Imaging Assessment of Swallowing and Speech Muscles in Inclusion Body Myositis

Advancing Bulbar Assessment in Inclusion Body Myositis Through a Multimodal Imaging Approach

This study will evaluate whether neuromuscular ultrasound and MRI can be used as imaging markers of bulbar involvement and swallowing dysfunction in individuals with inclusion body myositis (IBM). Researchers will compare imaging and clinical findings in participants with IBM with those in participants with other myopathies, ALS, PLS, and healthy volunteers.

The study is a one-time visit. Study procedures may include neurological assessments, swallowing questionnaires, tongue strength testing, neuromuscular ultrasound, and MRI. The goal is to identify more sensitive and objective ways to assess bulbar dysfunction biomarkers.

Study Overview

• Status: Recruiting
• Conditions: ALS (Amyotrophic Lateral Sclerosis); Myositis; Myopathy; Inclusion Body Myositis; IBM; PLS

Detailed Description

Researchers will evaluate ultrasound and Magnetic Resonance Imaging (MRI) as imaging markers to diagnose bulbar involvement and dysphagia in patients with IBM.

Objectives

Primary Objectives:

• Evaluate neuromuscular ultrasound as an imaging marker for assessing the presence and evaluate the severity of dysphagia and bulbar involvement in IBM
• Evaluate MRI of the bulbar region as an imaging marker for assessing the presence and evaluate the severity of dysphagia and bulbar involvement in IBM

Secondary Objectives:

• Evaluate ultrasound elastography (USE) as an imaging tool for assessing muscle stiffness in patients with IBM and compare the findings to motor neuron disease and healthy control cohorts
• Evaluate which imaging modality could be used as an early indicator of dysphagia and bulbar involvement

• Study Type: Observational
• Enrollment (Estimated): 30

Contacts and Locations

• Study Contact: Name: Joseph D Niang, B.A.; Phone Number: 410-570-3949; Email: jniang1@jh.edu
• Study Contact Backup: Name: Pamela Arrazola, M.S.; Phone Number: 410-955-0749; Email: parrazo1@jh.edu

Study Locations

• United States
  • Maryland
    • Baltimore, Maryland, United States, 21287
      • Recruiting
      • The Johns Hopkins Hospital
      • Contact: Abdullah AlQahtani, MD MPH; Phone Number: 443-819-9480; Email: aalqaht3@jh.edu

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: Yes

• Sampling Method: Non-Probability Sample

Study Population

The study population will include approximately 30 participants with inclusion body myositis (IBM), amyotrophic lateral sclerosis (ALS), or primary lateral sclerosis (PLS); non-IBM inflammatory or genetic myopathies; and healthy controls. The planned enrollment includes 10 participants with IBM and swallowing difficulties, 5 participants with IBM without swallowing difficulties, 5 participants with non-IBM inflammatory or genetic myopathies, 5 participants with bulbar ALS, and 5 healthy controls. The investigators will recruit patients primarily through the clinical practices of the principal investigator and co-investigators and through referrals. Healthy controls may be recruited from Johns Hopkins University/Johns Hopkins Hospital staff, Johns Hopkins affiliates, and family members of patients and staff. The participants will be screened to exclude pre-existing neuromuscular disorders. Healthy controls will be age-matched to the IBM cohort.

Description

Inclusion Criteria:

• Participants must have a confirmed diagnosis of inclusion body myositis (IBM), amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS), or myopathy in accordance with the respective diagnostic criteria.
• Participants with disease must have clinically relevant laboratory testing for disease biomarkers (e.g., Creatine Kinase (CK), Comprehensive Metabolic Panel (CMP), Complete Blood Count (CBC)) within the last 12 months.
• Participants must be able to remain supine for 30 minutes or longer and cannot have significant breathing difficulties.
• Healthy controls must not have any neuromuscular disease.

