{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T14:14:54Z","timestamp":1790604894762,"version":"4.1.0"},"reference-count":39,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,10,29]],"date-time":"2022-10-29T00:00:00Z","timestamp":1667001600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100002108","name":"FA Research Alliance","doi-asserted-by":"publisher","award":["92133"],"award-info":[{"award-number":["92133"]}],"id":[{"id":"10.13039\/100002108","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001807","name":"S\u00e3o Paulo Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001807","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005930","name":"ASCRS Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005930","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007249","name":"University of Minnesota","doi-asserted-by":"publisher","award":["NS076408"],"award-info":[{"award-number":["NS076408"]}],"id":[{"id":"10.13039\/100007249","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007249","name":"University of Minnesota","doi-asserted-by":"publisher","award":["P30"],"award-info":[{"award-number":["P30"]}],"id":[{"id":"10.13039\/100007249","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007249","name":"University of Minnesota","doi-asserted-by":"publisher","award":["EB027061"],"award-info":[{"award-number":["EB027061"]}],"id":[{"id":"10.13039\/100007249","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007249","name":"University of Minnesota","doi-asserted-by":"publisher","award":["P41"],"award-info":[{"award-number":["P41"]}],"id":[{"id":"10.13039\/100007249","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000346","name":"Ataxia UK","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000346","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Spinal cord damage is a hallmark of Friedreich's ataxia (FRDA), but its progression and clinical correlates remain unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The objective of this study was to perform a characterization of cervical spinal cord structural damage in a large multisite FRDA cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We performed a cross\u2010sectional analysis of cervical spinal cord (C1\u2013C4) cross\u2010sectional area (CSA) and eccentricity using magnetic resonance imaging data from eight sites within the ENIGMA\u2010Ataxia initiative, including 256 individuals with FRDA and 223 age\u2010 and sex\u2010matched control subjects. Correlations and subgroup analyses within the FRDA cohort were undertaken based on disease duration, ataxia severity, and onset age.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Individuals with FRDA, relative to control subjects, had significantly reduced CSA at all examined levels, with large effect sizes (\n                      <jats:italic>d<\/jats:italic>\n                      \u00a0&gt;\u20092.1) and significant correlations with disease severity (\n                      <jats:italic>r<\/jats:italic>\n                      \u00a0&lt;\u2009\u22120.4). Similarly, we found significantly increased eccentricity (\n                      <jats:italic>d<\/jats:italic>\n                      \u00a0&gt;\u20091.2), but without significant clinical correlations. Subgroup analyses showed that CSA and eccentricity are abnormal at all disease stages. However, although CSA appears to decrease progressively, eccentricity remains stable over time.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Previous research has shown that increased eccentricity reflects dorsal column (DC) damage, while decreased CSA reflects either DC or corticospinal tract (CST) damage, or both. Hence our data support the hypothesis that damage to the DC and damage to CST follow distinct courses in FRDA: developmental abnormalities likely define the DC, while CST alterations may be both developmental and degenerative. These results provide new insights about FRDA pathogenesis and indicate that CSA of the cervical spinal cord should be investigated further as a potential biomarker of disease progression. \u00a9 2022 The Authors.\n                      <jats:italic>Movement Disorders<\/jats:italic>\n                      published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mds.29261","type":"journal-article","created":{"date-parts":[[2022,10,29]],"date-time":"2022-10-29T12:46:51Z","timestamp":1667047611000},"page":"45-56","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Progressive Spinal Cord Degeneration in Friedreich's Ataxia: Results from\n                    <scp>ENIGMA\u2010Ataxia<\/scp>"],"prefix":"10.1002","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8453-0313","authenticated-orcid":false,"given":"Thiago J.R.","family":"Rezende","sequence":"first","affiliation":[{"name":"Department of Neurology, School of Medical Sciences University of Campinas (UNICAMP)  Campinas S\u00e3o Paulo Brazil"},{"name":"Brazilian Institute of Neuroscience and Neurotechnology (BRAINN), School of Medical Sciences University of Campinas (UNICAMP)  Campinas S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isaac M.","family":"Adanyeguh","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance Research, Department of Radiology University of Minnesota  Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filippo","family":"Arrigoni","sequence":"additional","affiliation":[{"name":"Neuroimaging Unit Scientific Institute, IRCCS Eugenio Medea  Bosisio Parini Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Bender","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Neuroradiology University Hospital T\u00fcbingen  T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9336-9568","authenticated-orcid":false,"given":"Fernando","family":"Cendes","sequence":"additional","affiliation":[{"name":"Department of Neurology, School of Medical Sciences University of Campinas (UNICAMP)  Campinas S\u00e3o Paulo Brazil"},{"name":"Brazilian Institute of Neuroscience and Neurotechnology (BRAINN), School of Medical Sciences University of Campinas (UNICAMP)  Campinas S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise A.","family":"Corben","sequence":"additional","affiliation":[{"name":"Turner Institute for Brain and Mental Health, School of Psychological Sciences Monash University  Clayton Victoria Australia"},{"name":"Bruce Lefroy Centre Murdoch Children's Research Institute  Parkville Victoria Australia"},{"name":"Department of Paediatrics University of Melbourne  Parkville Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Deistung","sequence":"additional","affiliation":[{"name":"University Clinic and Outpatient Clinic for Radiology, Department for Radiation Medicine University Hospital Halle (Saale)  Halle (Saale) Germany"},{"name":"Department of Neurology and Center for Translational and Behavioral Neuroscience (C\u2010TNBS), Essen University Hospital University of Duisburg\u2010Essen  Essen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Delatycki","sequence":"additional","affiliation":[{"name":"Bruce Lefroy Centre Murdoch Children's Research Institute  Parkville Victoria Australia"},{"name":"Department of Paediatrics University of Melbourne  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J\u00fclich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ludger","family":"Sch\u00f6ls","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Diseases, Center of Neurology and Hertie Institute for Clinical Brain Research University T\u00fcbingen  T\u00fcbingen Germany"},{"name":"German Center for Neurodegenerative Disease\"s (DZNE)  T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg B.","family":"Schulz","sequence":"additional","affiliation":[{"name":"Department of Neurology RWTH Aachen University  Aachen Germany"},{"name":"JARA\u2010BRAIN Institute Molecular Neuroscience and Neuroimaging Research Center J\u00fclich GmbH  J\u00fclich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2280-7273","authenticated-orcid":false,"given":"Matthis","family":"Synofzik","sequence":"additional","affiliation":[{"name":"Department of 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