{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T18:10:21Z","timestamp":1790619021988,"version":"4.1.0"},"reference-count":13,"publisher":"BMJ","issue":"9","license":[{"start":{"date-parts":[[2025,3,25]],"date-time":"2025-03-25T00:00:00Z","timestamp":1742860800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000368","name":"Brain Research Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000368","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000406","name":"Motor Neurone Disease Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000406","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100013013","name":"Target ALS","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013013","id-type":"DOI","asserted-by":"publisher"}]},{"name":"UCLH NIHR Biomedical Research Centre"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"accepted":{"date-parts":[[2025,3,4]]},"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:sec>\n                    <jats:title>Background and objectives<\/jats:title>\n                    <jats:p>Quantitative and operator-independent biomarkers of disease progression are urgently needed in amyotrophic lateral sclerosis (ALS) research. We assess the potential of skeletal muscle MRI as a sensitive and reliable outcome measure for future ALS clinical trials.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      In this longitudinal cohort study, muscle MRI of head-neck, upper and lower limb regions, alongside clinical and functional assessments, were acquired at three time points over the individual maximum observation period (iMOP) of 1 year in 20 patients with ALS and 16 healthy controls. Quantitative MRI parameters cross-sectional area (CSA), volume (VOL), fat fraction, functional rest muscle area and water T2 (T\n                      <jats:sub>2m<\/jats:sub>\n                      ) were correlated with changes in clinical disease severity (functional rating scales and myometry).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 20 patients with ALS, 17 completed follow-up. Progressive muscle atrophy (CSA, VOL) was observed at hand (rs=0.66), head-neck (partial \u03b7\u00b2=0.47) and lower-limb level (thighs: \u03b7\u00b2=0.56, calves: \u03b7\u00b2=0.54) over iMOP. MRI changes correlated with leg muscle strength (knee extension: r=0.77; plantar flexion: r=0.78), hand grip strength (r=0.71) and functional rating scales (r=0.68).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>Our findings demonstrate the effectiveness of muscle MRI as a sensitive neuroimaging biomarker of disease progression in ALS, highlighting its potential application in clinical trials.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/jnnp-2024-335571","type":"journal-article","created":{"date-parts":[[2025,3,26]],"date-time":"2025-03-26T01:51:50Z","timestamp":1742953910000},"page":"908-911","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis"],"prefix":"10.1136","volume":"96","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-0678-4326","authenticated-orcid":false,"given":"Uros","family":"Klickovic","sequence":"first","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"},{"name":"Department of Radiology, University Hospital Tulln, Tulln, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Zampedri","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nick","family":"Zafeiropoulos","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"},{"name":"Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1504-7245","authenticated-orcid":false,"given":"Oliver J","family":"Ziff","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher DJ","family":"Sinclair","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Wastling","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdalena","family":"Dudziec","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jodie","family":"Allen","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9203-5344","authenticated-orcid":false,"given":"Karin","family":"Trimmel","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"},{"name":"Department of Neurology, Medical University of Vienna, Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robin S","family":"Howard","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Malaspina","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikhil","family":"Sharma","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie CL","family":"Sidle","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sachit","family":"Shah","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Nasel","sequence":"additional","affiliation":[{"name":"Department of Radiology, University Hospital Tulln, Tulln, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tarek A","family":"Yousry","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Greensmith","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasper M","family":"Morrow","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John S","family":"Thornton","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"},{"name":"Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pietro","family":"Fratta","sequence":"additional","affiliation":[{"name":"MND Centre, UCL Queen Square Institute of Neurology, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,3,25]]},"reference":[{"key":"2025081402151280000_96.9.908.1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00242-2"},{"key":"2025081402151280000_96.9.908.2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2015.05.006"},{"key":"2025081402151280000_96.9.908.3","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1002\/ana.25345","article-title":"Disease progression and outcome measures in spinobulbar muscular atrophy","volume":"84","author":"Dahlqvist","year":"2018","journal-title":"Ann Neurol"},{"key":"2025081402151280000_96.9.908.4","first-page":"e895","article-title":"Skeletal muscle MRI differentiates SBMA and ALS and correlates with disease severity","volume":"93","author":"Klickovic","year":"2019","journal-title":"Neurology (ECronicon)"},{"key":"2025081402151280000_96.9.908.5","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2017-316744"},{"key":"2025081402151280000_96.9.908.6","doi-asserted-by":"crossref","DOI":"10.3389\/fneur.2021.749736","article-title":"Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis","volume":"12","author":"Paoletti","year":"2021","journal-title":"Front Neurol"},{"key":"2025081402151280000_96.9.908.7","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20977"},{"key":"2025081402151280000_96.9.908.8","doi-asserted-by":"publisher","DOI":"10.1007\/s00256-007-0409-0"},{"key":"2025081402151280000_96.9.908.9","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-019-09580-x"},{"key":"2025081402151280000_96.9.908.10","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1093\/brain\/awr351","article-title":"A proposed staging system for amyotrophic lateral sclerosis","volume":"135","author":"Roche","year":"2012","journal-title":"Brain (Bacau)"},{"key":"2025081402151280000_96.9.908.11","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2005.072660"},{"key":"2025081402151280000_96.9.908.12","doi-asserted-by":"publisher","DOI":"10.1007\/s13311-022-01271-2"},{"key":"2025081402151280000_96.9.908.13","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(08)70293-X"}],"container-title":["Journal of Neurology, Neurosurgery &amp; Psychiatry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jnnp-2024-335571","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,14]],"date-time":"2025-08-14T09:15:18Z","timestamp":1755162918000},"score":1,"resource":{"primary":{"URL":"https:\/\/jnnp.bmj.com\/lookup\/doi\/10.1136\/jnnp-2024-335571"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,25]]},"references-count":13,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2025,8,14]]},"published-print":{"date-parts":[[2025,9]]}},"alternative-id":["10.1136\/jnnp-2024-335571"],"URL":"https:\/\/doi.org\/10.1136\/jnnp-2024-335571","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2024.04.10.24305608","asserted-by":"object"}]},"ISSN":["0022-3050","1468-330X"],"issn-type":[{"value":"0022-3050","type":"print"},{"value":"1468-330X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3,25]]}}}