| Abstract [eng] |
Autoimmune encephalitis comprises a group of rare, autoimmune inflammatory central nervous system disorders, which are caused by autoantibodies targeting neuronal surface or synaptic antigens. Owing to advances in diagnostic methodologies, the reported incidence of autoimmune encephalitis has been increasing, although the exact prevalence remains undetermined. Among the various subtypes, anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common, predominantly affecting young females. While the condition may arise spontaneously, known risk factors include the presence of ovarian teratomas and preceding infections, particularly caused by Herpes simplex virus. The clinical phenotype of anti-NMDAR encephalitis is diverse, with frequent manifestations including rapidly progressing psychiatric symptoms, impaired cognitive functions, language deficits, seizures, movement disorders, and autonomic dysfunction. Diagnostic suspicion is typically based on the clinical features, supported by brain magnetic resonance imaging and cerebrospinal fluid analysis. Definitive diagnosis, however, necessitates the exclusion of alternative etiologies and the identification of disease-specific autoantibodies in serum or cerebrospinal fluid. Early recognition and initiation of therapy is crucial, as delayed treatment is associated with a more severe disease course and unfavourable outcomes. The therapeutic approach to autoimmune encephalitis is inherently linked to its immunopathogenesis. Pathogenic autoantibodies are produced by differentiated B lymphocytes, thus immunomodulatory therapy is a key factor for the management of autoimmune encephalitis. First-line immunotherapy include high-dose corticosteroids, plasma exchange, and intravenous immunoglobulin. In cases refractory to initial therapy within 2-3 weeks, second-line treatment with rituximab, an anti-CD20 monoclonal antibody that depletes B lymphocytes, is recommended. Rituximab has demonstrated efficacy in autoimmune encephalitis and is generally well-tolerated, with a favourable safety profile. In this study, two clinical cases of anti-NMDAR encephalitis in female patients are described. Both patients presented with catatonia, mutism, autonomic dysfunction, and abnormal movements. After rituximab administration, both individuals achieved complete functional recovery, underscoring the therapeutic potential of early and targeted immunotherapy in severe autoimmune encephalitis. |
