooling or coughing was indicated before, during, and
after the water swallow.
Quality of life. All participants completed the
SWAL-QOL (McHorney et al., 2000) questionnaire, which
1570 Journal of Speech, Language, and Hearing Research •
evaluates outcome in several areas including burden,
eating, symptoms, food selection, communication, fear,
mental health, socialization, and fatigue. Each category
contains multiple probes. Each probe is rated on a 5-point
scale ranging from 1 (a significant problem)to5(no prob-
lem or concern). For each participant, probes within each
category were averaged, yielding one score per category.
Weight. Using height and weight, two estimates of
weight were evaluated—body mass index and weight per-
centiles that were extracted from the American weight
database (Third National Health and Nutrition Exami-
nation Survey, 1988–1994; National Center for Health
Statistics, 2008).
Statistical differences between the two participant
groups were tested usinganalysis of variance. Effect size
was calculated using Cohen’s d (Cohen, 1992). Correla-
tions were computed to identify variables most impacted
by reductions in tongue strength.
Results
Intraoral Pressure
Table 2 shows means and standard deviations for
pressure measurements for each group. Participants with
OPMD produced maximal intraoral pressures with an
average of 26.9 kPa and a range from 11 to 39 kPa. These
pressures are significantly lowerthan those produced by
the control participants, who averaged 57.4 kPa with a
range from 37 to 71 kPa, F(1, 18) = 49.8, p < .001. We cal-
culated a large effect size (Cohen’s d = 3.12). Female par-
ticipants with OPMD produced an average maximum
pressure of 27.4 kPa, whereas male participants with
OPMD produced maximum pressures at an average of
25.7 kPa. Female control participants produced an aver-
age maximum pressure of 58.0 kPa; male control partic-
ipants produced maximum pressures at an average of
56.8 kPa.
Table 2. Means (and SDs) in kPa of maximal intraoral pressures and
saliva swallow pressures for participants with OPMD and control
participants.
Saliva swallow
Max Pressure %of
Variable (kPa) (kPa) max Reserve
OPMD 26.9 (7.8) 10.9 (3.7) 41 (20.6) 16.0 (7.9)
Controls 57.4 (10.4) 20.9 (7.0) 36 (12.5) 36.5 (12.1)
p <.001 <.001 .467 <.001
Cohen’s d 3.12 1.73 .40 1.80
Note. Percentage of maximum and pressure reserve are calculated for
swallow pressures. Effect size was calculated using Cohen’s d.
V o l. 5 3 • 1 5 6 7 – 1 5 7 8 • De cemb e r 2 0 1 0
 Participants with OPMD produced a statistically
weaker saliva swallow than controls, with an average
of 10.9 kPa and a range from 6 to 18 kPa, F(1, 18) = 15.9,
p < .001. We calculated a large effect size (Cohen’s d = 1.73).
Control participants produced a stronger saliva swallow,
with an average of 20.9 kPa and a range of 12–35 kPa.
Figure 2 displays the individual performance for each
participant on maximal pressure and saliva swallows.
We calculated pressure reserve as the difference be-
tween the maximum intraoral pressure and saliva swal-
low pressure (see the gray bar in Figure 2). Pressure
reserve was statistically smaller for participants with
OPMD (average = 16.0 kPa) than for the control partic-
ipants (average = 36.5 kPa), F(1, 18)= 16.5, p < .001 (see
Table 2). Percentage ofmaximal tongue strength applied
to a saliva swallow did not differ significantly across the
two participant groups and averaged 41% for partici-
pants with OPMD and 36% for controls, F(1, 18) = 0.55,
p = .467. We calculated a medium effect size (Cohen’s
d = 0.40).
For a subset of participants, intraoral pressure was
measured during a 15-ml water swallow. On average,
participants with OPMD produced a 15-ml swallow with
10.4 kPa (SD = 2.4); controls swallowed 15 ml of water
with an average of 22.3 kPa (SD = 8.9). Similar to the sa-
liva swallow, intraoral pressures during water swallows
were statistically reduced for participants with OPMD
when compared with controls, F(1, 18) = 9.24, p =.01,
Cohen’s d = 1.63.
Tongue Endurance and Fatigue
Three participants with OPMD and one control were
administered endurance trials with a 15-s cap. All of
these participants, with the exception of one participant
with OPMD, were able to reach the cap. The remaining
participants (eight with OPMD and seven controls) were
not capped during the endurance task. Excluding the par-
ticipants whose endurance data were capped, tongue en-
durance was maintained for an average of 119 s (SD =63),
with a range of 33–243 s for participants with OPMD.
Control participants maintained the endurance task for
an average of 122 s (SD = 43), with a range of 49–208 s.
There was no statistical difference and a negligible effect

