{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T16:35:01Z","timestamp":1774974901174,"version":"3.50.1"},"reference-count":29,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,3,25]],"date-time":"2025-03-25T00:00:00Z","timestamp":1742860800000},"content-version":"vor","delay-in-days":24,"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":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Friedreich's ataxia (FRDA) affects both the central and peripheral nervous system. Peripheral nerve involvement manifests predominantly as a progressive sensory neuropathy caused by dorsal root ganglionopathy. An additional direct involvement of peripheral nerves leading to abnormal myelination is increasingly discussed. Here, we characterize lower extremity peripheral nerve involvement in FRDA by quantitative magnetic resonance neurography (MRN).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Sixteen genetically confirmed FRDA patients and 16 age\u2010\/sex\u2010matched controls were prospectively enrolled. Patients underwent neurologic examinations and nerve conduction studies (NCS). Large\u2010coverage MRN of sciatic and tibial nerves was conducted utilizing dual\u2010echo turbo\u2010spin\u2010echo sequences with spectral fat saturation for T2\u2010relaxometry, and two gradient\u2010echo sequences with and without off\u2010resonance saturation rapid frequency pulses for magnetization transfer contrast imaging. Microstructural and morphometric MRN markers including T2\u2010relaxation time (T2<jats:sub>app<\/jats:sub>), proton spin density (<jats:italic>\u03c1<\/jats:italic>), magnetization transfer ratio (MTR), and cross\u2010sectional area (CSA) were calculated to characterize nerve lesions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Tibial nerve <jats:italic>\u03c1<\/jats:italic> and T2<jats:sub>app<\/jats:sub> were markedly decreased in FRDA at the thigh (<jats:italic>\u03c1<\/jats:italic>: 368.4\u2009\u00b1\u200911.0\u2009a.u.; T2<jats:sub>app<\/jats:sub>: 59.5\u2009\u00b1\u20091.8\u2009ms) and lower leg (<jats:italic>\u03c1<\/jats:italic>: 337.3\u2009\u00b1\u200912.6\u2009a.u.; T2<jats:sub>app<\/jats:sub>: 53.9\u2009\u00b1\u20091.4\u2009ms) versus controls (thigh, <jats:italic>\u03c1<\/jats:italic>: 458.9\u2009\u00b1\u20099.5\u2009a.u., <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001; T2<jats:sub>app<\/jats:sub>: 66.3\u2009\u00b1\u20090.8\u2009ms, <jats:italic>p<\/jats:italic>\u2009=\u20090.0019; lower leg, <jats:italic>\u03c1<\/jats:italic>: 449.9\u2009\u00b1\u200912.1\u2009a.u., <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001; T2<jats:sub>app<\/jats:sub>: 62.4\u2009\u00b1\u20091.2\u2009ms, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001) and correlated well with clinical scores, disease duration, and NCS. MTR and CSA did not differentiate between FRDA and controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our study results provide a profound characterization of peripheral nerve involvement in FRDA. The identified good correlation between <jats:italic>\u03c1<\/jats:italic> and T2<jats:sub>app<\/jats:sub> with clinical symptom scores and NCS suggests that parameters of T2 relaxometry may become relevant biomarkers to monitor disease progression and therapeutic responses in potential future clinical trials.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.70121","type":"journal-article","created":{"date-parts":[[2025,3,27]],"date-time":"2025-03-27T05:38:34Z","timestamp":1743053914000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Peripheral Nerve Involvement in Friedreich's Ataxia Characterized by Quantitative Magnetic Resonance Neurography"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6986-3969","authenticated-orcid":false,"given":"Heike","family":"Jacobi","sequence":"first","affiliation":[{"name":"Department of Neurology Heidelberg University Hospital  Heidelberg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8942-7662","authenticated-orcid":false,"given":"Markus","family":"Weiler","sequence":"additional","affiliation":[{"name":"Department of Neurology Heidelberg University Hospital  Heidelberg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Georges","family":"Sam","sequence":"additional","affiliation":[{"name":"Department of Neurology Heidelberg University Hospital  Heidelberg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sabine","family":"Heiland","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology Heidelberg University Hospital  Heidelberg Germany"},{"name":"Division of Experimental Radiology, Department of Neuroradiology Heidelberg University Hospital  Heidelberg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John M.","family":"Hayes","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Michigan  Ann Arbor USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Bendszus","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology Heidelberg University Hospital  Heidelberg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"Wick","sequence":"additional","affiliation":[{"name":"Department of Neurology Heidelberg University Hospital  Heidelberg Germany"},{"name":"Clinical Cooperation Unit Neurooncology German Cancer Research Center\/DKTK  Heidelberg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6254-9192","authenticated-orcid":false,"given":"Jennifer C.","family":"Hayes","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology Heidelberg University Hospital  Heidelberg Germany"},{"name":"Department of Radiology University of Michigan  Ann Arbor USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,3,25]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.271.5254.1423"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(14)70321-7"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19991119)87:2<168::AID-AJMG8>3.0.CO;2-2"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.12317"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2011.01.010"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-010-0675-0"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26012"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.15841"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25182"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000007945"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awu344"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24524"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.15305"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3940(02)01073-X"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/RLI.0000000000000837"},{"key":"e_1_2_10_17_1","first-page":"79","article-title":"Experimental MR Study of Cerebral Radiation Injury: Quantitative T2 Changes Over Time and Histopathologic Correlation","volume":"16","author":"Miot E.","year":"1995","journal-title":"AJNR. 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