{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T12:51:28Z","timestamp":1791204688615,"version":"4.2.0"},"reference-count":48,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,4,21]],"date-time":"2025-04-21T00:00:00Z","timestamp":1745193600000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,4,1]],"date-time":"2025-04-01T00:00:00Z","timestamp":1743465600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,4]]},"abstract":"ABSTRACT<\/jats:title>\n \n Introduction<\/jats:title>\n \n Hereditary transthyretin amyloid polyneuropathy (\n ATTRv<\/jats:styled-content>\n \u2010\n PN<\/jats:styled-content>\n ) is a rare and progressive neurodegenerative disorder characterized by axonal neuropathy and amyloid deposits. Early detection of disease onset and progression is crucial for timely therapeutic intervention. Quantitative\n MRI<\/jats:styled-content>\n (\n qMRI<\/jats:styled-content>\n ) can be used to measure potential biomarkers. Intraepineurial fat fraction (\n ieFF<\/jats:styled-content>\n ) may reflect lipid droplets in amyloid deposits as described in histological studies or the replacement of nerve fiber loss with fatty\u2010rich interfascicular epineurium. This study investigates the potential utility of\n ieFF<\/jats:styled-content>\n as a novel imaging\u2010related biomarker in differentiating\n ATTRv<\/jats:styled-content>\n \u2010\n PN<\/jats:styled-content>\n , asymptomatic carriers (\n ATTRv<\/jats:styled-content>\n \u2010C), and healthy controls (\n HCs<\/jats:styled-content>\n ).\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Fifty\u2010three patients with\n TTR<\/jats:styled-content>\n mutations were imaged (31\n ATTRv<\/jats:styled-content>\n \u2010\n PN<\/jats:styled-content>\n patients, 22\n ATTRv<\/jats:styled-content>\n \u2010C, and 24\n HC<\/jats:styled-content>\n ) and both clinical and electrophysiological parameters were quantified.\n 3D<\/jats:styled-content>\n volume,\n ieFF<\/jats:styled-content>\n , and magnetization transfer ratio (\n MTR<\/jats:styled-content>\n ) were quantified in sciatic and tibial nerves using\n qMRI<\/jats:styled-content>\n .\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Symptomatic\n ATTRv<\/jats:styled-content>\n \u2010\n PN<\/jats:styled-content>\n patients exhibited significantly higher\n ieFF<\/jats:styled-content>\n in both sciatic (32.4%\n IQR<\/jats:styled-content>\n [24.4\u201338.1]) and tibial nerves (13.7%,\n IQR<\/jats:styled-content>\n [9.97\u201320.7]) compared to controls (sciatic 22.3%,\n IQR<\/jats:styled-content>\n [16.6\u201328.5]; tibial 9.74%,\n IQR<\/jats:styled-content>\n [6.36\u201312.5]) (\n p<\/jats:italic>\n \u2009<\u20090.05).\n ieFF<\/jats:styled-content>\n values were positively correlated in both uni and multivariate analyses with the main clinical scores and electrophysiological measures.\n ATTRv<\/jats:styled-content>\n \u2010C also showed increased\n ieFF<\/jats:styled-content>\n values compared to controls (\n p<\/jats:italic>\n \u2009<\u20090.05). Comparatively,\n MTR<\/jats:styled-content>\n and nerve volumes exhibited less pronounced differences across groups.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n \n This study demonstrates that\n ieFF<\/jats:styled-content>\n effectively differentiates symptomatic and asymptomatic\n ATTRv<\/jats:styled-content>\n patients from\n HC<\/jats:styled-content>\n and correlates strongly with electrophysiological and clinical severity parameters. Furthermore, we compare\n ieFF<\/jats:styled-content>\n with conventional\n qMRI<\/jats:styled-content>\n biomarkers, highlighting its superior potential for monitoring nerve structural impairment.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/ene.70168","type":"journal-article","created":{"date-parts":[[2025,4,21]],"date-time":"2025-04-21T06:44:52Z","timestamp":1745217892000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Intraepineurial Fat Fraction: A Novel\n MR<\/scp>\n Neurography\u2010Based Biomarker in Transthyretin Amyloidosis Polyneuropathy"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3224-132X","authenticated-orcid":false,"given":"Eva Sole","family":"Cruz","sequence":"first","affiliation":[{"name":"Reference Center for Neuromuscular Diseases and ALS La Timone University Hospital, Aix\u2010Marseille University Marseille 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