Figure 2. Maximum isometric intraoral pressure for the two participant groups. The total bar represents maximal pressure produced by a single
participant. The bar is divided into the bottom (black) half, which represents the average pressure used during a saliva swallow, and the top (gray)
half, which represents the pressure reserve. AVG = average; OPMD = oculopharyngeal muscular dystrophy.

Pa l me r et a l . : Swallow in OPMD 1571

size between the two groups of participants with respect
to endurance, F(1, 14) = .006, p = .940, Cohen’s d = .05.
We further estimated fatigue for all participants by
conducting repeated measures of maximum isometric
pressure, saliva swallow pressure, and endurance both
early and late in the data collection session. Figure 3
shows the average performance on earlyand late trials of
maximal pressure, saliva swallow pressure, and endur-
ance. We found no significant differences between early
and late trials for any of the parameters.
Using the Fatigue subscale ofthe SWAL-QOL, fourof
the five control participant rated themselves as having
mild fatigue; the remaining controls did not report fa-
tigue. All participants with OPMD reported fatigue with
an average score of 2.9 out of 5.
Timed Water Swallow
Table 3 displays the mean number of swallows and
the amount of time required to ingest a 50-ml water bo-
lus. We calculated swallow capacity, which is defined as
Figure 3. Mean performance on early and late trials of (a) maximum pressure (kPa), swallow pressure (kPa), and (b) endurance (s).
1572 Journal of Speech, Language, and Hearing Research •
the number of milliliters per second, and volumeper swal-
low, which isdefined as the millilitersper swallow, for each
participant group. All participants completely swallowed
the 50-ml bolus. Neither group of participants had drool-
ing before, during, or after the timed water swallow, nor
did any participant display vocal quality changes. One
participant with OPMD had mildcoughingafterthe swal-
low; however, that participant reported a recent illness
with a persistent cough. It is unclear whether the cough-
ing event was related to the recent illness or the swallow
task. No other participants from either group displayed
any choking or coughing during or after the water swal-
low. Swallow capacity, F(1, 18) = 21.9, p < .001, and volume
per swallow, F(1, 18) = 12.23, p = .003, were significantly
reduced for participants with OPMD. Each had a large
effect size (Cohen’s ds = 2.06 and 1.50, respectively). Par-
ticipants with OPMD took from three to 12 swallows to
completely ingest the 50-ml water bolus, with an aver-
age of 6.9 swallows. Control participants used one to four
swallows with an average of 2.6 swallows; two of the male
control participants ingested the 50-ml bolus in one swal-
low. Time per swallow was similar across the two groups,
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Table 3. Mean performance (and SDs) on a timed water swallow test including the amount of time and number of swallows required to ingest
a 50-ml water bolus.

