Advancing Bulbar Assessment in Inclusion Body Myositis Through a Multimodal Imaging Approach
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 30
- 试验地点
- 1
- 主要终点
- Bulbar Muscle Thickness Assessed by Neuromuscular Ultrasound
研究概览
简要总结
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.
详细描述
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:
研究设计
- 研究类型
- Observational
- 观察模型
- Other
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •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.
排除标准
- •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.
研究组 & 干预措施
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
结局指标
主要结局
Bulbar Muscle Thickness Assessed by Neuromuscular Ultrasound
时间窗: Day 1 (one-time study visit)
Bulbar muscle thickness will be measured using B-mode neuromuscular ultrasound in short- and long-axis views.
Bulbar Muscle Cross-Sectional Area Assessed by Neuromuscular Ultrasound
时间窗: Day 1 (one-time study visit)
Cross-sectional area of the assessed bulbar muscles will be measured using neuromuscular ultrasound.
Bulbar Muscle Echogenicity Assessed by Quantitative Grayscale Ultrasound
时间窗: Day 1 (one-time study visit)
Bulbar muscle echogenicity will be evaluated using quantitative grayscale analysis of neuromuscular ultrasound images.
Bulbar Muscle Echogenicity Assessed by the Heckmatt Grading Scale
时间窗: Day 1 (one-time study visit)
Bulbar muscle echogenicity will be assessed using the Heckmatt grading scale on neuromuscular ultrasound images. Scale 1-4, lower score better.
Bulbar Muscle Volume Assessed by Magnetic Resonance Imaging
时间窗: Day 1 (one-time study visit)
Bulbar muscle volume will be measured from magnetic resonance imaging and reported in cubic centimeters (cm³).
Bulbar Muscle Cross-Sectional Area Assessed by Magnetic Resonance Imaging
时间窗: Day 1 (one-time study visit)
Bulbar muscle cross-sectional area will be measured from magnetic resonance imaging and reported in square centimeters (cm²).
Bulbar Muscle Fat Content Assessed by Magnetic Resonance Imaging
时间窗: Day 1 (one-time study visit)
Bulbar muscle fat content will be measured using magnetic resonance imaging and reported as a percentage (%).
Tongue Muscle Stiffness Assessed by Ultrasound Elastography
时间窗: Day 1 (one-time study visit)
Tongue muscle stiffness will be quantitatively assessed using shear-wave ultrasound elastography.
次要结局
- 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))
- Tongue Endurance Assessed by the Iowa Oral Performance Instrument(Day 1 (one-time study visit))
- Inclusion Body Myositis Functional Rating Scale Score(Day 1 (one-time study visit))
- Amyotrophic Lateral Sclerosis Functional Rating Scale Score(Day 1 (one-time study visit))