Exclusion Criteria:

• Unable or unwilling to provide written informed consent.
• Medical history or clinically significant physical examination or laboratory results that, in the opinion of the investigator, would render the participant unsuitable for the study.
• Participants who are unlikely to comply with the study protocol or, in the opinion of the investigator, would not be a suitable candidate for participation in the study.
• Patients with IBM, ALS, or myopathy who have another known neuromuscular condition with known bulbar involvement.
• Any patient who has undergone a procedure for dysphagia, including myotomy, dilatation, or Botox.

Study Plan

How is the study designed?

Number of groups / cohorts

4

Cohorts and Interventions

Group / Cohort
Healthy controls
Inclusion Body Myositis; Participants with diagnosis of IBM
Inflammatory or Genetic Myopathies; other types of myopathy that is not IBM
Motor neuron disease(ALS/PLS); Participants must have spastic dysarthria

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Bulbar Muscle Thickness Assessed by Neuromuscular Ultrasound	Bulbar muscle thickness will be measured using B-mode neuromuscular ultrasound in short- and long-axis views.	Day 1 (one-time study visit)
Bulbar Muscle Cross-Sectional Area Assessed by Neuromuscular Ultrasound	Cross-sectional area of the assessed bulbar muscles will be measured using neuromuscular ultrasound.	Day 1 (one-time study visit)
Bulbar Muscle Echogenicity Assessed by Quantitative Grayscale Ultrasound	Bulbar muscle echogenicity will be evaluated using quantitative grayscale analysis of neuromuscular ultrasound images.	Day 1 (one-time study visit)
Bulbar Muscle Echogenicity Assessed by the Heckmatt Grading Scale	Bulbar muscle echogenicity will be assessed using the Heckmatt grading scale on neuromuscular ultrasound images. Scale 1-4, lower score better.	Day 1 (one-time study visit)
Bulbar Muscle Volume Assessed by Magnetic Resonance Imaging	Bulbar muscle volume will be measured from magnetic resonance imaging and reported in cubic centimeters (cm³).	Day 1 (one-time study visit)
Bulbar Muscle Cross-Sectional Area Assessed by Magnetic Resonance Imaging	Bulbar muscle cross-sectional area will be measured from magnetic resonance imaging and reported in square centimeters (cm²).	Day 1 (one-time study visit)
Bulbar Muscle Fat Content Assessed by Magnetic Resonance Imaging	Bulbar muscle fat content will be measured using magnetic resonance imaging and reported as a percentage (%).	Day 1 (one-time study visit)
Tongue Muscle Stiffness Assessed by Ultrasound Elastography	Tongue muscle stiffness will be quantitatively assessed using shear-wave ultrasound elastography.	Day 1 (one-time study visit)

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Center for Neurologic Study Bulbar Function Scale Score	Bulbar function will be assessed using the Center for Neurologic Study Bulbar Function Scale (CNS-BFS), a 21-item patient-reported questionnaire evaluating speech, swallowing, and salivation. Score ranging from 21 to 112, where a score of 21 indicates no symptoms of bulbar dysfunction.	Day 1 (one-time study visit)
Neuromuscular Disease Swallowing Status Scale Score	Swallowing function will be assessed using the Neuromuscular Disease Swallowing Status Scale (NdSSS), an 8-stage clinician-rated scale ranging from normal oral intake to complete dependence on non-oral feeding. Stage 8 represents normal function, while Stage 1 represents the most severe impairment.	Day 1 (one-time study visit)
Eating Assessment Tool-10 Score	Dysphagia symptoms and the symptoms impact on quality of life will be assessed using the 10-item Eating Assessment Tool (EAT-10). Total score ranges from 0 to 40, with a score of 3 or higher indicating a potential swallowing problem.	Day 1 (one-time study visit)
Sydney Swallow Questionnaire Score	Severity of oropharyngeal dysphagia symptoms will be assessed using the 17-item Sydney Swallow Questionnaire (SSQ). Score range 0 to 1,700, with higher scores indicating more severe swallowing problem.	Day 1 (one-time study visit)
Maximum Tongue Strength Assessed by the Iowa Oral Performance Instrument	Maximum tongue strength will be measured using the Iowa Oral Performance Instrument (IOPI). Three trials will be performed, and the highest pressure will be recorded in kilopascals (kPa).	Day 1 (one-time study visit)
Tongue Endurance Assessed by the Iowa Oral Performance Instrument	Tongue endurance will be measured using the Iowa Oral Performance Instrument (IOPI) as the amount of time, in seconds, the participant can maintain 50% of their maximum tongue pressure.	Day 1 (one-time study visit)
Inclusion Body Myositis Functional Rating Scale Score	Functional status in participants with inclusion body myositis will be assessed using the Inclusion Body Myositis Functional Rating Scale (IBMFRS), including swallowing-related function. Score ranges from 0 to 40, higher score better.	Day 1 (one-time study visit)
Amyotrophic Lateral Sclerosis Functional Rating Scale Score	Functional disability and disease severity in participants with amyotrophic lateral sclerosis will be assessed using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS). Score ranges from 0 (maximum disability/worst performance) to 48 (normal function).	Day 1 (one-time study visit)

