Imaging Assessment of Swallowing and Speech Muscles in Inclusion Body Myositis
2026년 9월 11일 업데이트: 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.
연구 개요
상태
상태
모병
상세 설명
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
연구 유형
연구 유형
관찰
등록 (추정된)
등록
30
연락처 및 위치
이 섹션에서는 연구를 수행하는 사람들의 연락처 정보와 이 연구가 수행되는 장소에 대한 정보를 제공합니다.
연구 연락처
연구 연락처
- 이름: Joseph D Niang, B.A.
- 전화번호: 410-570-3949
- 이메일: jniang1@jh.edu
연구 연락처 백업
- 이름: Pamela Arrazola, M.S.
- 전화번호: 410-955-0749
- 이메일: parrazo1@jh.edu
연구 장소
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Maryland
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Baltimore, Maryland, 미국, 21287
- 모병
- The Johns Hopkins Hospital
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연락하다:
- Abdullah AlQahtani, MD MPH
- 전화번호: 443-819-9480
- 이메일: aalqaht3@jh.edu
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참여기준
연구원은 적격성 기준이라는 특정 설명에 맞는 사람을 찾습니다. 이러한 기준의 몇 가지 예는 개인의 일반적인 건강 상태 또는 이전 치료입니다.
자격 기준
자격 기준
공부할 수 있는 나이
- 성인
- 고령자
건강한 자원 봉사자를 받아들입니다
예
샘플링 방법
비확률 샘플
연구 인구
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.
설명
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.
공부 계획
이 섹션에서는 연구 설계 방법과 연구가 측정하는 내용을 포함하여 연구 계획에 대한 세부 정보를 제공합니다.
연구는 어떻게 설계됩니까?
디자인 세부사항
그룹/코호트 수
4
코호트 및 개입
그룹/코호트그룹/코호트 |
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건강한 통제
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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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연구는 무엇을 측정합니까?
주요 결과 측정
주요 결과 측정
결과 측정 |
측정값 설명 |
기간 |
|---|---|---|
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Bulbar Muscle Thickness Assessed by Neuromuscular Ultrasound
기간: 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
기간: 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
기간: 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
기간: 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
기간: 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
기간: 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
기간: 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
기간: 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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2차 결과 측정
2차 결과 측정
결과 측정 |
측정값 설명 |
기간 |
|---|---|---|
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Center for Neurologic Study Bulbar Function Scale Score
기간: 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
기간: 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
기간: 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
기간: 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
기간: 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
기간: 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
기간: 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
기간: 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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공동 작업자 및 조사자
여기에서 이 연구와 관련된 사람과 조직을 찾을 수 있습니다.
수사관
수사관
- 수석 연구원: Abdullah Z AlQahtani, MD MPH, the Johns Hopkins School of Medicine
간행물 및 유용한 링크
연구에 대한 정보 입력을 담당하는 사람이 자발적으로 이러한 간행물을 제공합니다. 이것은 연구와 관련된 모든 것에 관한 것일 수 있습니다.
일반 간행물
- 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.
연구 기록 날짜
이 날짜는 ClinicalTrials.gov에 대한 연구 기록 및 요약 결과 제출의 진행 상황을 추적합니다. 연구 기록 및 보고된 결과는 공개 웹사이트에 게시되기 전에 특정 품질 관리 기준을 충족하는지 확인하기 위해 국립 의학 도서관(NLM)에서 검토합니다.
연구 주요 날짜
연구 시작 (추정된)
연구 시작
2026년 10월 1일
기본 완료 (추정된)
기본 완료
2027년 12월 31일
연구 완료 (추정된)
연구 완료
2027년 12월 31일
연구 등록 날짜
최초 제출
최초 제출
2026년 9월 11일
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처음 게시됨 (실제)
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2026년 9월 16일
연구 기록 업데이트
마지막 업데이트 게시됨 (실제)
마지막 업데이트 게시됨
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마지막으로 확인됨
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추가 정보
이 연구와 관련된 용어
추가 관련 MeSH 약관
기타 연구 ID 번호
기타 연구 ID 번호
- IRB00553575
개별 참가자 데이터(IPD) 계획
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아니요
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아니
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