Myopathies featuring early or prominent dysphagia.

James D. Triplett, BM, MMED1, Marcus V. Pinto, MD, MS1, Emily A. Hosfield, MS, CCC-SLP2,

Margherita Milone, MD, PhD1, Teerin Liewluck, MD1

1 – Division of Neuromuscular Medicine, Department of Neurology, Mayo Clinic, Rochester,

Minnesota, USA

2 – Division of Speech Pathology, Department of Neurology, Mayo Clinic, Rochester, Minnesota,

USA

Keywords:Dysphagia, inclusion body myositis, muscular dystrophy, myopathy, myositis,

videofluoroscopy

Running title: Myopathy and dysphagia

Funding:

No funding as receieved for this manuscript.

Discolosures and conflict of interest:

This article has been accepted for publication and undergone full peer review but has not been
through the copyediting, typesetting, pagination and proofreading process which may lead to
differences between this version and the Version of Record. Please cite this article as doi:
10.1002/mus.26996

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M Milone receives compensation as an associate editor of Neurology Genetics and research

support from Mayo Clinic benefactors. The remeianing authors have no conflicts of interest.

Correspondence to:

Teerin Liewluck

Department of Neurology, Mayo Clinic

200 1st St SW. Rochester Minnesota. 55905

United States of America

E-mail: liewluck.teerin@mayo.edu

A preliminary analysis of these findings was presented at the 24th International Annual

Congress of the World Muscle Society (WMS) in October 2019 in Copenhagen, Denmark.

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Abstract:

Introduction:Limited data exists regarding myopathies with early or prominent dysphagia.

Methods:A retrospective study was performed (January 2002 -August 2019) to identify myopathy

patients in whom dysphagia was the initial symptom or was disproportionately severe compared

to limb weakness.

Results:Thirty-two patients were identified.The median age at diagnosis was 65 years (range 36-

80). Inclusion body myositis (IBM) (n=15), immune-mediated necrotizing myopathy (IMNM) (n=5)

and oculopharyngeal muscular dystrophy (n=4), were the most common diagnoses. In 4 patients

(3 IMNM and 1 nonspecific myositis) dysphagia evolved rapidly. At evaluation, 21 patients

required diet alterations, 5 required feeding tubes and 8 had aspiration pneumonia. Follow-up

data was available for 20 patients (median 24 months). Eight patients received

immunosuppressive therapies with improvement in 7, including 3 of 4 with rapidly progressive

dysphagia.

Discussion:IBM and IMNM accounted for approximately two-thirds of patients with early or

prominent dysphagia at our institution. Rapidly progressive dysphagia may predict

immunotherapy responsiveness.

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Introduction

Dysphagia is a common problem in clinical practice and can be a presentation of either

neurological or non-neurological disorders [1]. Among neurological disorders, various

neuromuscular diseases, including motor neuron diseases, myasthenia gravis (MG),

polyradiculoneuropathy and myopathies, can initially present with dysphagia. Among myopathies,

dysphagia may be a core clinical feature as can be seen in oculopharyngeal muscular dystrophy

(OPMD), or may be a part of more widespread weakness as can be seen in other myopathies,

such as immune-mediated myopathies, sporadic late onset nemaline myopathy (SLONM) and

light chain (AL) amyloidosis-associated myopathy [2-7].

In inclusion body myositis (IBM), dysphagia is present in 40% of patients at the time of diagnosis

[2,8,9], and is the sole or most prominent symptom at presentation in up to 10% [10-12]. The

presence of dysphagia in inflammatory myopathies has been reported in association with an

increased mortality rate, nutritional deficits, aspiration pneumonia, decreased quality of life, and

need for diet modification and feeding tube placement [12,13]. A formal swallow evaluation such

as a video fluoroscopic swallow study (VFSS) or esophageal manometry can identify these

abnormalities [3].

Though various myopathies are associated with dysphagia, their relative prevalence amongst

patients presenting with early or prominent dysphagia is lacking. We undertook a retrospective

study to identify the underlying etiologies and outcomes in myopathy patients with early or

prominent dysphagia at a tertiary neuromuscular referral center.

