IBMNCT07823101
Imaging Assessment of Swallowing and Speech Muscles in Inclusion Body Myositis
An observational study of IBM and Inclusion Body Myositis, run by Johns Hopkins University at 1 site in United States.
Trial summary
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). Read more about this study
- Who is it for?
- Ages 18 years and olderHealthy volunteers can take part
- Conditions the study is for
- IBM, Inclusion Body Myositis, Myopathy
- How many people
- 30 participants
- Where
- 1 siteUnited States
- Study dates
- Oct 2026to 31 Dec 2027
- Phase
- Observational
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- Healthy volunteers accepted
- Adult, Older adult
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.
Who may be able to join
- 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.
Who cannot join
- 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.
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
United States1 site, 1 recruiting
- Recruiting
The Johns Hopkins Hospital
Baltimore, Maryland, 21287
Abdullah AlQahtani, MD MPH, 443-819-9480, aalqaht3@jh.edu
About this study
Official title: 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.
Read the 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
- Conditions
- IBM, Inclusion Body Myositis, Myopathy, Myositis, ALS (Amyotrophic Lateral Sclerosis), PLS
- Keywords
- swallowing, tongue weakness, bulbar weakness, weakness
- Medical subject headings
- Myositis, Inclusion Body, Muscular Diseases, Myositis, Amyotrophic Lateral Sclerosis, Asthenia
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| 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 | |
| Healthy controls | Not described |
What is being measured
The primary outcome is the main question the study is designed to answer.
Bulbar Muscle Thickness Assessed by Neuromuscular Ultrasound
Bulbar muscle thickness will be measured using B-mode neuromuscular ultrasound in short- and long-axis views.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: 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).
Measured: 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).
Measured: 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 (%).
Measured: 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.
Measured: Day 1 (one-time study visit)
Secondary outcomes (8)
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.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: 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).
Measured: 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.
Measured: 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.
Measured: 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).
Measured: Day 1 (one-time study visit)
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted16 Sep 2026
- Study startOct 2026 (estimated)
- Primary completion31 Dec 2027 (estimated)
- Study completion31 Dec 2027 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (5)Papers the sponsor has linked to this study.
- Other trials for IBMStudies of the same condition, open and under way.
- More trials run by Johns Hopkins UniversityThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Other
- Time perspective
- Prospective
- Enrolment
- 30 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Johns Hopkins University
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Abdullah Z AlQahtani, MD MPH, Principal investigator, the Johns Hopkins School of Medicine
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07823101
- Sponsor's protocol ID
- IRB00553575
- Status last verified
- Sep 2026
- First submitted
- 11 Sep 2026
- Last updated
- 16 Sep 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
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.
PubMed 18236463Wada 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.
PubMed 26142025Reyngoudt 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.
PubMed 38613252McIlduff 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.
PubMed 32828046Anderson 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.
PubMed 39469805