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Magnetic Resonance Imaging and Ultrasound Comparison With Load Evaluation
Sponsor: Radboud University Medical Center
Summary
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.
Official title: Ultrasound and Magnetic Resonance Imaging for Assessing Muscle Contractile Performance in FSHD - An Exploratory Study
Key Details
Gender
All
Age Range
18 Years - 70 Years
Study Type
OBSERVATIONAL
Enrollment
100
Start Date
2024-04-10
Completion Date
2027-01-31
Last Updated
2025-01-13
Healthy Volunteers
Yes
Conditions
Interventions
Muscle ultrasound with surface electromyography and dynamometry
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.
MRI scan
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.
Locations (1)
Radboud university medical center
Nijmegen, Gelderland, Netherlands