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Methods:

We searched the Mayo clinic computerized database between January 1 2003 to August 30, 2019

for patients aged over 18 years of age diagnosed with a myopathy in which dysphagia was the

initial symptom or was disproportionately severe compared to limb weakness at initial

presentation. The latter was defined as evidence of aspiration on swallowing study, required diet

modification, or procedural intervention (percutaneous endoscopic gastrostomy, esophageal

dilation or cricopharyngeal myotomy), while maintaining ambulatory independence. Patients were

identified from electronic medical records by searching for those with a diagnosis of myopathy,

using the search terms “myopathy,” “myositis” and “muscular dystrophy.” We further narrowed the

search to include only patients who also underwent either video swallow assessment or formal

swallow evaluation, using the procedural codes for both procedures. Patient notes were then

reviewed by one author (JT or MVP) and if they met inclusion criteria were also reviewed by a

second author (JT,MVP or TL) for acceptance into the study. Patients were excluded if an

alternative cause of dysphagia, such as structural abnormalities unrelated to the myopathy, prior

neck radiotherapy or an alternate neuromuscular condition was present.

Ethics approval was granted by the Mayo Clinic Rochester institutional review board.

Retrospective chart review was undertaken. Information extracted included clinical features,

diagnoses, results of diagnostic studies including electrodiagnostic studies, serological

investigations, muscle pathological findings, clinical dysphagia examinations, and dysphagia

specific diagnostic studies. Patient outcomes including responses to immunosuppressive

therapies, and interventional procedures were assessed by reviewing follow-up clinical

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examination reports

Dysphagia evaluations were performed by occupational therapists and / or speech pathologists

and included review of medical history, patient symptoms, assessment of oral motor control,

phonation, respiratory control, and an oral intake trial. Examination included trials of

compensatory techniques, identification of appropriate rehabilitation interventions, and multiple

barium or food consistencies. Improvements following interventions and immunomodulatory

therapy were graded partial or complete, with complete defined as resumption of a normal diet.

Continuous variables were summarized as medians and ranges. Categorical variables were

summarized as frequencies and percentages. Fisher’s exact test was used for comparing

frequencies. Significance level was set at p < 0.05. Data analyses were performed using JMP

statistical software (version 14, SAS Institute Inc, Cary, NC)

Results:

We identified 32 patients, in 17 of whom dysphagia was the initial symptom and in 15 of whom it

was the most prominent symptom (Figure 1). The median age in patients with dysphagia as the

initial symptom was 66.5 years (range 36-80) and 7 of 17 patients were female. The median age

in patients with dysphagia as the most prominent symptom was 61 years (range 41-78) and 6 of

15 patients were female. The median time from dysphagia onset to evaluation at our institute was

36 months (range 6–300) for patients with initial dysphagia and 24 months (range 1-240) amongst

patients with prominent dysphagia. A mixture of hereditary and acquired myopathies were

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identified. IBM, nonspecific myositis, immune mediated necrotizing myopathy (IMNM) with and

without immune checkpoint inhibitor (ICI) exposure and OPMD were the most common

diagnoses, each with unique clinical and investigatory findings (Table 1). Patients with prominent

dysphagia had a higher frequency of ophthalmoparesis than those who initially presented with

dysphagia (7 versus 2; p-value <0.05).

Ophthalmoparesis was identified in 9 patients, accompanied by ptosis in 5 patients (2 OPMD, 1

ICI-associated IMNM, 1 genetically uncharacterized hereditary myopathy and 1 mitochondrial

DNA (mtDNA) deletion-related mitochonrial myopathy) and without ptosis in 4 patients (2 OPMD,

2 ICI-associated IMNM). Ptosis alone was identified in 2 patients (1 ICI-associated IMNM and 1

DNA polymerase gamma (POLG)-related mitochondrial myopathy). Limb weakness was present

in 32 patients, though no patient had isolated distal weakness. Disproportionate weakness of the

deep finger flexors compared to finger extensors was present in 12 of 15 IBM patients and not

seen in other diseases. Facial weakness was present in 14 patients (4 OPMD, 4 IBM, 2 IMNM, 1

ICI-associated IMNM, 1 nonspecific myositis, 1 hereditary myopathy of unknown genetic defect

and 1 mitochondrial myopathy).

Among the 17 patients with dysphagia at onset, 13 patients reported limb weakness at clinical

evaluation, which followed the onset of dysphagia by a median of 12 months (2-288 months).

Four patients [3 IBM and 1 congenital fibre type disproportion (CFTD)] reported no limb weakness

at 12-72 months after onset of dysphagia; however, 2 of IBM patients and the CFTD patient were

found to have weakness on exam.

