Autoantibody Encephalitis: Presentation, Diagnosis, and Management
- PMID: 35796263
- PMCID: PMC9262450
- DOI: 10.3988/jcn.2022.18.4.373
Autoantibody Encephalitis: Presentation, Diagnosis, and Management
Abstract
Autoantibody encephalitis causes distinct clinical syndromes involving alterations in mentation, abnormal movements, seizures, psychiatric symptoms, sleep disruption, spasms, and neuromyotonia. The diagnoses can be confirmed by specific antibody tests, although some antibodies may be better detected in spinal fluid and others in serum. Each disorder conveys a risk of certain tumors which may inform diagnosis and be important for treatment. Autoantibodies to receptors and other neuronal membrane proteins are generally thought to be pathogenic and result in loss of function of the targets, so understanding the pharmacology of the receptors may inform our understanding of the syndromes. Patients may be profoundly ill but the syndromes usually respond to immune therapy, although there are differences in the types of immune therapy that are thought to be most effective for the various disorders.
Keywords: anti-NMDAR encephalitis; antibody; cerebellar ataxia; encephalitis; paraneoplastic.
Copyright © 2022 Korean Neurological Association.
Conflict of interest statement
The author has no potential conflicts of interest to disclose.
Similar articles
-
Glutamate receptor antibodies in neurological diseases: anti-AMPA-GluR3 antibodies, anti-NMDA-NR1 antibodies, anti-NMDA-NR2A/B antibodies, anti-mGluR1 antibodies or anti-mGluR5 antibodies are present in subpopulations of patients with either: epilepsy, encephalitis, cerebellar ataxia, systemic lupus erythematosus (SLE) and neuropsychiatric SLE, Sjogren's syndrome, schizophrenia, mania or stroke. These autoimmune anti-glutamate receptor antibodies can bind neurons in few brain regions, activate glutamate receptors, decrease glutamate receptor's expression, impair glutamate-induced signaling and function, activate blood brain barrier endothelial cells, kill neurons, damage the brain, induce behavioral/psychiatric/cognitive abnormalities and ataxia in animal models, and can be removed or silenced in some patients by immunotherapy.J Neural Transm (Vienna). 2014 Aug;121(8):1029-75. doi: 10.1007/s00702-014-1193-3. Epub 2014 Aug 1. J Neural Transm (Vienna). 2014. PMID: 25081016 Review.
-
The Diagnosis and Treatment of Autoimmune Encephalitis.J Clin Neurol. 2016 Jan;12(1):1-13. doi: 10.3988/jcn.2016.12.1.1. J Clin Neurol. 2016. PMID: 26754777 Free PMC article. Review.
-
Autoimmune channelopathies in paraneoplastic neurological syndromes.Biochim Biophys Acta. 2015 Oct;1848(10 Pt B):2665-76. doi: 10.1016/j.bbamem.2015.04.003. Epub 2015 Apr 14. Biochim Biophys Acta. 2015. PMID: 25883091 Review.
-
Patient-Derived Anti-NMDAR Antibody Disinhibits Cortical Neuronal Networks through Dysfunction of Inhibitory Neuron Output.J Neurosci. 2022 Apr 13;42(15):3253-3270. doi: 10.1523/JNEUROSCI.1689-21.2022. Epub 2022 Mar 3. J Neurosci. 2022. PMID: 35241491 Free PMC article.
-
Anti-NMDAR encephalitis after resection of melanocytic nevi: report of two cases.BMC Neurol. 2015 Sep 14;15:165. doi: 10.1186/s12883-015-0424-z. BMC Neurol. 2015. PMID: 26370233 Free PMC article.
Cited by
-
VH2+ Antigen-Experienced B Cells in the Cerebrospinal Fluid Are Expanded and Enriched in Pediatric Anti-NMDA Receptor Encephalitis.J Immunol. 2023 Nov 1;211(9):1332-1339. doi: 10.4049/jimmunol.2300156. J Immunol. 2023. PMID: 37712756
-
Biomarkers in autoimmune diseases of the central nervous system.Front Immunol. 2023 Apr 5;14:1111719. doi: 10.3389/fimmu.2023.1111719. eCollection 2023. Front Immunol. 2023. PMID: 37090723 Free PMC article. Review.
References
-
- Rauschenberger V, von Wardenburg N, Schaefer N, Ogino K, Hirata H, Lillesaar C, et al. Glycine receptor autoantibodies impair receptor function and induce motor dysfunction. Ann Neurol. 2020;88:544–561. - PubMed
Publication types
LinkOut - more resources
Full Text Sources
