{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T12:00:48Z","timestamp":1788782448778,"version":"build-2803163510"},"reference-count":49,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,10,19]],"date-time":"2023-10-19T00:00:00Z","timestamp":1697673600000},"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"],"award-info":[{"award-number":["K23AG064029"]}],"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":[[2024,2]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To improve the timely recognition of patients with treatment\u2010responsive causes of rapidly progressive dementia (RPD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 226 adult patients with suspected RPD were enrolled in a prospective observational study and followed for up to 2\u2009years. Diseases associated with RPD were characterized as potentially treatment\u2010responsive or non\u2010responsive, referencing clinical literature. Disease progression was measured using Clinical Dementia Rating\u00ae Sum\u2010of\u2010Box scores. Clinical and paraclinical features associated with treatment responsiveness were assessed using multivariable logistic regression. Findings informed the development of a clinical criterion optimized to recognize patients with potentially treatment\u2010responsive causes of RPD early in the diagnostic evaluation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 155 patients met defined RPD criteria, of whom 86 patients (55.5%) had potentially treatment\u2010responsive causes. The median (range) age\u2010at\u2010symptom onset in patients with RPD was 68.9\u2009years (range 22.0\u201390.7\u2009years), with a similar number of men and women. Seizures, tumor (disease\u2010associated), magnetic resonance imaging suggestive of autoimmune encephalitis, mania, movement abnormalities, and pleocytosis (\u226510\u2009cells\/mm<jats:sup>3<\/jats:sup>) in cerebrospinal fluid at presentation were independently associated with treatment\u2010responsive causes of RPD after controlling for age and sex. Those features at presentation, as well as age\u2010at\u2010symptom onset &lt;50\u2009years (ie, STAM<jats:sub>3<\/jats:sub>P), captured 82 of 86 (95.3%) cases of treatment\u2010responsive RPD. The presence of \u22653 STAM<jats:sub>3<\/jats:sub>P features had a positive predictive value of 100%.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Selected features at presentation reliably identified patients with potentially treatment\u2010responsive causes of RPD. Adaptation of the STAM<jats:sub>3<\/jats:sub>P screening score in clinical practice may minimize diagnostic delays and missed opportunities for treatment in patients with suspected RPD. ANN NEUROL 2024;95:237\u2013248<\/jats:p><\/jats:sec>","DOI":"10.1002\/ana.26812","type":"journal-article","created":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T16:18:56Z","timestamp":1696263536000},"page":"237-248","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Improving Early Recognition of Treatment\u2010Responsive Causes of Rapidly Progressive Dementia: The <scp>STAM<sub>3<\/sub>P<\/scp> Score"],"prefix":"10.1002","volume":"95","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7302-2348","authenticated-orcid":false,"given":"Nihal","family":"Satyadev","sequence":"first","affiliation":[{"name":"Department of Neurology Mayo Clinic Florida  Jacksonville Florida USA"},{"name":"Georgia Institute of Technology  Atlanta Georgia 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 Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuka","family":"Martens","sequence":"additional","affiliation":[{"name":"Department of Neuroscience Mayo Clinic Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2840-3955","authenticated-orcid":false,"given":"S. Richard","family":"Dunham","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  Saint Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael D.","family":"Geschwind","sequence":"additional","affiliation":[{"name":"University of California San Francisco Department of Neurology, Memory and Aging Center  San Francisco California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C.","family":"Morris","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  Saint Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5987-8705","authenticated-orcid":false,"given":"Matthew R.","family":"Brier","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  Saint Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neill R.","family":"Graff\u2010Radford","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic 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 Florida  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,19]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0b013e31819e099b"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0000000000000472"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0000000000000276"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2008.534"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41582-022-00659-0"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0000000000000170"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/CON.0000000000001089"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00401-9"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2018.10.014"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1017\/cjn.2018.33"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21430"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0189832"},{"key":"e_1_2_9_14_1","first-page":"1695","article-title":"Rapidly progressive dementia caused by nonenhancing primary lymphoma of the central nervous system","volume":"17","author":"Carlson BA","year":"1996","journal-title":"AJNR Am J Neuroradiol"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2013-307327"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A3455"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1001\/2013.jamaneurol.79"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22454"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000178"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamanetworkopen.2022.1556"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.43.11.2412-a"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jalz.2011.11.002"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbi.2008.08.010"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2010.02522.x"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22307"},{"key":"e_1_2_9_26_1","unstructured":"American Psychiatric Association.Diagnostic and statistical manual of mental disorders 1980. p. 494."},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp191"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.3233\/JAD-201299"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2020.1782"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.18.00306"},{"key":"e_1_2_9_31_1","volume-title":"Applied statistics and probability for engineers","author":"Montgomery DC","year":"2010"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1093\/biomet\/73.1.43"},{"key":"e_1_2_9_33_1","volume-title":"Python tutorial","author":"Van Rossum G","year":"1995"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-004-0311-9"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1159\/000439251"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000004541"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2023.578071"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2022.4251"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1212\/CPJ.0000000000200097"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.3233\/JAD-151036"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awaa362"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(17)30044-3"},{"key":"e_1_2_9_43_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamanetworkopen.2022.25098"},{"key":"e_1_2_9_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2022.103145"},{"key":"e_1_2_9_45_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10072-013-1531-5"},{"key":"e_1_2_9_46_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.22253"},{"key":"e_1_2_9_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2020.577285"},{"key":"e_1_2_9_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2018.07.009"},{"key":"e_1_2_9_49_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12877-023-03841-1"},{"key":"e_1_2_9_50_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000203854"}],"container-title":["Annals of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ana.26812","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,26]],"date-time":"2024-01-26T14:18:45Z","timestamp":1706278725000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ana.26812"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,19]]},"references-count":49,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2024,2]]}},"alternative-id":["10.1002\/ana.26812"],"URL":"https:\/\/doi.org\/10.1002\/ana.26812","archive":["Portico"],"relation":{},"ISSN":["0364-5134","1531-8249"],"issn-type":[{"value":"0364-5134","type":"print"},{"value":"1531-8249","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,19]]},"assertion":[{"value":"2023-07-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-29","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-10-19","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}