{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T18:30:43Z","timestamp":1793039443716,"version":"4.5.0"},"reference-count":47,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2023,8,1]],"date-time":"2023-08-01T00:00:00Z","timestamp":1690848000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,8,1]],"date-time":"2023-08-01T00:00:00Z","timestamp":1690848000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","journal-of-hepatology.eu","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Hepatology"],"published-print":{"date-parts":[[2023,8]]},"DOI":"10.1016\/j.jhep.2023.04.007","type":"journal-article","created":{"date-parts":[[2023,4,26]],"date-time":"2023-04-26T20:43:25Z","timestamp":1682541805000},"page":"321-328","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":37,"title":["Neurological worsening in Wilson disease \u2013 clinical classification and outcome"],"prefix":"10.1016","volume":"79","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7354-0241","authenticated-orcid":false,"given":"Isabelle","family":"Mohr","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4741-3617","authenticated-orcid":false,"given":"Jan","family":"Pfeiffenberger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ecem","family":"Eker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta","family":"Merle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5640-7534","authenticated-orcid":false,"given":"Aur\u00e9lia","family":"Poujois","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aftab","family":"Ala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karl Heinz","family":"Weiss","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jhep.2023.04.007_bib1","doi-asserted-by":"crossref","first-page":"2089","DOI":"10.1002\/hep.22261","article-title":"Diagnosis and treatment of Wilson disease: an update","volume":"47","author":"Roberts","year":"2008","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2023.04.007_bib2","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1016\/j.jhep.2011.11.007","article-title":"EASL clinical practice guidelines: wilson's disease","volume":"56","year":"2012","journal-title":"J\u00a0Hepatol"},{"key":"10.1016\/j.jhep.2023.04.007_bib3","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1021\/tx900338d","article-title":"Risks of copper and iron toxicity during aging in humans","volume":"23","author":"Brewer","year":"2010","journal-title":"Chem Res Toxicol"},{"key":"10.1016\/j.jhep.2023.04.007_bib4","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1136\/gut.2005.087262","article-title":"Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study","volume":"56","author":"Merle","year":"2007","journal-title":"Gut"},{"key":"10.1016\/j.jhep.2023.04.007_bib5","doi-asserted-by":"crossref","first-page":"1294","DOI":"10.1053\/j.gastro.2007.02.057","article-title":"Late-onset Wilson's disease","volume":"132","author":"Ferenci","year":"2007","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jhep.2023.04.007_bib6","first-page":"1246","volume":"22","author":"Pfeiffer","year":"2016","journal-title":"Wilson Disease. Continuum (Minneap Minn)"},{"key":"10.1016\/j.jhep.2023.04.007_bib7","doi-asserted-by":"crossref","first-page":"337","DOI":"10.12740\/PP\/45218","article-title":"Psychiatric disturbances as a first clinical symptom of Wilson's disease - case report","volume":"50","author":"Litwin","year":"2016","journal-title":"Psychiatr Pol"},{"key":"10.1016\/j.jhep.2023.04.007_bib8","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1111\/j.1749-6632.2009.05109.x","article-title":"Neurologic Wilson's disease","volume":"1184","author":"Lorincz","year":"2010","journal-title":"Ann N Y Acad Sci"},{"key":"10.1016\/j.jhep.2023.04.007_bib9","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/S1474-4422(14)70190-5","article-title":"Wilson's disease and other neurological copper disorders","volume":"14","author":"Bandmann","year":"2015","journal-title":"Lancet Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib10","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1186\/1471-244X-12-52","article-title":"Bipolar disorders and Wilson's disease","volume":"12","author":"Carta","year":"2012","journal-title":"BMC Psychiatry"},{"key":"10.1016\/j.jhep.2023.04.007_bib11","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1002\/1531-8257(199907)14:4<551::AID-MDS1002>3.0.CO;2-S","article-title":"Penicillamine should not be used as initial therapy in Wilson's disease","volume":"14","author":"Brewer","year":"1999","journal-title":"Mov Disord"},{"key":"10.1016\/j.jhep.2023.04.007_bib12","doi-asserted-by":"crossref","first-page":"185","DOI":"10.2165\/00023210-200519030-00001","article-title":"Neurologically presenting Wilson's disease: epidemiology, pathophysiology and treatment","volume":"19","author":"Brewer","year":"2005","journal-title":"CNS Drugs"},{"key":"10.1016\/j.jhep.2023.04.007_bib13","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1001\/archneur.1987.00520170020016","article-title":"Worsening of neurologic syndrome in patients with Wilson's disease with initial penicillamine therapy","volume":"44","author":"Brewer","year":"1987","journal-title":"Arch Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib14","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1053\/j.gastro.2010.12.034","article-title":"Zinc monotherapy is not as effective as chelating agents in treatment of Wilson