{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T22:43:13Z","timestamp":1791931393974,"version":"4.3.4"},"reference-count":41,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,5,1]],"date-time":"2023-05-01T00:00:00Z","timestamp":1682899200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J cachexia sarcopenia muscle"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Becker muscular dystrophy (BMD) is an X\u2010linked disorder characterized by slow, progressive muscle damage and muscle weakness. Hallmarks include fibre\u2010size variation and replacement of skeletal muscle with fibrous and adipose tissues, after repeated cycles of regeneration. Muscle histology can detect these features, but the required biopsies are invasive, are difficult to repeat and capture only small muscle volumes. Diffusion\u2010tensor magnetic resonance imaging (DT\u2010MRI) is a potential non\u2010invasive alternative that can calculate muscle fibre diameters when applied with the novel random permeable barrier model (RPBM). In this study, we assessed muscle fibre diameters using DT\u2010MRI in BMD patients and healthy controls and compared these with histology.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We included 13 BMD patients and 9 age\u2010matched controls, who underwent water\u2010fat MRI and DT\u2010MRI at multiple diffusion times, allowing RPBM parameter estimation in the lower leg muscles. Tibialis anterior muscle biopsies were taken from the contralateral leg in 6 BMD patients who underwent DT\u2010MRI and from an additional 32 BMD patients and 15 healthy controls. Laminin and Sirius\u2010red stainings were performed to evaluate muscle fibre morphology and fibrosis. Twelve ambulant patients from the MRI cohort underwent the North Star ambulatory assessment, and 6\u2010min walk, rise\u2010from\u2010floor and 10\u2010m run\/walk functional tests.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>RPBM fibre diameter was significantly larger in BMD patients (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.015): mean (SD)\u00a0=\u00a068.0 (25.3) \u03bcm versus 59.4 (19.2) \u03bcm in controls. Inter\u2010muscle differences were also observed (<jats:italic>P<\/jats:italic>\u00a0\u2264\u00a00.002). Both inter\u2010 and intra\u2010individual RPBM fibre diameter variability were similar between groups. Laminin staining agreed with the RPBM, showing larger median fibre diameters in patients than in controls: 72.5 (7.9) versus 63.2 (6.9) \u03bcm, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.006. However, despite showing similar inter\u2010individual variation, patients showed more intra\u2010individual fibre diameter variability than controls\u2014mean variance (SD)\u00a0=\u00a034.2 (7.9) versus 21.4 (6.9) \u03bcm, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001\u2014and larger fibrosis areas: median (interquartile range)\u00a0=\u00a021.7 (5.6)% versus 14.9 (3.4)%, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001. Despite good overall agreement of RPBM and laminin fibre diameters, they were not associated in patients who underwent DT\u2010MRI and muscle biopsy, perhaps due to lack of colocalization of DT\u2010MRI with biopsy samples.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>DT\u2010MRI RPBM metrics agree with histology and can quantify changes in muscle fibre size that are associated with regeneration without the need for biopsies. They therefore show promise as imaging biomarkers for muscular dystrophies.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jcsm.13242","type":"journal-article","created":{"date-parts":[[2023,5,2]],"date-time":"2023-05-02T01:29:26Z","timestamp":1682990966000},"page":"1546-1557","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Diffusion\u2010tensor magnetic resonance imaging captures increased skeletal muscle fibre diameters in Becker muscular dystrophy"],"prefix":"10.1002","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9841-6909","authenticated-orcid":false,"given":"Donnie","family":"Cameron","sequence":"first","affiliation":[{"name":"C.J. 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