{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T12:00:50Z","timestamp":1788782450359,"version":"build-2803163510"},"reference-count":47,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,6,20]],"date-time":"2025-06-20T00:00:00Z","timestamp":1750377600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["K23AG064029; R01AG089380"],"award-info":[{"award-number":["K23AG064029; R01AG089380"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To determine the clinical features that identify patients with suspected rapidly progressive dementia (RPD) who will develop RPD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with suspected RPD were enrolled and followed at Mayo Clinic (Jacksonville, FL; January 2020 to October 2023) and Washington University (Saint Louis, MO; June 2016 to December 2019). Two dementia specialists independently reviewed clinical data and assigned diagnoses. Patients were diagnosed with RPD if they developed dementia within 1\u2009year or incapacitation within 2\u2009years of symptom onset. The associations between clinical features and causes and outcomes were explored via univariate and multivariate comparisons.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of 248 patients with suspected RPD, 185 (74.6%) met criteria for RPD. Patients with RPD were older (62.6\u2009\u00b1\u200914.5 vs 55.2\u2009\u00b1\u200918.1\u2009years;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001), and more frequently diagnosed with Alzheimer's disease or related dementias (OR 3.13, 95% CI 1.40, 7.31) or Creutzfeldt\u2013Jakob disease (OR 4.67, 95% CI 1.38, 15.75). Visual agnosia (15\/183; 8.2%), substantial brain atrophy (27\/182; 14.8%), and periodic epileptiform discharges (11\/151; 7.3%) were exclusively detected in patients with RPD. After controlling for age, intensive care unit admission (OR 4.77, 95% CI 1.61, 14.12), lack of seizures (OR 2.47, 95% CI 1.15, 5.31), abnormal brain magnetic resonance imaging (OR 2.01, 95% CI 0.997, 4.05), and cerebrospinal fluid white blood cell count \u22645\u2009cells\/mm\n                      <jats:sup>3<\/jats:sup>\n                      (OR 3.30, 95% CI 1.60, 6.82) were also independently associated with RPD. Patients with RPD were more likely to die or develop severe dementia (mean time to outcome, 36.1\u2009months; 95% CI 31.2\u201341.0).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>Selected clinical features may identify patients with suspected RPD who are likely to continue to decline. Early recognition of these features may improve diagnostic accuracy and inform prognosis. ANN NEUROL 2025;98:764\u2013776<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ana.27297","type":"journal-article","created":{"date-parts":[[2025,6,20]],"date-time":"2025-06-20T09:00:22Z","timestamp":1750410022000},"page":"764-776","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Decoding the Clinical Features that Associate with Progression, Causes, and Outcomes in Patients with Suspected Rapidly Progressive Dementia"],"prefix":"10.1002","volume":"98","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1004-4560","authenticated-orcid":false,"given":"Yoav D.","family":"Piura","sequence":"first","affiliation":[{"name":"Department of Neurology Mayo Clinic in Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7302-2348","authenticated-orcid":false,"given":"Nihal","family":"Satyadev","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic in Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6561-9870","authenticated-orcid":false,"given":"Nick","family":"Corriveau\u2010Lecavalier","sequence":"additional","affiliation":[{"name":"Department of Psychology and Psychiatry Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2987-1040","authenticated-orcid":false,"given":"Lindsey A.","family":"Kuchenbecker","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Mayo Clinic in Florida  Jacksonville Florida USA"},{"name":"Center for Clinical and Translational Science Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren E.","family":"Haydu","sequence":"additional","affiliation":[{"name":"Department of Quantitative Health Sciences Mayo Clinic in Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael D.","family":"Geschwind","sequence":"additional","affiliation":[{"name":"University of California San Francisco Memory and Aging Center, Weill Neurosciences, Department of Neurology  San Francisco California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4084-2248","authenticated-orcid":false,"given":"Philip W.","family":"Tipton","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic in Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neill R.","family":"Graff\u2010Radford","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic in Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5133-5538","authenticated-orcid":false,"given":"Gregory S.","family":"Day","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic in Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,6,20]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1159\/000439251"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0b013e31819e099b"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21430"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0000000000000276"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.26822"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.26812"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0000000000000472"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0000000000000170"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/CON.0000000000001089"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-024-12845-9"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamanetworkopen.2022.25098"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00401-9"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22307"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A3455"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.3233\/JAD-201299"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.98.18_supplement.1660"},{"key":"e_1_2_10_18_1","unstructured":"Centers for Disease Control and Prevention.CDC's diagnostic criteria for Creutzfeldt\u2010Jakob Disease (CJD) 2018 2018. 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