{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T13:18:42Z","timestamp":1780924722893,"version":"3.54.1"},"reference-count":49,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,3,29]],"date-time":"2024-03-29T00:00:00Z","timestamp":1711670400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100006955","name":"Office of Extramural Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006955","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":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p><jats:italic>VCP<\/jats:italic> multisystem proteinopathy 1 (MSP1), encompassing inclusion body myopathy (IBM), Paget's disease of bone (PDB) and frontotemporal dementia (FTD) (IBMPFD), features progressive muscle weakness, fatty infiltration, and disorganized bone structure in Pagetic bones. The aim of this study is to utilize dual\u2010energy x\u2010ray absorptiometry (DXA) parameters to examine it as a biomarker of muscle and bone disease in MSP1.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>DXA scans were obtained in 28 patients to assess body composition parameters (bone mineral density [BMD], <jats:italic>T<\/jats:italic>\u2010score, total fat, and lean mass) across different groups: total VCP disease (<jats:italic>n<\/jats:italic>\u2009=\u200919), including myopathy without Paget's (\u201cmyopathy\u201d; <jats:italic>n<\/jats:italic>\u2009=\u200912) and myopathy with Paget's (\u201cPaget\u201d; <jats:italic>n<\/jats:italic>\u2009=\u20097), and unaffected first\u2010degree relatives serving as controls (<jats:italic>n<\/jats:italic>\u2009=\u20096).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In the VCP disease group, significant declines in left hip BMD and <jats:italic>Z<\/jats:italic>\u2010scores were noted versus the control group (<jats:italic>p<\/jats:italic>\u2009\u2264\u2009.03). The VCP disease group showed decreased whole body lean mass % (<jats:italic>p<\/jats:italic>\u2009=\u2009.04), and increased total body fat % (<jats:italic>p<\/jats:italic>\u2009=\u2009.04) compared to controls. Subgroup comparisons indicated osteopenia in 33.3% and osteoporosis in 8.3% of the myopathy group, with 14.3% exhibiting osteopenia in the Paget group. Moreover, the Paget group displayed higher lumbar L1\u2010L4 <jats:italic>T<\/jats:italic>\u2010score values than the myopathy group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>In MSP1, DXA revealed reduced bone and lean mass, and increased fat mass. These DXA insights could aid in monitoring disease progression of muscle loss and secondary osteopenia\/osteoporosis in MSP1, providing value both clinically and in clinical research.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28095","type":"journal-article","created":{"date-parts":[[2024,3,29]],"date-time":"2024-03-29T09:14:20Z","timestamp":1711703660000},"page":"699-707","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Cross\u2010sectional study of patients with <scp>VCP<\/scp> multisystem proteinopathy 1 using dual\u2010energy x\u2010ray absorptiometry"],"prefix":"10.1002","volume":"69","author":[{"given":"Rod Carlo Agram","family":"Columbres","sequence":"first","affiliation":[{"name":"Division of Genetics and Metabolism, Department of Pediatrics School of Medicine, University of California  Irvine California USA"},{"name":"College of Osteopathic Medicine, William Carey University  Hattiesburg Mississippi 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