{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T18:20:42Z","timestamp":1773858042090,"version":"3.50.1"},"reference-count":25,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2016,5,24]],"date-time":"2016-05-24T00:00:00Z","timestamp":1464048000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2016,9]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Akinetic mutism is thought to be an appropriate therapeutic end\u2010point in patients with sporadic Creutzfeldt\u2212Jakob disease (<jats:styled-content style=\"fixed-case\">sCJD<\/jats:styled-content>). However, prognostic factors for akinetic mutism are unclear and clinical signs or symptoms that precede this condition have not been defined. The goal of this study was to identify prognostic factors for akinetic mutism and to clarify the order of clinical sign and symptom development prior to its onset.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The cumulative incidence of akinetic mutism and other clinical signs and symptoms was estimated based on Japanese <jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> surveillance data (455 cases) collected from 2003 to 2008. A proportional hazards model was used to identify prognostic factors for the time to onset of akinetic mutism and other clinical signs and symptoms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Periodic synchronous discharges on electroencephalography were present in the majority of cases (93.5%). The presence of psychiatric symptoms or cerebellar disturbance at <jats:styled-content style=\"fixed-case\">sCJD<\/jats:styled-content> diagnosis was associated with the development of akinetic mutism [hazard ratio (<jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>) 1.50, 95% confidence interval (<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>) 1.14\u20131.99, and <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content> 2.15, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>1.61\u20132.87, respectively]. The clinical course from cerebellar disturbance to myoclonus or akinetic mutism was classified into three types: (i) direct path, (ii) path via pyramidal or extrapyramidal dysfunction and (iii) path via psychiatric symptoms or visual disturbance.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The presence of psychiatric symptoms or cerebellar disturbance increased the risk of akinetic mutism of <jats:styled-content style=\"fixed-case\">sCJD<\/jats:styled-content> cases with probable <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">MV<\/jats:styled-content> subtypes. Also, there appear to be sequential associations in the development of certain clinical signs and symptoms of this disease.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13057","type":"journal-article","created":{"date-parts":[[2016,5,25]],"date-time":"2016-05-25T05:42:55Z","timestamp":1464154975000},"page":"1455-1462","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Specific clinical signs and symptoms are predictive of clinical course in sporadic Creutzfeldt\u2212Jakob disease"],"prefix":"10.1111","volume":"23","author":[{"given":"E.","family":"Nakatani","sequence":"first","affiliation":[{"name":"Translational Research Informatics Center Foundation for Biomedical Research and Innovation Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.","family":"Kanatani","sequence":"additional","affiliation":[{"name":"Department of Health Crisis Management National Institute of Public Health Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Kaneda","sequence":"additional","affiliation":[{"name":"Translational Research Informatics Center Foundation for Biomedical Research and Innovation Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.","family":"Nagai","sequence":"additional","affiliation":[{"name":"Translational Research Informatics Center Foundation for Biomedical Research and Innovation Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Teramukai","sequence":"additional","affiliation":[{"name":"Department of Biostatistics Graduate School of Medical Science Kyoto Prefectural University of Medicine Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Translational Research Informatics Center Foundation for Biomedical Research and Innovation Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Zhou","sequence":"additional","affiliation":[{"name":"Translational Research Informatics Center Foundation for Biomedical Research and Innovation Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Kojima","sequence":"additional","affiliation":[{"name":"Translational Research Informatics Center Foundation for Biomedical Research and Innovation Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Kono","sequence":"additional","affiliation":[{"name":"Translational Research Informatics Center Foundation for Biomedical Research and Innovation Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Fukushima","sequence":"additional","affiliation":[{"name":"Translational Research Informatics Center Foundation for Biomedical Research and Innovation Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Kitamoto","sequence":"additional","affiliation":[{"name":"Department of Neurological Science Tohoku University Graduate School of Medicine Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Mizusawa","sequence":"additional","affiliation":[{"name":"National Center of Neurology and Psychiatry Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,5,24]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0950268814001605"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410050212"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000160117.56690.B2"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh249"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/bmb\/66.1.213"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0895-4356(02)00606-6"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awm063"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2008.533"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410390613"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.2188\/jea.JE20100085"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2010.03185.x"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2014.03.024"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-006-0111-7"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)61393-8"},{"key":"e_1_2_7_17_1","unstructured":"Japan Intractable Diseases Information Center.http:\/\/www.nanbyou.or.jp\/english\/nan_kenkyu_45.htm(accessed 6\/29\/2014)."},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0258(19990330)18:6<695::AID-SIM60>3.0.CO;2-O"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1080\/01621459.1999.10474144"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1176\/jnp.2007.19.4.428"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awq216"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000106954.54011.80"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000151847.57956.FA"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199905273402104"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.52.9.1757"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1440-1789.2001.00383.x"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fene.13057","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.13057","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ene.13057","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.13057","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T22:35:33Z","timestamp":1695508533000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.13057"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,5,24]]},"references-count":25,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2016,9]]}},"alternative-id":["10.1111\/ene.13057"],"URL":"https:\/\/doi.org\/10.1111\/ene.13057","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,5,24]]},"assertion":[{"value":"2015-11-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2016-04-21","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2016-05-24","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}