Collaborators and Investigators

• Sponsor: Johns Hopkins University
• Investigators: Principal Investigator: Abdullah Z AlQahtani, MD MPH, the Johns Hopkins School of Medicine

Publications and helpful links

General Publications

• Jackson CE, Barohn RJ, Gronseth G, Pandya S, Herbelin L; Muscle Study Group. Inclusion body myositis functional rating scale: a reliable and valid measure of disease severity. Muscle Nerve. 2008 Apr;37(4):473-6. doi: 10.1002/mus.20958.
• Wada A, Kawakami M, Liu M, Otaka E, Nishimura A, Liu F, Otsuka T. Development of a new scale for dysphagia in patients with progressive neuromuscular diseases: the Neuromuscular Disease Swallowing Status Scale (NdSSS). J Neurol. 2015 Oct;262(10):2225-31. doi: 10.1007/s00415-015-7836-y. Epub 2015 Jul 4.
• Reyngoudt H, Baudin PY, Caldas de Almeida Araujo E, Bachasson D, Boisserie JM, Mariampillai K, Annoussamy M, Allenbach Y, Hogrel JY, Carlier PG, Marty B, Benveniste O. Effect of sirolimus on muscle in inclusion body myositis observed with magnetic resonance imaging and spectroscopy. J Cachexia Sarcopenia Muscle. 2024 Jun;15(3):1108-1120. doi: 10.1002/jcsm.13451. Epub 2024 Apr 13.
• McIlduff CE, Martucci MG, Shin C, Qi K, Pacheck AK, Gutierrez H, Mortreux M, Rutkove SB. Quantitative ultrasound of the tongue: Echo intensity is a potential biomarker of bulbar dysfunction in amyotrophic lateral sclerosis. Clin Neurophysiol. 2020 Oct;131(10):2423-2428. doi: 10.1016/j.clinph.2020.06.027. Epub 2020 Jul 17.
• Anderson NC, Lloyd TE. Inclusion body myositis: an update. Curr Opin Rheumatol. 2025 Jan 1;37(1):80-85. doi: 10.1097/BOR.0000000000001060. Epub 2024 Oct 21.

Study record dates

Study Major Dates

• Study Start (Estimated): October 1, 2026
• Primary Completion (Estimated): December 31, 2027
• Study Completion (Estimated): December 31, 2027

Study Registration Dates

• First Submitted: September 11, 2026
• First Submitted That Met QC Criteria: September 11, 2026
• First Posted (Actual): September 16, 2026

Study Record Updates

• Last Update Posted (Actual): September 16, 2026
• Last Update Submitted That Met QC Criteria: September 11, 2026
• Last Verified: September 1, 2026

More Information

Terms related to this study

• Keywords: swallowing; weakness; tongue weakness; bulbar weakness
• Additional Relevant MeSH Terms: Musculoskeletal Diseases; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Metabolic Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Motor Neuron Disease; Pathological Conditions, Signs and Symptoms; Nutritional and Metabolic Diseases; Signs and Symptoms; Muscular Diseases; Amyotrophic Lateral Sclerosis; Asthenia; Myositis; Myositis, Inclusion Body
• Other Study ID Numbers: IRB00553575

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No