{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T15:40:21Z","timestamp":1791214821115,"version":"4.1.0"},"reference-count":25,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,12,21]],"date-time":"2024-12-21T00:00:00Z","timestamp":1734739200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009036","name":"Canadian Institute for Theoretical Astrophysics","doi-asserted-by":"publisher","award":["Grant Number203930\/B\/16\/Z"],"award-info":[{"award-number":["Grant Number203930\/B\/16\/Z"]}],"id":[{"id":"10.13039\/100009036","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>We describe neurologic phenotype, clinical associations, and outcomes in autoimmune brainstem encephalitis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Medical records of neural\u2010IgG positive autoimmune brainstem encephalitis patients diagnosed at Mayo Clinic (January 1, 2006\u2013December 31, 2022) were reviewed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Ninety\u2010eight patients (57 male) were included. Median age of symptom onset was 51\u2009years (range, 8\u2009months\u201085\u2009years). Frequent presenting features were\u2009\u22651: diplopia (80%), ataxia (78%), dysarthria (68%), vestibulocochlear symptoms (67%), dysphagia (61%), nausea\/vomiting (42%), and facial weakness (32%). Altered mental status (11%) was uncommon. Neural antibodies detected were as follows: KLHL\u201011 (26 patients), GAD65 (high titer, 12), ANNA\u20101 (anti\u2010Hu, 8), ANNA\u20102 (anti\u2010Ri, 8), Ma2 (7), IgLON\u20105 (6), AQP4 (6), MOG (4), glycine receptor (4), GQ1B (4), PCA\u20101 (anti\u2010Yo, 4), DPPX (2), neurochondrin (2), neurofilament (2), NMDA\u2010R (2), AGNA\u20101 (SOX\u20101, 1), ANNA\u20103 (DACH1, 1), amphiphysin (1), CRMP\u20105 (1), ITPR\u20101 (1), PCA\u2010Tr (DNER, 1), and PDE10A (1). Cancer was identified in 55 patients: germ cell (23 patients; 3 extra\u2010testicular), ductal breast adenocarcinoma (8), small cell carcinoma (6, lung 4), adenocarcinomas (6), neuroendocrine carcinoma (3), hematologic (2), squamous cell (2), and other (7). Median modified Ranking score (mRS) at last follow\u2010up was 3 (range, 0\u20136). Factors associated with poor outcome included abnormal brain MRI, bulbar symptoms, and elevated CSF IgG index. Kaplan\u2013Meier analysis revealed faster progression to wheelchair in patients who were immunotherapy refractory and with elevated CSF IgG index. Diagnostic criteria for autoimmune brainstem encephalitis (definite and probable) are proposed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Autoimmune brainstem encephalitis is a distinct clinical subphenotype of autoimmune encephalitis. Abnormal brain MRI, bulbar symptoms, and elevated CSF\u2010IgG index associate with poor outcome.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.52273","type":"journal-article","created":{"date-parts":[[2024,12,21]],"date-time":"2024-12-21T16:39:52Z","timestamp":1734799192000},"page":"213-225","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Autoimmune brainstem encephalitis: Clinical associations, outcomes, and proposed diagnostic criteria"],"prefix":"10.1002","volume":"12","author":[{"given":"Michael","family":"Gilligan","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"},{"name":"St Vincent's Hospital, University College Dublin  Elm Park Dublin 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