Imaging Assessment of Swallowing and Speech Muscles in Inclusion Body Myositis
11 septembre 2026 mis à jour par: Johns Hopkins University
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.
Aperçu de l'étude
Statut
Recrutement
Description détaillée
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
Type d'étude
Observationnel
Inscription (Estimé)
30
Contacts et emplacements
Cette section fournit les coordonnées de ceux qui mènent l'étude et des informations sur le lieu où cette étude est menée.
Coordonnées de l'étude
- Nom: Joseph D Niang, B.A.
- Numéro de téléphone: 410-570-3949
- E-mail: jniang1@jh.edu
Sauvegarde des contacts de l'étude
- Nom: Pamela Arrazola, M.S.
- Numéro de téléphone: 410-955-0749
- E-mail: parrazo1@jh.edu
Lieux d'étude
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Maryland
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Baltimore, Maryland, États-Unis, 21287
- Recrutement
- The Johns Hopkins Hospital
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Contact:
- Abdullah AlQahtani, MD MPH
- Numéro de téléphone: 443-819-9480
- E-mail: aalqaht3@jh.edu
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Critères de participation
Les chercheurs recherchent des personnes qui correspondent à une certaine description, appelée critères d'éligibilité. Certains exemples de ces critères sont l'état de santé général d'une personne ou des traitements antérieurs.
Critère d'éligibilité
Âges éligibles pour étudier
- Adulte
- Adulte plus âgé
Accepte les volontaires sains
Oui
Méthode d'échantillonnage
Échantillon non probabiliste
Population étudiée
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.
La 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.
Plan d'étude
Cette section fournit des détails sur le plan d'étude, y compris la façon dont l'étude est conçue et ce que l'étude mesure.
Comment l'étude est-elle conçue ?
Détails de conception
Cohortes et interventions
Groupe / Cohorte |
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Contrôles sains
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Inclusion Body Myositis
Participants with diagnosis of IBM
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Inflammatory or Genetic Myopathies
other types of myopathy that is not IBM
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Motor neuron disease(ALS/PLS)
Participants must have spastic dysarthria
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Que mesure l'étude ?
Principaux critères de jugement
Mesure des résultats |
Description de la mesure |
Délai |
|---|---|---|
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Bulbar Muscle Thickness Assessed by Neuromuscular Ultrasound
Délai: Day 1 (one-time study visit)
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Bulbar muscle thickness will be measured using B-mode neuromuscular ultrasound in short- and long-axis views.
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Day 1 (one-time study visit)
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Bulbar Muscle Cross-Sectional Area Assessed by Neuromuscular Ultrasound
Délai: Day 1 (one-time study visit)
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Cross-sectional area of the assessed bulbar muscles will be measured using neuromuscular ultrasound.
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Day 1 (one-time study visit)
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Bulbar Muscle Echogenicity Assessed by Quantitative Grayscale Ultrasound
Délai: Day 1 (one-time study visit)
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Bulbar muscle echogenicity will be evaluated using quantitative grayscale analysis of neuromuscular ultrasound images.
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Day 1 (one-time study visit)
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Bulbar Muscle Echogenicity Assessed by the Heckmatt Grading Scale
Délai: Day 1 (one-time study visit)
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Bulbar muscle echogenicity will be assessed using the Heckmatt grading scale on neuromuscular ultrasound images.
Scale 1-4, lower score better.
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Day 1 (one-time study visit)
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Bulbar Muscle Volume Assessed by Magnetic Resonance Imaging
Délai: Day 1 (one-time study visit)
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Bulbar muscle volume will be measured from magnetic resonance imaging and reported in cubic centimeters (cm³).
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Day 1 (one-time study visit)
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Bulbar Muscle Cross-Sectional Area Assessed by Magnetic Resonance Imaging
Délai: Day 1 (one-time study visit)
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Bulbar muscle cross-sectional area will be measured from magnetic resonance imaging and reported in square centimeters (cm²).
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Day 1 (one-time study visit)
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Bulbar Muscle Fat Content Assessed by Magnetic Resonance Imaging
Délai: Day 1 (one-time study visit)
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Bulbar muscle fat content will be measured using magnetic resonance imaging and reported as a percentage (%).