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Dysphagia evaluation:

The most frequently reported dysphagia-related symptoms were difficulty with dry and solid foods

(97%) and coughing while eating (69%). Eight patients reported a history of pneumonia, and 10

noted weight loss secondary to reduced food intake due to dysphagia. Formal dysphagia

evaluation findings in common causes of dysphagia and myopathy are presented in the

supplemental table. At the time of evaluation, 21 patients required diet alterations, and 5 had

required feeding tubes [3 percutaneous endoscopic gastrostomy (PEG) tube and 2 nasogastric

tube (NGT)]. Clinical examination revealed oral motor control abnormalities in 18 patients (56%)

and laryngeal abnormalities in 17 patients (53%). Thirty patients (94%) underwent formal

dysphagia evaluations.

All patients, except for 1 with ICI-associated IMNM, underwent VFSS. VFSS was abnormal in

those tested patients (Supplementary Table 1),with the pharyngeal phase affected more

frequently and severely than the oral phase. Cricopharyngeal prominence was observed in 15

patients (10 IBM, 4 OPMD, 1 IMNM). Following vidoeflusocopy and swallow evaluation, 17

patients (53%) received recommendations regarding further diet alterations. Other diagnostic

procedures were performed in 11 patients (34%) including double contrast esophagram (8), fiber

optic nasopharyngeal endoscopy (2), esophageal manometry (2) and upper endoscopy (1) and

revealed evidence of small sliding esophageal hiatal hernias in 3 patients, a small Zeckner

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diverticulum in 1 patient and gastro-esophageal reflux in 1 patient in addition to myopathy-related

dysphagia.

Laboratory investigations:

Creatine kinase (CK) was elevated in 22 of 31 patients (median = 258 U/L, range 42 – 4120,

normal < 177). Albumin was reduced in 9 of 23 patients (3.5 median, range 2.8 – 4.9, normal 3.4-

4.7). A normal to mildly elevated CK (1-3 times upper limit of normal) was seen in the majority of

OPMD (3 of 4) and IBM (13 of 16) patients. However the CK was more markedly elevated in

patients (> 5 times upper limit of normal) with nonspecific myositis and IMNM (median 2029 U/L).

Electrodiagnsotic studies:

Nerve conduction studies (NCS) and needle electromyography (EMG) were performed in 31 of

32 patients and revealed short duration, polyphasic motor unit potentials in all patients, including

1 IBM patient without clinical evidence of limb weakness . Facial and proximal upper limb muscles

were most severely affected in patients with OPMD with more diffuse abnormalities identified in

IBM, IMMN and nonspecific myositis. Mixed short-duration, low-amplitude and long-duration,

high-amplitude motor unit potentials were identified in 5 patients, including 3 IBM, 1 ICI-related

IMNM and 1 SLONM. Flexor pollicus longus or flexor digitorum profundus were sampled in 12/15

IBM patients and were the most abnormal muscle sampled in 11, including 1 patient without

weakness on the exam. Ten patients had evidence of an axonal predominant peripheral

neuropathy on NCS [4 IBM, 3 IMNM (1 ICI-related), 1 AL amyoid myopathy, 1 SLONM, and 1

POLG-mitochondrial myopathy), two of whom also had diabetes (1 AL amyloid myopathy and 1

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IBM). Low frequency repetitive nerve stimulation was normal in the 18 patients in whom it was

performed.

Follow up and treatment responses:

Follow-up data was available in 20 patients with a median follow-up period of 24 months (range

1-180 months). Dysphagia evolved rapidly, leading to diet alterations within 6 months of symptom

onset in 4 patients (3 IMNM and 1 nonspecific myositis). Two patients (1 AL amyloid myopathy

and 1 ICI-associated IMNM) died within 3 months of dysphagia onset, due to systemic illnesses.