Swallow capacity Volume per swallow Time per swallow

Variable # seconds # swallows (ml/sec) (ml/swallow) (sec/swallow)

OPMD 13.5 (9.1) 6.9 (2.6) 5.2 (2.2) 8.4 (3.5) 1.9 (0.7)
Controls 5.0 (1.8) 2.6 (1.0) 11.2 (3.7) 24.5 (14.0) 2.0 (0.7)

Note. Swallow capacity and volume per swallow were calculated.

averaging 1.9 s per swallow in participants with OPMD Weight

and 2.0 s in control participants.
On average, body massindex was lower for participants
with OPMD (M = 27.1) than for healthy controls (M =
Quality of Life 30.7) (see Table 4). Although these differences were not
statistically significant, F(1, 18) = 49.8, p = .161, they did
Quality of life was significantly lower for participants
have alarge effect size (Cohen’s d = 0.64). Figure 5 displays
with OPMD with a large effect size (p < .001, Cohen’s
the weight percentiles for each participant. Weight percen-
d = 3.24). Figure 4 displays the mean ratings from the
tiles for participants with OPMD averaged in the 39th
SWAL-QOL categories for each of the participant groups.
percentile, with a range from the 2nd to the 65 th percen-
For five of the nine control participants, the maximum
tile. Control participants averaged in the 58th percentile,
score of 5, which indicates no problem, was achieved in all
with a range from the 2nd to the 98th percentile.
areas. The remainingfourcontrol participants had scores
between 4 and 5 for one or two categories. Participants Correlations
with OPMD showed reduced ratings in all areas, with av-
erages ranging from 2.2 for burden to 3.6 for social, which Table 5 shows correlations between maximum intra-
indicates that the swallow deficit is often a problem. oral strength, as measured by maximal pressure, and

Figure 4. Mean ratings obtained on the SWAL-QOL for controls and participants with OPMD.

