{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:51:43Z","timestamp":1786978303605,"version":"build-2736575974"},"reference-count":32,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,6,11]],"date-time":"2022-06-11T00:00:00Z","timestamp":1654905600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2022,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction\/Aims<\/jats:title>\n                    <jats:p>Functional performance tests are the gold standard to assess disease progression and treatment effects in neuromuscular disorders. These tests can be confounded by motivation, pain, fatigue, and learning effects, increasing variability and decreasing sensitivity to disease progression, limiting efficacy assessment in clinical trials with small sample sizes. We aimed to develop and validate a quantitative and objective method to measure skeletal muscle volume and fat content based on whole\u2010body fat\u2010referenced magnetic resonance imaging (MRI) for use in multisite clinical trials.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Subjects aged 18 to 65\u2009years, genetically confirmed facioscapulohumeral muscular dystrophy 1 (FSHD1), clinical severity 2 to 4 (Ricci's scale, range 0\u20135), were enrolled at six sites and imaged twice 4\u201312\u2009weeks apart with T1\u2010weighted two\u2010point Dixon MRI covering the torso and upper and lower extremities. Thirty\u2010six muscles were volumetrically segmented using semi\u2010automatic multi\u2010atlas\u2010based segmentation. Muscle fat fraction (MFF), muscle fat infiltration (MFI), and lean muscle volume (LMV) were quantified for each muscle using fat\u2010referenced quantification.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Seventeen patients (mean age\u2009\u00b1\u2009SD, 49.4\u00a0years \u00b113.02; 12 men) were enrolled. Within\u2010patient SD ranged from 1.00% to 3.51% for MFF and 0.40% to 1.48% for MFI in individual muscles. For LMV, coefficients of variation ranged from 2.7% to 11.7%. For the composite score average of all muscles, observed SDs were 0.70% and 0.32% for MFF and MFI, respectively; composite LMV coefficient of variation was 2.0%.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>We developed and validated a method for measuring skeletal muscle volume and fat content for use in multisite clinical trials of neuromuscular disorders.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mus.27638","type":"journal-article","created":{"date-parts":[[2022,5,19]],"date-time":"2022-05-19T01:09:11Z","timestamp":1652922551000},"page":"183-192","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Quantitative muscle analysis in facioscapulohumeral muscular dystrophy using\n                    <scp>whole\u2010body fat\u2010referenced MRI<\/scp>\n                    : Protocol development, multicenter feasibility, and repeatability"],"prefix":"10.1002","volume":"66","author":[{"given":"Per","family":"Widholm","sequence":"first","affiliation":[{"name":"AMRA Medical AB Link\u00f6ping Sweden"},{"name":"Department of Radiology, and Department of Health, Medicine and Caring Sciences Link\u00f6ping University Link\u00f6ping Sweden"},{"name":"Centre for Medical Image Science and Visualization (CMIV) Link\u00f6ping University Link\u00f6ping Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e9","family":"Ahlgren","sequence":"additional","affiliation":[{"name":"AMRA Medical AB Link\u00f6ping Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Karlsson","sequence":"additional","affiliation":[{"name":"AMRA Medical AB Link\u00f6ping Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thobias","family":"Romu","sequence":"additional","affiliation":[{"name":"AMRA Medical AB Link\u00f6ping Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rabi","family":"Tawil","sequence":"additional","affiliation":[{"name":"University of Rochester Medical Center Rochester New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn R.","family":"Wagner","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute Johns Hopkins School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0790-5315","authenticated-orcid":false,"given":"Jeffrey M.","family":"Statland","sequence":"additional","affiliation":[{"name":"University of Kansas Medical Center Kansas City Kansas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leo H.","family":"Wang","sequence":"additional","affiliation":[{"name":"University of Washington Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7145-7663","authenticated-orcid":false,"given":"Perry B.","family":"Shieh","sequence":"additional","affiliation":[{"name":"University of California Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baziel G. 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