ArticleScientific reports2017
Muscle MRI and functional outcome measures in Becker muscular dystrophy.
Article in Scientific reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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The trial behind it
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Who cites it
32 citing papers in PubMed, 47 citations in OpenAlex.
- Trial
- Characterization of patients with Becker muscular dystrophy by histology, magnetic resonance imaging, function, and strength assessments.Muscle & nerve · 2022Trial
- Combined qualitative and quantitative MRI reveals short-term progression in facioscapulohumeral muscular dystrophy.Journal of neuromuscular diseases · 2026Article
- Total body and appendicular lean mass by dual-energy X-ray absorptiometry are highly associated with muscle function and bone mineral density in Becker muscular dystrophy.Journal of neuromuscular diseases · 2026Article
- Human Disposition, Metabolism, and Excretion of Sevasemten (EDG-5506), a Selective Modulator of Fast Myosin in Healthy Volunteers.Journal of clinical pharmacology · 2026Article
- Diagnostic Value of Muscle MRI in a Case of Very Late-Onset Becker Muscular Dystrophy.Clinical neuroradiology · 2026Article
- Sensitivity of Different Clinical Outcome Measures in Assessing Adults With Becker Muscular Dystrophy: A 3-Year Natural History Study.Neurology · 2025Article
- The Persistence of Duchenne vs Becker Muscular Dystrophies:Neurology. Genetics · 2025Article
- A multi-sensor approach to improve interpretability of the 6-min walk test as an outcome in muscular dystrophies: an observational study.Brain communications · 2025Article
- Deletion of miR-146a enhances therapeutic protein restoration in model of dystrophin exon skipping.Molecular therapy. Nucleic acids · 2024Article
- Lessons for future clinical trials in adults with Becker muscular dystrophy: Disease progression detected by muscle magnetic resonance imaging, clinical and patient-reported outcome measures.European journal of neurology · 2024Article
- Findings from the Longitudinal CINRG Becker Natural History Study.Journal of neuromuscular diseases · 2024Article
- Draft Guidance for Industry Duchenne Muscular Dystrophy, Becker Muscular Dystrophy, and Related Dystrophinopathies - Developing Potential Treatments for the Entire Spectrum of Disease.Journal of neuromuscular diseases · 2024Article
- Givinostat for Becker muscular dystrophy: A randomized, placebo-controlled, double-blind study.Frontiers in neurology · 2023Article
- [Magnetic resonance imaging for the diagnosis of muscular dystrophy].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2022Article
- NOX4 inhibition promotes the remodeling of dystrophic muscle.JCI insight · 2022Article
- Baseline fat fraction is a strong predictor of disease progression in Becker muscular dystrophy.NMR in biomedicine · 2022Article
- Diagnostic interest of whole-body MRI in early- and late-onset LAMA2 muscular dystrophies: a large international cohort.Journal of neurology · 2022Article
- Adult-onset selective quadriceps femoris weakness in RYR1-related myopathy.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022Article
- Muscle magnetic resonance imaging in myotonic dystrophy type 1 (DM1): Refining muscle involvement and implications for clinical trials.European journal of neurology · 2022Article
Corrections and comments
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Authors and funding
12 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Becker muscular dystrophy (BMD) is a neuromuscular disorder allelic to Duchenne muscular dystrophy (DMD), caused by in-frame mutations in the dystrophin gene, and characterized by a clinical progression that is both milder and more heterogeneous than DMD. Muscle magnetic resonance imaging (MRI) has been proposed as biomarker of disease progression in dystrophinopathies. Correlation with clinically meaningful outcome measures such as North Star Ambulatory Assessment (NSAA) and 6 minute walk test (6MWT) is paramount for biomarker qualification. In this study, 51 molecularly confirmed BMD patients (aged 7-69 years) underwent muscle MRI and were evaluated with functional measures (NSAA and 6MWT) at the time of the MRI, and subsequently after one year. We confirmed a pattern of fatty substitution involving mainly the hip extensors and most thigh muscles. Severity of muscle fatty substitution was significantly correlated with specific DMD mutations: in particular, patients with an isolated deletion of exon 48, or deletions bordering exon 51, showed milder involvement. Fat infiltration scores correlated with baseline functional measures, and predicted changes after 1 year. We conclude that in BMD, skeletal muscle MRI not only strongly correlates with motor function, but also helps in predicting functional deterioration within a 12-month time frame.
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