disease","volume":"140","author":"Weiss","year":"2011","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jhep.2023.04.007_bib15","doi-asserted-by":"crossref","first-page":"e2189","DOI":"10.1212\/WNL.0000000000009474","article-title":"Liver transplantation as a rescue therapy for severe neurologic forms of Wilson disease","volume":"94","author":"Poujois","year":"2020","journal-title":"Neurology"},{"key":"10.1016\/j.jhep.2023.04.007_bib16","first-page":"269","article-title":"Effects of long-term treatment in Wilson's\u00a0disease with D-penicillamine and zinc sulphate","volume":"243","author":"Czlonkowska","year":"1996","journal-title":"J\u00a0Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib17","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1111\/ene.12348","article-title":"D-penicillamine versus zinc sulfate as first-line therapy for Wilson's disease","volume":"21","author":"Czlonkowska","year":"2014","journal-title":"Eur J Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib18","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1016\/j.jocn.2012.02.041","article-title":"Trientine-induced neurological deterioration in a patient with Wilson's disease","volume":"20","author":"Kim","year":"2013","journal-title":"J\u00a0Clin Neurosci"},{"key":"10.1016\/j.jhep.2023.04.007_bib19","doi-asserted-by":"crossref","first-page":"59","DOI":"10.33176\/AACB-18-00014","article-title":"Biochemical markers for the diagnosis and monitoring of Wilson disease","volume":"40","author":"Mohr","year":"2019","journal-title":"Clin Biochem Rev"},{"key":"10.1016\/j.jhep.2023.04.007_bib20","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/j.jns.2015.06.010","article-title":"Early neurological worsening in patients with Wilson's disease","volume":"355","author":"Litwin","year":"2015","journal-title":"J\u00a0Neurol Sci"},{"key":"10.1016\/j.jhep.2023.04.007_bib21","doi-asserted-by":"crossref","first-page":"1234","DOI":"10.1002\/hep.1840120526","article-title":"Perspectives on Wilson's disease","volume":"12","author":"Sternlieb","year":"1990","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2023.04.007_bib22","series-title":"GeneReviews((R))","article-title":"Wilson disease","author":"Weiss","year":"1993"},{"key":"10.1016\/j.jhep.2023.04.007_bib23","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.jns.2011.08.028","article-title":"Gender differences in Wilson's disease","volume":"312","author":"Litwin","year":"2012","journal-title":"J\u00a0Neurol Sci"},{"key":"10.1016\/j.jhep.2023.04.007_bib24","first-page":"698","article-title":"Wilson's disease-cause of mortality in 164 patients during 1992-2003 observation period","volume":"252","author":"Czlonkowska","year":"2005","journal-title":"J\u00a0Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib25","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1159\/000355276","article-title":"Worsening of Wilson disease following penicillamine therapy","volume":"71","author":"Kalita","year":"2014","journal-title":"Eur Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib26","doi-asserted-by":"crossref","first-page":"S69","DOI":"10.21037\/atm.2019.02.48","article-title":"Current anti-copper therapies in management of Wilson disease","volume":"7","author":"Mohr","year":"2019","journal-title":"Ann Transl Med"},{"key":"10.1016\/j.jhep.2023.04.007_bib27","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1159\/000007894","article-title":"Acute dystonia with thalamic and brainstem lesions after initial penicillamine treatment in Wilson's disease","volume":"39","author":"Huang","year":"1998","journal-title":"Eur Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib28","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1002\/mds.21913","article-title":"Neurological presentation of Wilson's disease in a patient after liver transplantation","volume":"23","author":"Litwin","year":"2008","journal-title":"Mov Disord"},{"key":"10.1016\/j.jhep.2023.04.007_bib29","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1007\/s007020050026","article-title":"CSF copper concentrations, blood-brain barrier function, and coeruloplasmin synthesis during the treatment of Wilson's disease","volume":"107","author":"Stuerenburg","year":"2000","journal-title":"J\u00a0Neural Transm"},{"key":"10.1016\/j.jhep.2023.04.007_bib30","first-page":"405","article-title":"Filterable and non-filterable serum copper. 1. The action of penicillamine","volume":"25","author":"Walshe","year":"1963","journal-title":"Clin Sci"},{"key":"10.1016\/j.jhep.2023.04.007_bib31","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.trsl.2009.05.002","article-title":"Treatment of Wilson's disease with tetrathiomolybdate: V. Control of free copper by tetrathiomolybdate and a comparison with trientine","volume":"154","author":"Brewer","year":"2009","journal-title":"Transl Res"},{"key":"10.1016\/j.jhep.2023.04.007_bib32","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/0887-8994(88)90022-7","article-title":"Does CSF copper level in Wilson disease reflect copper accumulation in the brain?","volume":"4","author":"Kodama","year":"1988","journal-title":"Pediatr Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib33","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/0022-510X(87)90275-9","article-title":"CSF copper concentration: a new parameter for diagnosis and monitoring therapy of Wilson's disease with cerebral manifestation","volume":"79","author":"Weisner","year":"1987","journal-title":"J\u00a0Neurol