{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T16:27:47Z","timestamp":1759422467654,"version":"3.41.2"},"reference-count":15,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,11,27]],"date-time":"2024-11-27T00:00:00Z","timestamp":1732665600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Objectives<\/jats:title><jats:p>Intramuscular fat fraction (FF), assessed using quantitative MRI (qMRI), has emerged as a promising biomarker for hereditary transthyretin amyloidosis (ATTRv) patients. Currently, the main drawbacks to its use in future therapeutic trials are its sensitivity to change over a short period of time and the time\u2010consuming manual segmentation step to extract quantitative data. This pilot study aimed to demonstrate the suitability of an Artificial Intelligence\u2010based (AI) segmentation technique to assess disease progression in a real\u2010life cohort of ATTRv patients over 1\u2009year.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Fifteen ATTRv patients were included in this monocentric, observational, prospective study. FF, magnetization transfer ratio (MTR), and quantitative T2 were extracted from patients' lower limb qMRI at two time points, 1\u2009year apart, at thigh and leg levels. qMRI parameters were correlated with clinical and electrophysiological parameters assessed at the same time.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Global FF at leg level significantly progressed over 1\u2009year: +1.28\u2009\u00b1\u20092.62% (<jats:italic>p<\/jats:italic>\u2009=\u20090.017). At thigh level, no significant change in global FF, MTR, or T2 was measured. The leg FF was strongly correlated with the main clinical and electrophysiological scores.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>AI\u2010based CNN network segmentation combined with qMRI can be used to obtain quantitative metrics for longitudinal studies in ATTRv patients. Global FF at the leg level seems to be the most sensitive MRI biomarker to track disease progression in a 1\u2010year period. Larger studies with treatment\u2010specific groups will now be necessary to determine the place of qMRI markers compared to the current clinical and electrophysiological scores.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.16574","type":"journal-article","created":{"date-parts":[[2024,11,27]],"date-time":"2024-11-27T13:04:24Z","timestamp":1732712664000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Quantitative muscle <scp>MRI<\/scp> combined with <scp>AI<\/scp>\u2010based segmentation as a follow\u2010up biomarker for <scp>ATTRv<\/scp> patients: A longitudinal pilot study"],"prefix":"10.1111","volume":"32","author":[{"given":"Etienne","family":"Fortanier","sequence":"first","affiliation":[{"name":"Reference Center for Neuromuscular Diseases and ALS La Timone University Hospital, Aix\u2010Marseille University  Marseille France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Constance P.","family":"Michel","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance in Biology and Medicine Aix\u2010Marseille University, UMR CNRS 7339  Marseille France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marc Adrien","family":"Hostin","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance in Biology and Medicine Aix\u2010Marseille University, UMR CNRS 7339  Marseille France"},{"name":"Aix Marseille University, CNRS, LIS  Marseille France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5591-2774","authenticated-orcid":false,"given":"Emilien","family":"Delmont","sequence":"additional","affiliation":[{"name":"Reference Center for Neuromuscular Diseases and ALS La Timone University Hospital, Aix\u2010Marseille University  Marseille France"},{"name":"Medicine Faculty Aix\u2010Marseille University, UMR 7286  Marseille 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France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1502-0958","authenticated-orcid":false,"given":"David","family":"Bendahan","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance in Biology and Medicine Aix\u2010Marseille University, UMR CNRS 7339  Marseille France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shahram","family":"Attarian","sequence":"additional","affiliation":[{"name":"Reference Center for Neuromuscular Diseases and ALS La Timone University Hospital, Aix\u2010Marseille University  Marseille France"},{"name":"Aix\u2010Marseille University, Inserm, GMGF  Marseille 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