{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T22:19:39Z","timestamp":1789165179148,"version":"build-2803163510"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T00:00:00Z","timestamp":1779321600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T00:00:00Z","timestamp":1779321600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["5K23NS091379"],"award-info":[{"award-number":["5K23NS091379"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Although magnetic resonance imaging (MRI) has been used to selectively characterize patterns of muscle involvement in myotonic dystrophy type 2 (DM2), the relationship between quantitative MRI\u2010derived muscle composition and functional performance remains incompletely defined. This study aimed to characterize the imaging phenotype in DM2 using whole\u2010body MRI scans and to evaluate the association between MRI\u2010based muscle measurements and clinical endpoints.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Clinical endpoints collected included strength, physical function, and patient\u2010reported outcomes. AI\u2010based algorithms were utilized to segment 38 muscles bilaterally from Dixon MRI scans collected in a prospective, cross\u2010sectional study. The segmentations were used to calculate muscle fat fraction (MFF) and contractile volume (CV) for each muscle. Associations between MRI measurements and clinical outcomes were examined using Spearman correlations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Adult patients (6 females, 6 males) with DM2 and no contraindications to MRI were enrolled at a single center. The mean age was 52.5\u2009\u00b1\u200915.4\u2009years and mean disease duration was 12.5\u2009\u00b1\u20097.8\u2009years. The gluteus maximus and erector spinae muscles had the highest average MFF. Mean MFF in fully captured muscles ranged from 7.4% to 36.5%. Higher MFFs were moderately associated with shorter 6\u2010min walk distances (\n                      <jats:italic>\u03c1<\/jats:italic>\n                      \u2009=\u2009\u22120.62,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.03) and slower 10\u2010m walk\/run times (\n                      <jats:italic>\u03c1<\/jats:italic>\n                      \u2009=\u20090.61,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.04). Higher CVs were moderately associated with longer 6\u2010min walk distance (\n                      <jats:italic>\u03c1<\/jats:italic>\n                      \u2009=\u20090.67,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.02) and faster 10\u2010m walk\/run (\n                      <jats:italic>\u03c1<\/jats:italic>\n                      \u2009=\u2009\u22120.67,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.02).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>These results identify a pattern of proximal muscle involvement and moderate associations between MFFs and CVs with physical function. Larger longitudinal studies are needed to validate the utility of imaging biomarkers in DM2.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mus.70294","type":"journal-article","created":{"date-parts":[[2026,5,22]],"date-time":"2026-05-22T02:05:16Z","timestamp":1779415516000},"page":"366-373","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["<scp>MRI<\/scp>\n                    \u2010Determined Muscle Fat Fractions and Contractile Volumes in Myotonic Dystrophy Type 2"],"prefix":"10.1002","volume":"74","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1338-0383","authenticated-orcid":false,"given":"Lauren M.","family":"Hinkley","sequence":"first","affiliation":[{"name":"The Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn R.","family":"Wagner","sequence":"additional","affiliation":[{"name":"The Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"TREAT\u2010NMD  Newcastle upon Tyne UK"},{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Jacobs","sequence":"additional","affiliation":[{"name":"The Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth Houston)  Houston Texas USA"},{"name":"Graduate School of Biomedical Sciences, MD Anderson Cancer Center The University of Texas  Houston Texas USA"},{"name":"Department of Computer Science Rice University  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2558-7798","authenticated-orcid":false,"given":"Doris G.","family":"Leung","sequence":"additional","affiliation":[{"name":"The Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,5,21]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-006-0111-5"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-017-8547-3"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1259\/bjr.20170344"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.27821"},{"key":"e_1_2_13_6_1","first-page":"95","article-title":"Muscle MRI in Muscular Dystrophies","volume":"34","author":"D\u00edaz\u2010Manera J.","year":"2015","journal-title":"Acta Myologica"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25804"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24569"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598\u2010024\u201065802\u2010x"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jval.2019.02.004"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1213\/ANE.0000000000002864"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-017-8574-0"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000204312"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.98.18_supplement.1825"},{"key":"e_1_2_13_15_1","first-page":"S116","article-title":"Sub\u2010Regional Body Composition and Clinical Endpoints in Myotonic Dystrophy Type 2 (DM2)","volume":"84","author":"Puwanant A.","year":"2018","journal-title":"Annals of Neurology"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.98.18_supplement.2406"},{"key":"e_1_2_13_17_1","first-page":"S205","article-title":"Lower Extremity Muscle Fat Fraction Is Associated With Motor Performance in Myotonic Dystrophy Type 2","volume":"92","author":"Madrid D. A.","year":"2022","journal-title":"Annals of Neurology"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/01616412.2020.1716494"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.70294","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.70294","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.70294","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T22:04:57Z","timestamp":1789164297000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.70294"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,21]]},"references-count":17,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,8]]}},"alternative-id":["10.1002\/mus.70294"],"URL":"https:\/\/doi.org\/10.1002\/mus.70294","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,21]]},"assertion":[{"value":"2025-08-23","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-14","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-21","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}