{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T17:30:17Z","timestamp":1788888617737,"version":"build-2803163510"},"reference-count":36,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2020,10,13]],"date-time":"2020-10-13T00:00:00Z","timestamp":1602547200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003246","name":"Nederlandse Organisatie voor Wetenschappelijk Onderzoek","doi-asserted-by":"publisher","award":["016.196.310"],"award-info":[{"award-number":["016.196.310"]}],"id":[{"id":"10.13039\/501100003246","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To explore the potential of neurofilament light (NfL) and glial fibrillary acidic protein (GFAP) as biomarkers of spinal cord degeneration in adrenoleukodystrophy, as objective treatment\u2010outcome parameters are needed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Plasma NfL and GFAP levels were measured in 45 male and 47 female ALD patients and compared to a reference cohort of 73 healthy controls. For male patients, cerebrospinal fluid (CSF) samples (n\u00a0=\u00a033) and 1\u2010year (n\u00a0=\u00a039) and 2\u2010year (n\u00a0=\u00a018) follow\u2010up data were also collected. Severity of myelopathy was assessed with clinical parameters: Expanded Disability Status Scale (EDSS), Severity Scoring system for Progressive Myelopathy (SSPROM), and timed up\u2010and\u2010go.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>NfL and GFAP levels were higher in male (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001, effect size (partial \u019e<jats:sup>2<\/jats:sup>) NfL\u00a0=\u00a00.49, GFAP\u00a0=\u00a00.13) and female (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001, effect size NfL\u00a0=\u00a00.19, GFAP\u00a0=\u00a00.23) patients compared to controls; levels were higher in both symptomatic and asymptomatic patients. In male patients, NfL levels were associated with all three clinical parameters of severity of myelopathy (EDSS, SSPROM, and timed up\u2010and go), while GFAP in male and NfL and GFAP in female patients were not. Changes in clinical parameters during follow\u2010up did not correlate with (changes in) NfL or GFAP levels. Plasma and CSF NfL were strongly correlated (r\u00a0=\u00a00.60, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001), but plasma and CSF GFAP were not (r\u00a0=\u00a00.005, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.98).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Our study illustrates the potential of plasma NfL as biomarker of spinal cord degeneration in adrenoleukodystrophy, which was superior to plasma GFAP in our cohort.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.51188","type":"journal-article","created":{"date-parts":[[2020,10,13]],"date-time":"2020-10-13T14:26:16Z","timestamp":1602599176000},"page":"2127-2136","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Plasma NfL and GFAP as biomarkers of spinal cord degeneration in adrenoleukodystrophy"],"prefix":"10.1002","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3885-2415","authenticated-orcid":false,"given":"Wouter J. C.","family":"van Ballegoij","sequence":"first","affiliation":[{"name":"Department of Paediatric Neurology Amsterdam Leukodystrophy Center Emma Children\u2019s Hospital Amsterdam University Medical Centers University of Amsterdam Amsterdam The Netherlands"},{"name":"Department of Neurology OLVG Hospital Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie I.W.","family":"van de Stadt","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology Amsterdam Leukodystrophy Center Emma Children\u2019s Hospital Amsterdam University Medical Centers University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene C.","family":"Huffnagel","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology Amsterdam Leukodystrophy Center Emma Children\u2019s Hospital Amsterdam University Medical Centers University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephan","family":"Kemp","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology Amsterdam Leukodystrophy Center Emma Children\u2019s Hospital Amsterdam University Medical Centers University of Amsterdam Amsterdam The Netherlands"},{"name":"Laboratory Genetic Metabolic Diseases Department of Clinical Chemistry Amsterdam University Medical Centers Amsterdam Gastroenterology &amp; Metabolism University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eline A. 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