biostudies-literatureGilligan MNational Institutes of Health, USACanadian Institute for Theoretical AstrophysicsNINDS NIH HHSIrish Clinical Academic Training (ICAT)Wellcome Trust213-225https://www.ebi.ac.uk/biostudies/studies/S-EPMC11752097biostudies-literatureUnknown12(1)<h4>Objective</h4>We describe neurologic phenotype, clinical associations, and outcomes in autoimmune brainstem encephalitis.<h4>Methods</h4>Medical records of neural-IgG positive autoimmune brainstem encephalitis patients diagnosed at Mayo Clinic (January 1, 2006-December 31, 2022) were reviewed.<h4>Results</h4>Ninety-eight patients (57 male) were included. Median age of symptom onset was 51 years (range, 8 months-85 years). Frequent presenting features were ≥1: 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-11 (26 patients), GAD65 (high titer, 12), ANNA-1 (anti-Hu, 8), ANNA-2 (anti-Ri, 8), Ma2 (7),Annals of clinical and translational neurologyAutoimmune brainstem encephalitis: Clinical associations, outcomes, and proposed diagnostic criteria.PMC11752097R01 NS113828203930/B/16/ZRO1NS126227R01 NS126227Grant Number203930/B/16/ZR01NS113828Valencia Sanchez CDubey DBanks SMills JROrozco EGilligan MThakolwiboon STillema JMMcKeon AZekeridou APittock SJFlanagan EPLopez-Chiriboga SfalseAutoimmune brainstem encephalitis: Clinical associations, outcomes, and proposed diagnostic criteria.<h4>Objective</h4>We describe neurologic phenotype, clinical associations, and outcomes in autoimmune brainstem encephalitis.<h4>Methods</h4>Medical records of neural-IgG positive autoimmune brainstem encephalitis patients diagnosed at Mayo Clinic (January 1, 2006-December 31, 2022) were reviewed.<h4>Results</h4>Ninety-eight patients (57 male) were included. Median age of symptom onset was 51 years (range, 8 months-85 years). Frequent presenting features were ≥1: 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-11 (26 patients), GAD65 (high titer, 12), ANNA-1 (anti-Hu, 8), ANNA-2 (anti-Ri, 8), Ma2 (7),2025-01-01T00:00:00Z2025 Jan2025-04-21T16:49:01.329Z2025-04-21T16:49:01.329ZS-EPMC117520973970829310.1002/acn3.52273