Autoimmune Encephalitis

Abstract

The purpose of this paper is to provide a comprehensive review of recent literature data for autoimmune encephalitis (AIE). AIE refers to inflammatory, non-infectious, immune-mediated encephalitis characterised by neuroinflammation, synthesis of neuronal autoantibodies (NAAs), directed against surface, synaptic and intracellular antigens, with subsequent neuronal dysfunction. It is characterised by heterogeneous anatomic-clinical syndromes and prominent neuropsychiatric symptoms. Due to overlapping of different clinical and diagnostic biomarkers, AIE is often considered diagnosis of exclusion and requires an extensive work-up. Systematic search of the term «autoimmune encephalitis» in the PubMed database was performed, with limitation set for systematic review in papers English, published from 2004- 2022. Further analysis was performed by the search of the author’s reference list and Autoimmune Encephalitis Alliance (AEA) website. The analysis was conducted according to PRISMA (The Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Outcomes that were sought included: AIE classification and presentation; diagnostic processing; treatment. Preset search of published systematic reviews in PubMed database, derived eighty six papers. Further screening of derived data, author’s reference list and AEA website was performed, according to previously defined out- comes. Finally, sixteen papers were independently selected and thoroughly analysed, with relevant conclusions present- ed in this paper. AIE is a severe inflammatory central nervous system (CNS) disorder with a complex differential diagnosis that often remains unrecognised. AIE research has established a wide range of new autoimmune antibodies syndromes, clinical and diagnostic biomarkers, which have improved diagnostic approach and treatment. Initial application of immunotherapy improves the outcome of disease.