The 2 patients diagnosed with ICI-associated IMNM discontinued their ICIs following the

development of dysphagia. Immunomodulatory therapy was commenced in 8 patients who had

follow up (Table 2). Therapeutic responses varied depending on underlying etiology with some

improvement in dysphagia seen in 7 of 8 patients.,None of these patients underwent esophageal

dilation or cricopharyngeal myotomy. Despite clinical improvement following immunosuppressive

therapy, one patient (Patient 6) with ICI-IMNM had withdrawal of care following an episode of

aspiration penumonia. Three IBM patients received immunosuppressive therapy, with two

patients (Patients 2 and 3) having partial improvement. Patient 2 presented with dysphagia to

solids and liquids. Following prednisolone (50 mg/day), there was intial improvement in dysphagia

for 3 months followed by deterioration to the original level of dysfunction. Patient 3 noted

dysphagia with soft foods and liquids at presentation and dysphagia improved following 30mg

daily prednisolone, allowing the patient to eat a near normal diet without dysphagia to liquids.

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Interventional procedures were performed in 12 patients (36%) and most frequently performed in

patients with IBM (8 patients). Cricopharyngeal myotomy was performed in 6 patients (4 IBM, 1

OPMD, 1 non-specific myositis) and esophageal dilations were performed in 7 patients (6 IBM, 1

OPMD). No patient had sustained improvement in the degree of dysphagia following

cricopharyngeal myotomy or eseophageal dilatation. During their clinical course, 7 patients

required PEG (4IBM, 2 OPMD and one IMNM) and 3 other patients required NGT (1 ICI-

associated IMMN, 1 nonspecific myositis and 1 AL amyloid myopathy).

Discussion:

Three-quaters of myopathy patients presenting with dysphagia as an initial or prominent symptom

fell into one of three groups, IBM, IMNM, and OPMD, with nearly two-thirds having IBM or IMNM.

Patients in whom dysphagia was the initial symptom at presentation had a longer time to diagnosis

from dysphagia onset than those with more diffuse symptoms (36 vs 24 months). This may be

secondary to a limited appreciation of myopathies causing dysphagia, especially when no or

minimal limb weakness is present.

Thoughtful history taking and clinical exam may allow clinicians to determine a myopathic cause

of dysphagia and may potentially ascertain the underlying diagnosis at the time of presentation.

Our data suggests the presence of a family history of dysphagia or presence of ptosis and

ophthalmoparesis is strongly suggestive of OPMD. An acute or subacute clinical course is more

typical of IMMN or a non-specific myositis. IBM may present with chronic isolated dysphagia [11],

but is generally accompanied by finger flexor and quadriceps weakness, though 2 patients with

IBM in this cohort had prominent facial diplegia which is rarely reported as a presenting complaint

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in IBM, and may be underappreciated on clinical exam [14]. No IBM patient in our series had

NT5c1A antibody testing performed, the presence of which was reported to have a higher rate of

dysphagia [15].

CK values can be a helpful diagnostic tool in the evaluation of myopathy patients with prominent

or early dysphagia. The presence of a moderate to markedly elevated CK (> 5 times upper limit

of normal) in the setting of subacute dysphagia should alert phsyicians to consider IMNM or

nonspecific myositis as the cause, while normal or milder elevations of CK (1-3 times upper limit

of normal) and more chronic history are suggestive of IBM and OPMD.

Evaluation with NCS and needle EMG in this cohort not only confirmed the presence of a

myopathic process but gave diagnostic information regarding the underlying myopathy. Needle

EMG was particularly useful in cases with minimal or no clinical weakness. Repetitive nerve

stimulation is particularly important in OPMD patients given its weakness pattern mimicking

myasthenia gravis. EMG findings in this cohort suggest forearm flexor muscles should be routinely

included in EMG of patients presenting with early or prominent dysphagia and the presence of

more severe myopathic changes seen in the forearm flexors should raise suspicion for IBM.

Formal dysphagia evaluation should be performed in patients presenting with dysphagia as

further information regarding the underlying etiology may be suggested. Although coexistent

pharyngeal dysfunction and a prominent cricopharyngeus can be associated with a variety of

neuromuscular disorders, limited evidence is available as how to utilize these findings when

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suspecting a myopathy. In our cohort a prominent cricopharyngeus was noted exclusively with

IBM and OPMD patients, potentially reflecting chronic disease and though not specific for any

entity, its presence should alert clinicians to consider IBM or OPMD in the appropriate clinical

context.

Various treatment modalities to manage myopathy-associated dysphagia often requires input

from both medical practitioners and occupational therapists or speech pathologists.

