{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T17:50:05Z","timestamp":1787507405098,"version":"build-2736575974"},"reference-count":47,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,12,27]],"date-time":"2022-12-27T00:00:00Z","timestamp":1672099200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2023,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Wilson's disease (WD) is usually diagnosed in children and young adults; limited data exist on late\u2010onset forms.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The aim was to characterize the clinical and paraclinical presentations, therapeutic management, and outcomes in patients with late\u2010onset WD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients diagnosed with WD after age 40\u2009years were identified from the French Wilson's Disease Registry (FWDR). Clinical, laboratory, and imaging findings and treatment were reported at diagnosis and last follow\u2010up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Forty\u2010five patients were identified (median age: 49, range: 40\u201364) and placed in three groups according to their clinical presentation: neurological (n\u00a0=\u00a020, median diagnostic delay: 20\u00a0months), hepatic (n\u00a0=\u00a013, diagnostic delay: 12\u00a0months), and family screening (n\u00a0=\u00a012), all confirmed genetically. Six neurological patients had an atypical presentation (1 torticollis, 2 writer's cramps, 2 functional movement disorders, and 1 isolated dysarthria), without T2\/fluid\u2010attenuated inversion recovery brain magnetic resonance imaging (MRI) hyperintensities; 5 of 6 had no Kayser\u2013Fleischer ring (KFR); 5 of 6 had liver involvement. In the neurological group, 84% of patients improved clinically, and 1 developed copper deficiency. In the hepatic group, 77% had cirrhosis; 6 patients required liver transplantation. In the screened group, 43% had mild liver involvement; 3 were not treated and remained stable; 24\u2010h urinary copper excretion was normal in 33% of patients at diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      In the FWDR, late\u2010onset forms of WD affect 8% of patients, mostly with neurological presentations. Thirty percent of the neurological forms were atypical (isolated long\u2010lasting symptoms, inconspicuous brain MRI, no KFR). With personalized treatment, prognosis was good. This study emphasized that WD should be suspected at any age and even in cases of atypical presentation. \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.29292","type":"journal-article","created":{"date-parts":[[2022,12,27]],"date-time":"2022-12-27T07:49:41Z","timestamp":1672127381000},"page":"321-332","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Diagnosis and Outcomes of Late\u2010Onset Wilson's Disease: A National Registry\u2010Based Study"],"prefix":"10.1002","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5834-3266","authenticated-orcid":false,"given":"Christelle","family":"Nilles","sequence":"first","affiliation":[{"name":"Department of Neurology Rothschild Foundation Hospital  Paris France"},{"name":"National Reference Center for Wilson's Disease and Other Copper\u2010Related Rare Diseases, Rothschild Foundation Hospital  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mickael Alexandre","family":"Obadia","sequence":"additional","affiliation":[{"name":"Department of Neurology Rothschild Foundation Hospital  Paris France"},{"name":"National Reference Center for Wilson's Disease and Other Copper\u2010Related Rare Diseases, Rothschild Foundation Hospital  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rodolphe","family":"Sobesky","sequence":"additional","affiliation":[{"name":"Centre H\u00e9pato\u2010Biliaire, AP\u2010HP, DHU Hepatinov INSERM UMR\u2010S 1193, H\u00f4pital Paul Brousse  Villejuif France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00e9r\u00f4me","family":"Dumortier","sequence":"additional","affiliation":[{"name":"National 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