Pa l me r et a l . : Swallow in OPMD 1573

Table 4. Mean (and SD) body mass index (BMI) and weight
percentile for the two participant groups.
Variable OPMD Controls Cohen
BMI 27.1 (3.2) 30.7 (6.4) 0.64
Weight percentile 39th (17.5) 58th (29.7) 0.69
other swallow variables. Maximal pressure correlated
significantly with swallow pressures, swallow capacity,
and quality of life. It did not correlate with endurance
and weight.
Discussion
To define the swallowfunction in patientswith OPMD,
we compared performance of participants with OPMD
and controls on measures of isometric strength, swallow
strength, endurance, swallow function, weight, and qual-
ity of life. We hypothesized that the reduction in the num-
ber of muscle fibers along with fat replacement that
occurs with OPMD would result in reduced oral strength.
The outcome would be weaker tongue pressures during
Figure 5. Weight percentiles for each participant.
1574 Journal of Speech, Language, and Hearing Research •
Table 5. Correlations between various parameters and maximal
intraoral pressure for all participants.
’s d Variable max 1 2 3 4 5
1. Weight percentile .21 —
2. Swallow pressure .60** .34 —
3. Swallow capacity .55** .64** .60** —
4. SWAL-QOL .74** .37 .63** .71** —
5. Endurance .20 .20 .26 .35 .25 —
Note. SWAL-QOL = Swallow Quality of Life Instrument.
**p < .01.
maximal isometric and swallow tasks. Reduced tongue
strength might reduce bolus transport, thus yielding
sl ower, weaker swallows and negativelyimpactingswal-
low capacity, swallow volume, and quality of life. Weight
would be expected to decline with swallow function.
Oral Weakness and Swallow Function
Control participants in this investigation performed
similarly on both isometric and swallow pressures to
V o l. 5 3 • 1 5 6 7 – 1 5 7 8 • De cemb e r 2 0 1 0
those in other studies. Maximal pressures in our control
group averaged 57 kPa as compared with the healthy el-
derly participants measured by Stierwalt and Youmans
(2007), who averaged 55 kPa. Likewise, the swallow pres-
sures obtained in our controls (which averaged 20 kPa)
are similar to those of the elderly participants measured
byNicosia et al. (2000), who averaged 20 kPa forswallow.
Participants with OPMD have weaker oropharyngeal
muscles than their healthy peers as measured by maximal
isometric pressure. This is no surprise considering that
King et al. (2005) found a reduced muscle signal as well
as fatty infiltration in the tongue. Isometric pressures
generated by pressing the tongue against the palate re-
flect not onlythe tongue; floor-of-mouth muscles are also
recruited (Palmer et al., 2008; Palmer, Luschei, Jaffe, &
McCulloch, 1999). However, the King et al. data showed
no fat infiltration in the floor-of-mouth muscles that they
tested from patients with OPMD. Combined, these data
suggest that participants with OPMD produce a maxi-
mal pressure by engaging remaining(unaffected)tongue
muscle as well as floor-of- mouth muscles.
Reduced maximal strength could alter swallow pres-
sures in two ways. First, swallow pressure may be main-
tained despite a reduction in maximal pressure, yielding
a smaller pressure reserve and an increased effort for
swallowing. Second, reductions in maximal pressure would
coincide with incremental reductions in swallow pressure,
thus maintaining a swallow pressure equal to some per-
centage of maximal performance. In this investigation,
when compared with controls, on average, participants
with OPMD had swallow pressures that were reduced at
a similar rate to their maximal pressure. Further sup-
port for the notion that swallow pressure is dictated to
some extent by a percentage of maximal ability is gained
from the observation that both controls and participants
with OPMD used an average of approximately 40% of
their maximal strength to perform a swallow.
If participants with OPMD have greater intraoral
strength available to them (i.e., pressure reserve), then
why do they not use it to swallow? Although general mus-
cle fatigue may play a role, it is plausible, as noted above,
that swallow pressure is dictated by a percentage of max-
imal strength, and this percentage is associated with a
sense ofeffort. Perhaps the sense of effort is used to gauge
the amount of pressure (or percentage of maximum pres-
sure) used for the swallow. In the absence of intervention,
patients maintain the effort associated with swallowing
despite disease progression. Thus, with reductions in the
number of muscle fibers, a similar sense of effort yields a
lower swallow pressure.
Although not evaluated in this study, it is possible
that increased effort (i.e., engaging greater amounts of
the pressure reserve during swallow) may improve the
swallow function of patients with OPMD. In healthy
populations, the use of an effortful swallow increases
tongue movement, thus improving driving force on the
bolus (Hind, Nicosia, Roecker, Carnes, & Robbins, 2001;
Pouderoux & Kahrilas, 1995). In patients with head and
neck cancer, similar observations have been reported as
well as reduced pharyngeal residue (Lazarus, Logemann,
Song, Rademaker, & Kahrilas, 2002). Further research
is needed to test the hypothesis that a stronger swallow
improves swallow function in patients with myopathic