Conservative measures include dietary modifications and swallowing therapy [4]. Diet

alterations and exercises were recommended in a majority of patients in this cohort following

swallow assessment, though persistent improvements in degree of dysphagia were lacking. In

addition to conservative measures, immunomodulatory therapy and surgical interventions may

also be undertaken. In our cohort, IBM patients had a short follow up with mixed responses to

immunomodulatory therapy, similar to prior reports [9]. Intravenous immunoglobulins (IVIG)

therapy has been reported to be effective for dysphagia in few patients with IBM, with greater

benefits reported when combined with oesophageal balloon dilatation [9, 17]. Though two

patients in this series had imporved dysphagia following steroid therapy, the response was

short. The majority of evidence suggests that IBM is not steroid responsive [18], and multicenter

randomized controlled trials are necessary to verify the long-term efficacy of immunomodulatory

therapy in IBM patients with severe dysphagia.

Surgical interventions including cricopharyngeal myotomy and PEG tube insertion are

recommended when conservative therapies fail or when severe weight loss and malnutrition are

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apparent [3]. Cricopharyngeal myotomy is believed to improve bolus movement through the

esophagus by decreasing pressure which the propulsive force of the pharyngeal constrictors

work against [3]. Outcomes were poor following cricopharyngeal myotomy and esophageal

balloon dilatation in this cohort with 3 of 4 IBM patients who underwent cricopharyngeal

myotomy requiring PEG tube insertion. The poor outcome following cricopharyngeal myotomy

observed in our cohort should be taken with caution given our limited patient numbers, as

benefits were previously reported in up to 60% of patients[19,20,21]. However, cricopharyngeal

myotomy may be less effective when it is performed in patients with abnormal hyolaryngeal

excursion or hyporeflexic upper esophageal sphincters or when a hiatal hernia is present [3, 21,

22].

The progression of dysphagia may also help in defining the underlying diagnosis. Four patients

in this series had rapid onset and increasing severity of symptoms and presented within 6 months

of symptom onset, 3 with IMNM, and one with nonspecific myositis. All of these were treated with

immunomodulatory therapy and 3 returned to normal, suggesting rapid onset of severe dysphagia

is likely immune-mediated. In such cases early commencement of immunomodulatory therapy is

recommended.

Limitations of this study include its retrospective design leading to patient's receiving inconsistent

investigations, and management strategies at the discretion of the treating neurologists. The study

is also limited by a referral bias given the tertiary nature of our center and thus the referral

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population having often previously being assessed by a neurologist. The geographical location of

our center led to a lower rate of OPMD than one would expect in regions of higher prevalence of

OPMD.

Despite these limitations we describe the importance of a formal swallow evaluation and utility

of ancillary testing including vidoefluroscopic studies and EMG in identifying primary myopathic

conditions in patients in whom dysphagia is the primary symptom or when limb weakness is

absent or subtle. Based on our experience, we present a suggested algorithm for the

evaluation of patients with early or prominent myopathic dysphagia (Figure 3). Early evaluation

of patients with dysphagia may allow the identification of characteristic abnormalities consistent

with underlying muscle pathology, which can lead to confirmatory investigations, diagnosis and

treatment.

Abbreviations:

AL, light chain amyloidosis; CFTD, congenital fiber type disproportion; CK, creatine kinase;

EMG, needle electromyogrphay; IBM, inclusion body myositis; ICI, immune checkpoint inhibitor;

IMNM, immune-mediated necrotizing myopathy; IVIG, intravenous immunoglobulins; MG,

myasthenia gravis; mtDNA, mitochondrial DNA; NCS, nerve conduction studies; NGT,

nasogastic tube; OPMD, oculopharyngeal muscular dystrophy; PEG, ercutaneous endoscopic

gastrostomy; POLG, DNA polymerase gamma; SLONM, sporadic late onset nemaline

myopathy; VFSS, video fluoroscopic study

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Figure titles and legends:

Figure 1: Diagnostic spectrum of myopathies with early or prominent dysphagia.

Figure 1: Diagnostic spectrum of myopathies with early or prominent dysphagia.

AL amyloid myopathy, immunoglobulin light chain amyloidosis-associated myopathy;

CFTD, congenital fibre type disproportion; IBM, inclusion body myositis; IMNM, immune-

mediated necrotizing myopathy (2 patients with immune checkpoint inhibitor (ICI)

exposure); OPMD, oculopharyngeal muscular dystrophy; SLONM, sporadic late onset

nemaline myopathy; Mitochondrial myopathies were secondary to POLG mutation (n=1)

and mtDNA deletion (n=1).

