{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T16:56:24Z","timestamp":1790787384683,"version":"4.1.0"},"reference-count":25,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,10,17]],"date-time":"2022-10-17T00:00:00Z","timestamp":1665964800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100014364","name":"National Children's Research Centre","doi-asserted-by":"publisher","award":["EXC 2049\u201089829218"],"award-info":[{"award-number":["EXC 2049\u201089829218"]}],"id":[{"id":"10.13039\/100014364","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":[[2023,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>To investigate severe autoimmune encephalitis (AE) in the intensive care unit (ICU) with regard to standard treatment in responsive patients and additional escalation therapies for treatment\u2010refractory cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective, single\u2010center study analyzed medical records of ICU\u2010dependent AE patients for clinical characteristics, treatments, prognostic factors, and neurological outcome as quantified by modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>From 40 enrolled patients (median age\u00a0=\u00a052\u2009years; range\u00a0=\u00a016\u201389\u2009years) with AE mediated by neuronal surface antibodies (nsAb; 90%) and AE with onconeuronal antibodies (10%), 98% received first\u2010line therapy. Of those, 62% obtained additional second\u2010line therapy, and 33% received escalation therapy with bortezomib and\/or daratumumab. Good neurological outcome, defined as mRS\u00a0=\u00a00\u20132, was observed in 47% of AE with nsAb (CASE\u00a0=\u00a05), 77% of anti\u2010N\u2010methyl D\u2010aspartate receptor encephalitis patients (CASE\u00a0=\u00a01), whereas AE patients with onconeuronal antibodies had the poorest outcome (mRS\u00a0=\u00a06, 100%). Treatment\u2010refractory AE patients with nsAb requiring escalation therapy achieved similarly good recovery (mRS\u00a0=\u00a00\u20132, 39%, CASE\u00a0=\u00a03) as patients improving without (mRS\u00a0=\u00a00\u20132, 54%, CASE\u00a0=\u00a04), although they presented a higher disease severity at disease maximum (mRS\u00a0=\u00a05 100% versus 68%, CASE\u00a0=\u00a024 versus 17; <jats:italic>p<\/jats:italic>\u00a0=\u20090.0036), had longer ICU stays (97 versus 23\u2009days; <jats:italic>p<\/jats:italic>\u00a0=\u20090.0002), and a higher survival propability during follow\u2010up (<jats:italic>p<\/jats:italic>\u00a0=\u20090.0203). Prognostic factors for good recovery were younger age (<jats:italic>p<\/jats:italic>\u00a0=\u20090.025) and lack of preexisting comorbidities (<jats:italic>p<\/jats:italic>\u00a0=\u20090.011).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our findings suggest that treatment\u2010refractory AE patients with nsAb in the ICU can reach similarly good outcomes after plasma cell\u2013depleting escalation therapy as patients already responding to standard first\u2010 and\/or second\u2010line therapies.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.15585","type":"journal-article","created":{"date-parts":[[2022,9,30]],"date-time":"2022-09-30T09:44:09Z","timestamp":1664531049000},"page":"474-489","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Clinical characteristics, treatments, outcome, and prognostic factors of severe autoimmune encephalitis in the intensive care unit: Standard treatment and the value of additional plasma cell\u2013depleting escalation therapies for treatment\u2010refractory patients"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1283-3478","authenticated-orcid":false,"given":"Lisa","family":"Schwarz","sequence":"first","affiliation":[{"name":"Department of Neurology and Experimental Neurology Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3481-4956","authenticated-orcid":false,"given":"Nilufar","family":"Akbari","sequence":"additional","affiliation":[{"name":"Institute of Biometry and Clinical Epidemiology, Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8283-7976","authenticated-orcid":false,"given":"Harald","family":"Pr\u00fcss","sequence":"additional","affiliation":[{"name":"Department 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