diseases.
In addition to reduced swallowpressure, participants
with OPMD used smaller bolus volumes and required in-
creased time to drink a 50-ml glass of water. High inter-
correlations were observed between maximal pressure,
swallow pressure, and swallow capacity. Thus, these data
support the hypothesis that a reduced number of muscle
fibers leads to oral motor weakness, and this weakness
leads to reduced swallow function. Some caution is war-
ranted, however, as this investigation did not evaluate
oropharyngeal bolus flow parameters. Therefore, it is
unclear from this study whether reduced pressures lead
to reduced swallow efficiency with respect to bolus flow.
Certainly in other disease processes, such as oral can-
cer and stroke, correlates have been reported between
tongue strength and reduced swallow function (Lazarus
et al., 2000; Stierwalt & Youmans, 2007). Stierwalt and
Youmans (2007) measured the intraoral pressure in
healthy individuals as well as those with documented
dysphagia. Although videofluorographic data were not
included, the participants with disordered swallow func-
tion had lower intraoral pressure than their healthy peers.
Furthermore, in patients with oropharyngeal cancer, re-
duced intraoral pressure correlated with a less efficient
bolus flow as indicated by temporal parameters mea-
sured using videofluoroscopy.
Muscle fiber loss and muscle weakness can result
from factors other than disease. Sarcopenia, or loss of
muscle mass, can occurwith healthy aging (Evans, 1995).
Age-related reduced muscle mass is likely associated with
the general physical decline that accompanies aging or a
sedentary lifestyle. In fact, in a healthy aging population,
maximal tongue strength decreases with advanced age,
and these decreases in strength (or increases in weakness)
can result in dysphagia (Nicosia et al., 2000). In the pres-
ent investigation, one control participant performed sim-
ilarly to the participants with OPMD with respect to
intraoral pressure tasks. Interestingly, this participant
was the oldest participant in the study. This result is con-
sistent with Robbins, Levine, Wood, Roecker, and Luschei
(1995) as well as with Nicosia et al. (2000), who showed
reduced intraoral pressure as a function of age in healthy
adults.
Unlike their healthy peers, people with disease-
related oropharyngeal muscle weakness may alter the
Pa l me r et a l . : Swallow in OPMD 1575
amount or type of liquids or foods selected for ingestion.
Due to the extensive pharyngeal weakness, patients with
OPMD may avoid thick liquids or masticated material.
With a diet high in thin liquids, there is less muscle work
required during mealtimes. Thus, the remaining healthy
muscle fibers experience not onlythe muscle loss related
to age and disease but also the impact ofreduced exercise
imposed by softer diet selections. These dietary changes
may accelerate the impact of age-related sarcopenia. Thus,
although the deficits noted in patients with OPMD may
be similarto those seen in the aging population, patients
with OPMD develop weakness, and therefore functional
deficits, at an earlier age. A 50-year-old patient with
OPMD may have oropharyngeal function comparable to
that of an 80-year-old healthy aging individual.
Weight
Despite oral weakness, although, on average, weight
percentiles were lower in participants with OPMD, no
obvious signs of malnutrition were observed. This aligns
well with Becher et al. (2001), who noted no reduction
in life span in this patient population. Collectively, these
findings differ from those reported byTaylor (1915) in the
early part of the 20th century. Taylor noted that patients
with OPMD often died from malnutrition and starvation.
There are a couple of explanations for these differences.
First, it is plausible that our participant sample was bi-
ased toward younger participants. Thus, in the present
investigation, participants with OPMD had a moderate
disease severity, whereas the older participants in Taylor ’s
investigation were likely more severely impaired. Cer-
tainly an older and more severely impaired group would be
expected to have more oral weakness leading to dysphagia
and, therefore, weight loss. Second, weight maintenance is
presently possible through alternate approaches that were
not available in the early part of the 20th century. The
ability to maintain weight despite dysphagia may be the
result of (a) readily available pureed foods, milkshakes,
and so forth; (b)medical treatment options such as botox
injections, dilatation, and myotomy for UES dysfunction
(a common problem in patients with OPMD); and (c) al-
ternate enteral and parenteral nutrition options. Although
none of our participants with OPMD were receiving al-
ternate nutrition, this technologyis available to patients
in the later stages of the disease, thus reducing the risk
of malnutrition and starvation and supporting an aver-
age life span.
Quality of Life
Although presently, life span may not be impacted
by the muscle weakness and dysphagia associated with
OPMD, quality of life is greatly reduced. In this inves-
tigation, all participants with OPMD reported reduced
1576 Journal of Speech, Language, and Hearing Research •
quality of life in all the areas questioned. In fact, quality
