{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T10:59:53Z","timestamp":1789037993557,"version":"build-2803163510"},"reference-count":30,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2020,3,22]],"date-time":"2020-03-22T00:00:00Z","timestamp":1584835200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain and Behavior"],"published-print":{"date-parts":[[2020,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To present our experience with ammonium tetrathiomolybdate (ATTM) in the decoppering phase treatment of Wilson's disease (WD) with neurological symptoms.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>An uncontrolled longitudinal study was carried out to describe a case series of five patients diagnosed of WD with neurological symptoms in our hospital over the last 5\u00a0years and receiving ATTM for 8 (or 16) weeks. Unified Wilson's Disease Rating Scale (UWDRS), Global Assessment Scale (GAS) for WD and the Brewer\u2010adapted Unified Huntington's Disease Rating Scale (UHDRS) for WD, magnetic resonance imaging, and monitoring for potential adverse effects were carried out in all patients before starting ATTM and 3\u00a0months later when ATTM was stopped and zinc treatment was initiated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>All five patients presented neurological clinical improvement in UWDRS, GAS, and Brewer\u2010adapted UHDRS for WD. Neuroimaging improvement was present in 2\/5 patients with brain edema reduction. Mild anemia, leukopenia, and elevation of transaminases were detected in 1 patient, with complete remission after stopping ATTM for 1\u00a0week and then restarting at a half dose.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>ATTM could be a good treatment for the initial treatment of WD with neurological symptoms due to its high efficacy, with a lower rate of neurological deterioration than the drugs currently available, despite the potential adverse effects.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/brb3.1596","type":"journal-article","created":{"date-parts":[[2020,3,22]],"date-time":"2020-03-22T23:29:30Z","timestamp":1584919770000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Ammonium tetrathiomolybdate in the decoppering phase treatment of Wilson's disease with neurological symptoms: A case series"],"prefix":"10.1002","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3518-5338","authenticated-orcid":false,"given":"Oriol","family":"De Fabregues","sequence":"first","affiliation":[{"name":"Movement Disorders Unit Neurology Department Vall d'Hebron University Hospital Neurodegenerative Diseases Research Group\u2010Vall d'Hebron Research Institute Autonomous University of Barcelona Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaume","family":"Vi\u00f1as","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit Neurology Department Vall d'Hebron University Hospital Neurodegenerative Diseases Research Group\u2010Vall d'Hebron Research Institute Autonomous University of Barcelona Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoni","family":"Palas\u00ed","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit Neurology Department Vall d'Hebron University Hospital Neurodegenerative Diseases Research Group\u2010Vall d'Hebron Research Institute Autonomous University of Barcelona Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel","family":"Quintana","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit Neurology Department Vall d'Hebron University Hospital Neurodegenerative Diseases Research Group\u2010Vall d'Hebron Research Institute Autonomous University of Barcelona Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignasi","family":"Cardona","sequence":"additional","affiliation":[{"name":"Pharmacy Department Vall d'Hebron University Hospital Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Auger","sequence":"additional","affiliation":[{"name":"Magnetic Resonance Unit Department of Radiology (IDI) Vall d'Hebron University Hospital Vall d'Hebron Research Institute Autonomous University of Barcelona Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V\u00edctor","family":"Vargas","sequence":"additional","affiliation":[{"name":"Liver Unit Vall d'Hebron University Hospital CIBERehd Vall d'Hebron Research Institute Autonomous University of Barcelona Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,3,22]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.22231"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.5334\/tohm.435"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(07)60196-2"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.14179"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2004.11.013"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.trsl.2009.05.002"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.63.4.521"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1994.00540180023009"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1991.00530130050019"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.60.3.379"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1987.00520170020016"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-018-0018-3"},{"key":"e_1_2_10_14_1","first-page":"1","article-title":"Unified Wilson's disease Rating Scale: A proposal for the neurological scoring of Wilson's disease","volume":"41","author":"Czlonkowska A.","year":"2007","journal-title":"Neurologia i Neurochirurgia Polska"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13256-018-1608-0"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.21037\/atm.2019.02.43"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.21037\/atm.2019.03.30"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2011.11.007"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.livres.2017.08.003"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1177\/2045125318759461"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2015.06.010"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.2009.05109.x"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.21037\/atm.2019.01.67"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-971173"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1093\/qjmed\/hci095"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22261"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.1111"},{"key":"e_1_2_10_28_1","volume-title":"Efficacy and safety of ALXN1840 (formerly named WTX101) administered for 48 weeks versus standard of care in patients with Wilson disease with an extension period of up to 60 months","author":"Swenson E.","year":"2019"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp149"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2468-1253(17)30293-5"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.36.2.244"}],"container-title":["Brain and Behavior"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fbrb3.1596","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/brb3.1596","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/brb3.1596","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/brb3.1596","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,3]],"date-time":"2023-09-03T00:06:56Z","timestamp":1693699616000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/brb3.1596"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,3,22]]},"references-count":30,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2020,5]]}},"alternative-id":["10.1002\/brb3.1596"],"URL":"https:\/\/doi.org\/10.1002\/brb3.1596","archive":["Portico"],"relation":{"has-review":[{"id-type":"doi","id":"10.1002\/BRB3.1596\/v1\/review2","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/BRB3.1596\/v3\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/BRB3.1596\/v1\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/BRB3.1596\/v2\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/BRB3.1596\/v2\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/BRB3.1596\/v3\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/BRB3.1596\/v1\/review3","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/BRB3.1596\/v2\/response1","asserted-by":"object"},{"id-type":"doi","id":"10.1002\/BRB3.1596\/v1\/decision1","asserted-by":"object"}]},"ISSN":["2162-3279","2162-3279"],"issn-type":[{"value":"2162-3279","type":"print"},{"value":"2162-3279","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,3,22]]},"assertion":[{"value":"2019-10-24","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-02-13","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-03-22","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e01596"}}