NCT06227182|RecruitingDMC
Magnetic Resonance Imaging and Ultrasound Comparison With Load Evaluation
MUSCLE+
Ultrasound and Magnetic Resonance Imaging for Assessing Muscle Contractile Performance in FSHD - An Exploratory Study
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Sponsor study identifier
observational
100
1 country
1
Study Start
April 10, 2024
Actual (reported)Primary Completion
January 31, 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
Study Completion
January 31, 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
Facioscapulohumeral dystrophy (FSHD) is one of the most common hereditary neuromuscular disorders (NMD), with an estimated prevalence of 2000 patients in the Netherlands. Magnetic resonance imaging (MRI) and muscle ultrasound have contributed to an enhanced understanding of the pathophysiology of Facioscapulohumeral Muscular Dystrophy (FSHD). Previously, our group demonstrated the potential presence of an intermediate factor between muscle fiber loss and clinical weakness in FSHD. The influence of disrupted muscle architecture in FSHD on muscle contractile efficiency is a likely candidate for this factor, and remains relatively unexplored. In this study, we aim to assess the use of ultrasound-defined contractile performance, in comparison with current measures including structural MRI, for monitoring disease progression in FSHD.
Registry-reported information. Elapsed time and enrollment totals alone do not measure study progress or recruitment pace.
January 31, 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
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Dates and qualifiers reported by the registry. Estimated dates do not confirm that a milestone has occurred.
January 18, 2024
Actual (reported)January 26, 2024
Actual (reported)April 10, 2024
Actual (reported)January 31, 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
January 31, 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
Stage I: Repeatability and feasibility of the ultrasound-defined muscle contractile performance.
The feasibility of the ultrasound-defined contractile performance procedure is expressed in the number of dropouts in stage I. The repeatability of the muscle contractile performance procedure will be determined with the coefficient of variation in stage I.
At baseline
Stage II: Difference in contractile performance, MRI measures and clinical measures between healthy individuals and patients with FSHD.
The ultrasound Speckle-Tracking technique is employed during the dynamic approach to establish the muscle contractile performance, determined by muscle strain and displacement. The Ultrafast Shear Wave Elastography Imaging technique is used during the static approach to evaluate muscle stiffness, grey values(Z-scores) and muscle pinnation angle. With the MRI measurements we evaluate the muscle fat fraction (%), contractile volume(mm\^3), muscle edema, muscle inflammation, fiber curvature, fascicle length(mm), PCSA(mm\^2).
At baseline
Stage II: Change of contractile performance, MRI measures and clinical measures with FSHD disease progression after 1 year.
The ultrasound, MRI and clinical measures at baseline are compared with the measures after 1 year. The ultrasound Speckle-Tracking technique is employed during the dynamic approach to establish the muscle contractile performance, determined by muscle strain and displacement. The Ultrafast Shear Wave Elastography Imaging technique is used during the static approach to evaluate muscle stiffness, grey values(Z-scores) and muscle pinnation angle. For FSHD patients in this study the Medical Research Counsel scale (MRC) and/or Ricci score will be known. These measures will also be used to evaluate disease progression. The range of the MRC score is 0-5, in which '0' means no contraction of the muscle and '5' means normal contraction of the muscle. The Ricci score ranges from 0 - 10 (0= no symptoms and 10=wheelchair bound). With the MRI measurements we evaluate the muscle fat fraction (%), contractile volume(mm\^3), fiber curvature, fascicle length(mm), PCSA(mm\^2).
Healthy individuals between 18 and 70 years old.
Individuals with clinically and genetically proven FSHD type 1 or type 2.
The three procedures are conducted simultaneously for upper and lower extremity muscles. A standard muscle ultrasound preset with a fixed depth of 4 cm or 6 cm will be used, depending on which muscle is visualized in accordance with our routine clinical protocols. During the dynamic approach, the transducer will be placed in a longitudinal fixed position on the muscle using a ProbeFix. All measured contractions in the different muscles will be recorded as short ultrasound videos.
In the Netherlands, FSHD has an estimated prevalence of 2000 patients. All patients will be recruited from one of the largest longitudinal follow up database, FSHD-FOCUS, which is maintained at the Dutch Radboudumc expertise center. This FSHD-FOCUS cohortwas started in 2014 and includes around 200 patients with clinically and genetically proven FSHD type 1 and type 2. A 5-year-follow-up study (FSHD-FOCUS2) collected comprehensive data on the natural course of FSHD using multiple clinical outcome measures. Only patients who have given consent during the FSHD-FOCUS study to be contacted for subsequent research, will be contacted for this study.
Radboud university medical center
Nijmegen, Gelderland, Netherlands
Nens Van Alfen, MD. PhD.
Radboud University Medical Center
First Submitted
January 18, 2024
First Posted
January 26, 2024
Study Start
April 10, 2024
Actual (reported)Primary Completion
January 31, 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
Motor Outcomes to Validate Evaluations in Pediatric FSHD (MOVE Peds)
change from baseline to 1 year follow-up
In Stage II, all patients and 10 healthy participants will undergo MRI. During the MRI procedure, we evaluate muscle fat fraction, muscle contractile volume, muscle inflammation and edema.
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Contact the study team to confirm eligibility.
Study Completion
January 31, 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
Last Updated
January 13, 2025
Record last verified: 2024-01
All anonymized (imaging) data will be made available in a suitable open-source online repository after the study results are published in peer-reviewed journals. A DOI will be assigned to dataset published in the Donders Data Repository (soon: Radboud Data Repository).
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Source: ClinicalTrials.gov.
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