of life correlated with maximal swallow pressure and
swallow capacity. Healthy peers consistently reported
only mild changes with respect to symptoms, communi-
cation, and fatigue. These findings agree with the large
body of literature on health-related quality of life. In gen-
eral, healthy active adults experience a higher quality
oflife (e.g., Vallance, Murray, Johnson, & Elavsky, 2010).
Endurance and Fatigue
Reduced muscle endurance could alter performance
by changing the ability of muscles to persist in a task
over time (such as mealtimes). Although, theoretically,
endurance may change as a function of strength, the
available evidence suggests that endurance is main-
tained with advanced age despite reductions in maximal
strength (Crow & Ship, 1996; Stierwalt &Youmans, 2007).
Our data further support these earlier observations that
endurance is not greatly altered with age. Additionally,
participants with myopathic disease did not display per-
formance decay.
Using performance comparisons from the begin-
ning and end of a long protocol, we attempted to measure
the fatigue that patients with OPMD often report. Al-
though participants with OPMD reported fatigue on the
SWAL-QOL, performance did not decay on any of the tasks
used in this investigation. The facts that endurance is
maintained despite weakness and performance does not
decay over a short time may be due, at least in part, to
the predominance of fatigue-resistant fibers in the oro-
facial muscles. Accepting that the tongue is composed
largely of fatigue-resistant fibers (DePaul & Abbs, 1996),
it is plausible that endurance, when performed at 25% of
maximal ability, at least initially, engages the smaller
muscle fibers (slow, fatigue resistant). Larger fatigue-
resistant muscle fibers may be recruited as the endur-
ance task is accompanied by a greater sense of effort. As
swallow is likely more associated with the larger muscle
fibers, endurance may not serve as a sensitive or specific
metric for oral swallow pressure. With so few fatigable
fibers in the tongue, it may not be susceptible to fatigue,
even in the disordered population.
Another possibility is that the tasks used in this in-
vestigation did not adequately invoke fatigue. Two dif-
ferences are noted when this research is compared with
previous investigations. Typically, endurance is performed
at 50% of maximal pressure, whereas we measured endur-
ance at 25% of maximal pressure. Also, previous investiga-
tions that include endurance encouraged participants to
endure as long as possible, whereas we discouraged our
participants from continuing in the task at the first sign
of discomfort. Our approach, which was adopted to pre-
vent muscle fiber damage, may have obscured any fa-
tigue that might have occurred had we performed the
V o l. 5 3 • 1 5 6 7 – 1 5 7 8 • De cemb e r 2 0 1 0
endurance task using a more traditional paradigm (e.g.,
Solomon et al., 2000).
In several cases, performance at the end of the
data collection session was better than the earlier trials.
Although these differences were slight, it is possible that
patients with muscle weakness require a gentle warm-up
foroptimal performance. This warm-up phenomenon has
been noted in congenital myotonia (Horlings et al., 2009)
and myotonic dystrophy (Cooper, Stokes, & Edwards, 1988;
De Swart, van Engelen, van de Kerkhof, & Maassen, 2004;
Versino, Rossi, Beltrami, Sandrini, & Cosi, 2002). Applying
this warm-up phenomenon to swallow function and meal-
time management in patients with OPMD, performance
may be optimized if the swallow system is warmed up
with an easy (thin) consistency, reserving thicker consis-
tencies or chopped foods for later in the meal.
Although this investigation shows that oral muscle
weakness leads to oral dysphagia, the implications are
limited by the lack of pharyngeal visualization or more
specific measures of pharyngeal physiology. The incorpo-
ration of oropharyngeal visualization would greatly en-
hance researchers’ understanding of this disease and
should be used in future investigations. Future investi-
gations should also give some consideration to oropha-
ryngeal sensory function and overall disease severity.
In conclusion, oral muscle weakness, which is noted
in patients with OPMD, results in reduced swallow func-
tion as evidenced by decreased intraoral swallow pres-
sures and swallow capacity. These deficits result in only
mild alterations to weight, yet quality of life is greatly
affected.
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Received April 13, 2009
Revision received September 28, 2009
Accepted April 5, 2010
DOI: 10.1044/1092-4388(2010/09-0068)
Contact author: Phyllis M. Palmer, Department of Speech and
Hearing Sciences, University of New Mexico, MSC01 1195,
1 University of New Mexico, Albuquerque, NM 87131.
E-mail: ppalmer @unm.edu.
V o l. 5 3 • 1 5 6 7 – 1 5 7 8 • De cemb e r 2 0 1 0
Swallow Characteristics in Patients With Oculopharyngeal Muscular Dystrophy

Phyllis M. Palmer, Amy T. Neel, Gwyneth Sprouls, and Leslie Morrison

J Speech Lang Hear Res 2010;53;1567-1578; originally published online Aug 10,
2010;
DOI: 10.1044/1092-4388(2010/09-0068)

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