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Day 1 (one-time study visit)
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Tongue Muscle Stiffness Assessed by Ultrasound Elastography
Délai: Day 1 (one-time study visit)
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Tongue muscle stiffness will be quantitatively assessed using shear-wave ultrasound elastography.
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Day 1 (one-time study visit)
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Mesures de résultats secondaires
Mesure des résultats |
Description de la mesure |
Délai |
|---|---|---|
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Center for Neurologic Study Bulbar Function Scale Score
Délai: Day 1 (one-time study visit)
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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.
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Day 1 (one-time study visit)
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Neuromuscular Disease Swallowing Status Scale Score
Délai: Day 1 (one-time study visit)
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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.
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Day 1 (one-time study visit)
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Eating Assessment Tool-10 Score
Délai: Day 1 (one-time study visit)
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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.
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Day 1 (one-time study visit)
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Sydney Swallow Questionnaire Score
Délai: Day 1 (one-time study visit)
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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.
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Day 1 (one-time study visit)
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Maximum Tongue Strength Assessed by the Iowa Oral Performance Instrument
Délai: Day 1 (one-time study visit)
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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).
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Day 1 (one-time study visit)
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Tongue Endurance Assessed by the Iowa Oral Performance Instrument
Délai: Day 1 (one-time study visit)
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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.
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Day 1 (one-time study visit)
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Inclusion Body Myositis Functional Rating Scale Score
Délai: Day 1 (one-time study visit)
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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.
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Day 1 (one-time study visit)
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Amyotrophic Lateral Sclerosis Functional Rating Scale Score
Délai: Day 1 (one-time study visit)
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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).
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Day 1 (one-time study visit)
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Collaborateurs et enquêteurs
C'est ici que vous trouverez les personnes et les organisations impliquées dans cette étude.
Parrainer
Les enquêteurs
- Chercheur principal: Abdullah Z AlQahtani, MD MPH, the Johns Hopkins School of Medicine
Publications et liens utiles
La personne responsable de la saisie des informations sur l'étude fournit volontairement ces publications. Il peut s'agir de tout ce qui concerne l'étude.
Publications générales
- 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.
Dates d'enregistrement des études
Ces dates suivent la progression des dossiers d'étude et des soumissions de résultats sommaires à ClinicalTrials.gov. Les dossiers d'étude et les résultats rapportés sont examinés par la Bibliothèque nationale de médecine (NLM) pour s'assurer qu'ils répondent à des normes de contrôle de qualité spécifiques avant d'être publiés sur le site Web public.
Dates principales de l'étude
Début de l'étude (Estimé)
1 octobre 2026
Achèvement primaire (Estimé)
31 décembre 2027
Achèvement de l'étude (Estimé)
31 décembre 2027
Dates d'inscription aux études
Première soumission
11 septembre 2026
Première soumission répondant aux critères de contrôle qualité
11 septembre 2026
Première publication (Réel)
16 septembre 2026
Mises à jour des dossiers d'étude
Dernière mise à jour publiée (Réel)
16 septembre 2026
Dernière mise à jour soumise répondant aux critères de contrôle qualité
11 septembre 2026
Dernière vérification
1 septembre 2026
Plus d'information
Termes liés à cette étude
Mots clés
Termes MeSH pertinents supplémentaires
- Maladies musculo-squelettiques
- Maladies du système nerveux central
- Maladies du système nerveux
- Maladies neuromusculaires
- Maladies métaboliques
- Maladies neurodégénératives
- Maladies de la moelle épinière
- TDP-43 Protéinopathies
- Déficits de protéostase
- Maladie du motoneurone
- Conditions pathologiques, signes et symptômes
- Maladies nutritionnelles et métaboliques
- Signes et symptômes
- Maladies musculaires
- La sclérose latérale amyotrophique
- Asthénie
- Myosite
- Myosite, corps d'inclusion
Autres numéros d'identification d'étude
- IRB00553575
Plan pour les données individuelles des participants (IPD)
Prévoyez-vous de partager les données individuelles des participants (DPI) ?
NON
Informations sur les médicaments et les dispositifs, documents d'étude
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Non
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Non
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