ArticleAnnals of clinical and translational neurology2025
Autoimmune brainstem encephalitis: Clinical associations, outcomes, and proposed diagnostic criteria.
Article in Annals of clinical and translational neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers, 1 of them a synthesis that pooled it.
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Who cites it
10 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Clinical features of paraneoplastic neurologic syndromes with anti-Ri antibodies: PRISMA systematic review.Journal of neurology · 2026Pooled it
- Copine-4 Paraneoplastic Autoimmune Encephalitis.Annals of clinical and translational neurology · 2026Article
- Clinical approach to neurological paraneoplastic syndromes.Internal medicine journal · 2026Review
- Intrathecal immunoglobulin production in the diagnosis, prognosis, and monitoring of autoimmune diseases of the central nervous system.Journal of translational autoimmunity · 2026Review
- Current and Future Biomarkers in the Diagnosis of Autoimmune Encephalitis: A Review of Biomarker Detection Techniques and Their Performance.Medicina (Kaunas, Lithuania) · 2026Review
- Novel methods for neural IgG characterization and validation in autoimmune neurologic disorders.Journal of immunological methods · 2026Review
- Insights Into the Antigenic Repertoire of Unclassified Synaptic Antibodies.Annals of clinical and translational neurology · 2026Article
- Hearing Loss in Neuromyelitis Optica Spectrum Disorder: Case Report and Systematic Review.Journal of clinical medicine · 2026Review
- Neuroimmune mechanisms of the cerebellum-brainstem network in autoimmune encephalitis and related neuroimmune disorders: antibody targets, selective vulnerability, and translational opportunities.Frontiers in immunology · 2026Review
- Autoimmune Encephalitis with Neuronal Surface Autoantibodies and Other Suspected Cases of Autoimmune Etiology: A Single-Center Experience in Poland.International journal of molecular sciences · 2025Article
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Authors and funding
13 authors.
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Abstract
objectiveWe describe neurologic phenotype, clinical associations, and outcomes in autoimmune brainstem encephalitis.
methodsMedical records of neural-IgG positive autoimmune brainstem encephalitis patients diagnosed at Mayo Clinic (January 1, 2006-December 31, 2022) were reviewed.
resultsNinety-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), IgLON-5 (6), AQP4 (6), MOG (4), glycine receptor (4), GQ1B (4), PCA-1 (anti-Yo, 4), DPPX (2), neurochondrin (2), neurofilament (2), NMDA-R (2), AGNA-1 (SOX-1, 1), ANNA-3 (DACH1, 1), amphiphysin (1), CRMP-5 (1), ITPR-1 (1), PCA-Tr (DNER, 1), and PDE10A (1). Cancer was identified in 55 patients: germ cell (23 patients; 3 extra-testicular), 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-up was 3 (range, 0-6). Factors associated with poor outcome included abnormal brain MRI, bulbar symptoms, and elevated CSF IgG index. Kaplan-Meier 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.
interpretationAutoimmune brainstem encephalitis is a distinct clinical subphenotype of autoimmune encephalitis. Abnormal brain MRI, bulbar symptoms, and elevated CSF-IgG index associate with poor outcome.
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