Sci"},{"key":"10.1016\/j.jhep.2023.04.007_bib34","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1111\/nan.12341","article-title":"Brain iron accumulation in Wilson disease: a post mortem 7 Tesla MRI - histopathological study","volume":"43","author":"Dusek","year":"2017","journal-title":"Neuropathol Appl Neurobiol"},{"key":"10.1016\/j.jhep.2023.04.007_bib35","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1159\/000452658","article-title":"Liver transplantation in wilson's disease with neurological impairment: evaluation in 4 patients","volume":"77","author":"Laurencin","year":"2017","journal-title":"Eur Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib36","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1111\/ene.13171","article-title":"Exchangeable copper: a reflection of the neurological severity in Wilson's disease","volume":"24","author":"Poujois","year":"2017","journal-title":"Eur J Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib37","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/B978-0-444-63625-6.00008-2","article-title":"Wilson disease: brain pathology","volume":"142","author":"Poujois","year":"2017","journal-title":"Handb Clin Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib38","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1007\/s12640-013-9453-9","article-title":"Recent discoveries on the functions of astrocytes in the copper homeostasis of the brain: a brief update","volume":"26","author":"Pal","year":"2014","journal-title":"Neurotox Res"},{"key":"10.1016\/j.jhep.2023.04.007_bib39","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1001\/archneur.60.3.379","article-title":"Treatment of Wilson disease with ammonium tetrathiomolybdate: III. Initial therapy in a total of 55 neurologically affected patients and follow-up with zinc therapy","volume":"60","author":"Brewer","year":"2003","journal-title":"Arch Neurol"},{"key":"10.1016\/j.jhep.2023.04.007_bib40","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1001\/archneur.63.4.521","article-title":"Treatment of Wilson disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double-blind study of treatment of the neurologic presentation of Wilson disease","volume":"63","author":"Brewer","year":"2006","journal-title":"Arch Neurol"},{"issue":"4","key":"10.1016\/j.jhep.2023.04.007_bib41","doi-asserted-by":"crossref","first-page":"1428","DOI":"10.1002\/hep.32805","article-title":"A multidisciplinary approach to the diagnosis and management of Wilson disease: Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases","volume":"77","author":"Schilsky","year":"2023","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2023.04.007_bib42","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1007\/s10620-014-3495-6","article-title":"Prospective pilot study of a single daily dosage of trientine for the treatment of Wilson disease","volume":"60","author":"Ala","year":"2015","journal-title":"Dig Dis Sci"},{"key":"10.1016\/j.jhep.2023.04.007_bib43","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1016\/S0140-6736(86)90949-9","article-title":"Dangers of non-compliance in Wilson's disease","volume":"1","author":"Walshe","year":"1986","journal-title":"Lancet"},{"key":"10.1016\/j.jhep.2023.04.007_bib44","doi-asserted-by":"crossref","first-page":"1481","DOI":"10.1002\/jimd.12430","article-title":"Adherence to treatment, a challenge even in treatable metabolic rare diseases: a cross sectional study of Wilson's disease","volume":"44","author":"Jacquelet","year":"2021","journal-title":"J\u00a0Inherit Metab Dis"},{"key":"10.1016\/j.jhep.2023.04.007_bib45","doi-asserted-by":"crossref","first-page":"502","DOI":"10.5114\/ninp.2013.38230","article-title":"Acute focal dystonia induced by a tricyclic antidepressant in a patient with Wilson disease: a case report","volume":"47","author":"Litwin","year":"2013","journal-title":"Neurol Neurochir Pol"},{"key":"10.1016\/j.jhep.2023.04.007_bib46","first-page":"73","article-title":"Association of dopamine receptor gene polymorphisms with the clinical course of Wilson disease","volume":"8","author":"Litwin","year":"2013","journal-title":"JIMD Rep"},{"key":"10.1016\/j.jhep.2023.04.007_bib47","first-page":"2838","article-title":"Zinc and copper intakes and their major food sources for older adults in the 1994-96 continuing survey of food intakes by individuals (CSFII)","volume":"130","author":"Ma","year":"2000","journal-title":"J\u00a0Nutr"}],"container-title":["Journal of Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0168827823002301?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0168827823002301?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,29]],"date-time":"2025-09-29T19:50:57Z","timestamp":1759175457000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0168827823002301"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,8]]},"references-count":47,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2023,8]]}},"alternative-id":["S0168827823002301"],"URL":"https:\/\/doi.org\/10.1016\/j.jhep.2023.04.007","relation":{},"ISSN":["0168-8278"],"issn-type":[{"value":"0168-8278","type":"print"}],"subject":[],"published":{"date-parts":[[2023,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Neurological worsening in Wilson disease \u2013 clinical classification and outcome","name":"articletitle","label":"Article Title"},{"value":"Journal of Hepatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jhep.2023.04.007","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}