{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T07:05:45Z","timestamp":1791615945423,"version":"4.3.4"},"reference-count":43,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,1,22]],"date-time":"2021-01-22T00:00:00Z","timestamp":1611273600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004243","name":"Prinses Beatrix Spierfonds","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004243","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100013428","name":"Stichting Spieren voor Spieren","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100013428","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["analyticalsciencejournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["NMR in Biomedicine"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Quantitative MRI (qMRI) of muscles is a promising tool to measure disease progression or to assess therapeutic effects in neuromuscular diseases. Longitudinal imaging studies are needed to show sensitivity of qMRI in detecting disease progression in spinal muscular atrophy (SMA). In this pilot study we therefore studied one\u2010year changes in quantitative MR parameters in relation to clinical scores.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We repeated quantitative 3 T MR analysis of thigh muscles and clinical testing one year after baseline in 10 treatment\u2010na\u00efve patients with SMA, 5 with Type 2 (21.6 \u00b1 7.0 years) and 5 with Type 3 (33.4 \u00b1 11.9 years). MR protocol consisted of Dixon,\n                      <jats:italic>T<\/jats:italic>\n                      <jats:sub>2<\/jats:sub>\n                      mapping and diffusion tensor imaging (DTI). The temporal relation of parameters was examined with a mixed model.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      We detected a significant increase in fat fraction (baseline, 38.2% SE 0.6; follow\u2010up, 39.5% SE 0.6; +1.3%,\n                      <jats:italic>p<\/jats:italic>\n                      = 0.001) in all muscles. Muscles with moderate to high fat infiltration at baseline show a larger increase over time (+1.6%,\n                      <jats:italic>p<\/jats:italic>\n                      &lt; 0.001). We did not find any changes in DTI parameters except for low fat\u2010infiltration muscles (m. adductor longus and m. biceps femoris (short head)). The\n                      <jats:italic>T<\/jats:italic>\n                      <jats:sub>2<\/jats:sub>\n                      of muscles decreased from 28.2 ms to 28.0 ms (\n                      <jats:italic>p<\/jats:italic>\n                      = 0.07). Muscle strength and motor function scores were not significantly different between follow\u2010up and baseline.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Longitudinal imaging data show slow disease progression in skeletal muscle of the thigh of (young\u2010) adult patients with SMA despite stable strength and motor function scores. Quantitative muscle imaging demonstrates potential as a biomarker for disease activity and monitoring of therapy response.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/nbm.4473","type":"journal-article","created":{"date-parts":[[2021,1,22]],"date-time":"2021-01-22T15:24:01Z","timestamp":1611329041000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Quantification of disease progression in spinal muscular atrophy with muscle MRI\u2014a pilot study"],"prefix":"10.1002","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2998-4683","authenticated-orcid":false,"given":"Louise A.M.","family":"Otto","sequence":"first","affiliation":[{"name":"Department of Neurology, UMC Utrecht Brain Center University Medical Center Utrecht, Utrecht University  Utrecht The 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