Figure 2: Algorithm for evaluating myopathy presenting with prominent

dysphagia based on the current cohort

Figure 2: Algorithm for evaluating myopathy presenting with prominent

dysphagia based on the current cohort. IBM inclusion body myositis, ICI immune

checkpoint inhibitor, IMNM immune mediated necrotizing myopathy, OPMD

oculopharyngeal muscular dystrophy, RNS repetitive nerve stimulation, +ve positive, -ve

negative.

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Accepted Article

Figure 1: Diagnostic spectrum of myopathies with early or prominent dysphagia. AL amyloid myopathy,
immunoglobulin light chain amyloidosis-associated myopathy; CFTD, congenital fibre type disproportion;
IBM, inclusion body myositis; IMNM, immune-mediated necrotizing myopathy (2 patients with immune
checkpoint inhibitor (ICI) exposure); OPMD, oculopharyngeal muscular dystrophy; SLONM, sporadic late
onset nemaline myopathy; Mitochondrial myopathies were secondary to POLG mutation (n=1) and mtDNA
deletion (n=1).

165x129mm (96 x 96 DPI)

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Accepted Article

Algorithm for evaluating myopathy presenting with prominent dysphagia based on the current cohort.

1905x1049mm (72 x 72 DPI)

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 Table 1: Findings in common myopathies with prominent dysphagia.

Diagnosis History Examination (n) Median CK EMG findings (n)

(range) (U/L)
IBM (15) Finger flexor > finger extensor Preferential finger flexor weakness 290 (42- Mixed neurogenic-myopathic
weakness (12/15) 1113) abnormalities (3)
Chronic symptom progression Facial weakness occasionally seen Forearm flexors most abnormal
Accepted Article

IMNM and Sub-acute symptom onset
nonspecific May be rapidly progressive
myositis (5) Often immunotherapy responsive
ICI associated Sub-acute onset
IMNM (2) History of ICI exposure
Rapidly progressive
Often immunotherapy responsive
OPMD (4) Chronic symptom progression
Family history
Limited or no limb weakness
CK creatine kinase; IBM inclusion body myositis; ICI immune checkpoint inhibitor; IMNM immune mediated necrotizing myopathy; OPMD
oculopharyngeal muscular dystrophy.
(4/15)
Prominent or enlarged
cricopharyngeus muscle (10/15)
Proximal predominant weakness (5)
Oculobulbar and proximal weakness
(2)
Ptosis and/or ophthalmoparesis (4)
Mild proximal limb weakness
Prominent cricopharyngeus muscle
(4)
(11/12)
Concomitant axonal predominant
peripheral neuropathy (3)
Fibrillation potentials (13/15)
2029 (572- Diffuse myopathic abnormalities (5)
4120) Fibrillation potentials (5)
(72- 218) Mixed neurogenic-myopathic
abnormalities (1)
Concomitant axonal predominant
peripheral neuropathy (1)
202 (67-415) Myopathic abnormalities limited to
facial and proximal upper limbs (4)
No fibrillation potentials(3)

John Wiley
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 Table 2: Responses following immunotherapy administration:

Patient Age at Sex Diagnosis Diet before Prior Treatment Follow up Outcome

diagnosis treatment procedure length

(years) (months)

1 48 Female IBM Puree diet Methotrexate, 33 No change

Accepted Article

mycophenolate

2 76 Female IBM Puree diet Prednisolone 6 Partial

improvement

3 65 Female IBM Puree diet Prednisolone 6 Partial

improvement

4 65 Female IMNM PEG PEG IVIG (prednisolone 16 Complete

no response) improvement

5 46 Female IMNM NGT NGT IVIG, 14 Complete

prednisolone, improvement

methotrexate

6 78 Male ICI- IMNM NGT NGT IVMP, PLEX 1 Partial

improvement

7 55 Male ICI- IMNM Nectar thick IVIG 4 Complete

liquids improvement

8 56 Female Non-specific Puree diet Prednisolone, 6 Complete

myositis methotrexate improvement

IBM inclusion body myositis; ICI immune checkpoint inhibitor; IMNM immune mediated necrotizing

myopathy; IVIG intravenous immunoglobulin; IVMP intravenous methylprednisolone; NGT nasogastric

tube; PEG percutaneous gastroscopy; PLEX plasma exchange.

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