{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,8]],"date-time":"2026-02-08T01:33:49Z","timestamp":1770514429197,"version":"3.49.0"},"reference-count":5,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2019,5,2]],"date-time":"2019-05-02T00:00:00Z","timestamp":1556755200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2019,10]]},"abstract":"Background and purpose<\/jats:title>Adrenomyeloneuropathy (AMN<\/jats:styled-content>) is the most frequent metabolic hereditary spastic paraplegia. Accordingly, its main site of pathological changes is the spinal cord. It is difficult to quantify AMN<\/jats:styled-content> progression because commonly used clinical scales have limitations and reliable biomarkers are lacking. The goal was to investigate whether spinal cord and brain quantitative magnetic resonance imaging may assess structural changes in AMN<\/jats:styled-content> over a relatively short time period.<\/jats:p><\/jats:sec>Methods<\/jats:title>In this longitudinal observational study, the total cord areas (TCA<\/jats:styled-content>s) from the C2\u2013C3 to T2\u2013T3 level and diffusion tensor imaging (DTI<\/jats:styled-content>) metrics of the cervical spinal cord and brain portion of the corticospinal tracts in six AMN<\/jats:styled-content> and six age\u2010matched control subjects at baseline and at a mean follow\u2010up of 22.6\u00a0months were assessed.<\/jats:p><\/jats:sec>Results<\/jats:title>A significant reduction of the mean TCA<\/jats:styled-content> at the T1\u2013T2 level (\u22123.79%) and a trend of reduction at the lowest cervical levels were observed only in AMN<\/jats:styled-content> patients. Additionally, DTI<\/jats:styled-content> metrics revealed significant changes in fractional anisotropy (\u22128.84%), mean diffusivity (+12.62%) and radial diffusivity (+25.91%) at the C2\u2013C3 level.<\/jats:p><\/jats:sec>Discussion<\/jats:title>The study encourages the assessment of TCA<\/jats:styled-content>s and spinal cord DTI<\/jats:styled-content> metrics as surrogate outcome measures in AMN<\/jats:styled-content>, by focusing on the cervical\u2013thoracic junction and the uppermost part of the cervical spinal cord. Despite the limitation of the results due to the small number of investigated subjects, these observations are useful for forthcoming clinical trials in AMN<\/jats:styled-content> and possibly other hereditary diseases with predominant spinal cord involvement.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13959","type":"journal-article","created":{"date-parts":[[2019,4,1]],"date-time":"2019-04-01T09:32:53Z","timestamp":1554111173000},"page":"1341-1344","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Longitudinal quantitative magnetic resonance imaging in adrenomyeloneuropathy"],"prefix":"10.1111","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6190-6688","authenticated-orcid":false,"given":"L. S.","family":"Politi","sequence":"first","affiliation":[{"name":"Hematology and Oncology Division and Radiology Department Boston Children's Hospital Boston MA USA"},{"name":"Radiology Department University of Massachusetts Medical School Worcester MA USA"},{"name":"Humanitas University and Humanitas Clinical and Research Center \u2010 IRCCS Milano Italy"},{"name":"Neuroradiology Unit and C.E.R.M.A.C. Vita\u2010Salute San Raffaele University and IRCCS Ospedale San Raffaele Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Castellano","sequence":"additional","affiliation":[{"name":"Neuroradiology Unit and C.E.R.M.A.C. Vita\u2010Salute San Raffaele University and IRCCS Ospedale San Raffaele Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Papinutto","sequence":"additional","affiliation":[{"name":"Department of Neurology University of California San Francisco San Francisco CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Mauro","sequence":"additional","affiliation":[{"name":"UOC Malattie Neurodegenerative e Neurometaboliche Rare Fondazione IRCCS Istituto Neurologico Carlo Besta Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Pareyson","sequence":"additional","affiliation":[{"name":"UOC Malattie Neurodegenerative e Neurometaboliche Rare Fondazione IRCCS Istituto Neurologico Carlo Besta Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. G.","family":"Henry","sequence":"additional","affiliation":[{"name":"Department of Neurology University of California San Francisco San Francisco CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Falini","sequence":"additional","affiliation":[{"name":"Neuroradiology Unit and C.E.R.M.A.C. Vita\u2010Salute San Raffaele University and IRCCS Ospedale San Raffaele Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Salsano","sequence":"additional","affiliation":[{"name":"UOC Malattie Neurodegenerative e Neurometaboliche Rare Fondazione IRCCS Istituto Neurologico Carlo Besta Milano Italy"},{"name":"University of Milano\u2010Bicocca Monza Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,5,2]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aww068"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20643"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.21827"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1159\/000439340"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A5334"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.13959","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ene.13959","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.13959","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,9]],"date-time":"2023-09-09T22:46:23Z","timestamp":1694299583000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.13959"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,5,2]]},"references-count":5,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2019,10]]}},"alternative-id":["10.1111\/ene.13959"],"URL":"https:\/\/doi.org\/10.1111\/ene.13959","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,5,2]]},"assertion":[{"value":"2018-11-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-03-11","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-05-02","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}