Imaging Assessment of Swallowing and Speech Muscles in Inclusion Body Myositis

NCT07823101 | Recruiting

• 1 other identifier: IRB00553575
• Study Type: observational
• Target: 30
• Locations: 1 country
• Sites: 1

Brief 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). 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.

Conditions

IBM; Myopathy; ALS (Amyotrophic Lateral Sclerosis); Inclusion Body Myositis; Myositis; PLS

Keywords

swallowing; tongue weakness; bulbar weakness; weakness

Outcome Measures

Primary Outcomes (8)

• 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 Outcomes (8)

• Center for Neurologic Study Bulbar Function Scale Score

  Day 1 (one-time study visit)

• Neuromuscular Disease Swallowing Status Scale Score

  Day 1 (one-time study visit)

• Eating Assessment Tool-10 Score

  Day 1 (one-time study visit)

• Sydney Swallow Questionnaire Score

  Day 1 (one-time study visit)

• Maximum Tongue Strength Assessed by the Iowa Oral Performance Instrument

  Day 1 (one-time study visit)

Study Arms (4)

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

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• 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.

You may qualify if:

• 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.

You may not qualify if:

• 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.

Sponsors & Collaborators

• Johns Hopkins University: lead

Study Sites (1)

The Johns Hopkins Hospital

Baltimore, Maryland, 21287, United States

RECRUITING

Related Publications (5)

• 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.

  PMID: 18236463 BACKGROUND

• 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.

  PMID: 26142025 BACKGROUND

• 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.

  PMID: 38613252 BACKGROUND

• 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.

  PMID: 32828046 BACKGROUND

• 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.

  PMID: 39469805 BACKGROUND

MeSH Terms

Conditions

Myositis, Inclusion Body; Muscular Diseases; Myositis; Amyotrophic Lateral Sclerosis; Asthenia

Condition Hierarchy (Ancestors)

Musculoskeletal Diseases; Neuromuscular Diseases; Nervous System Diseases; Spinal Cord Diseases; Central Nervous System Diseases; Motor Neuron Disease; Neurodegenerative Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases; Signs and Symptoms; Pathological Conditions, Signs and Symptoms

Study Officials

• Abdullah Z AlQahtani, MD MPH

  the Johns Hopkins School of Medicine

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Joseph D Niang, B.A.

CONTACT

410-570-3949; jniang1@jh.edu

Pamela Arrazola, M.S.

CONTACT

410-955-0749; parrazo1@jh.edu

Study Design

• Study Type: observational
• Observational Model: OTHER
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: September 11, 2026
• First Posted: September 16, 2026
• Study Start: October 1, 2026
• Primary Completion (Estimated): December 31, 2027
• Study Completion (Estimated): December 31, 2027
• Last Updated: September 16, 2026

Record last verified: 2026-09

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)