Imaging Assessment of Swallowing and Speech Muscles in Inclusion Body Myositis

Johns Hopkins University30 enrolled

Overview

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.

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

Conditions

IBM Inclusion Body Myositis Myopathy Myositis

Eligibility

Sex: ALLMin age: 18 YearsHealthy volunteers:

Medical Language ↔ Plain English

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.

Outcomes

Primary Outcomes

Bulbar Muscle Thickness Assessed by Neuromuscular Ultrasound

Bulbar muscle thickness will be measured using B-mode neuromuscular ultrasound in short- and long-axis views.