Pediatric Autoimmune Encephalitis and Its Relationship With Infection

Pediatr Neurol. 2021 Jul:120:27-32. doi: 10.1016/j.pediatrneurol.2021.04.001. Epub 2021 Apr 7.

Abstract

Autoimmune encephalitis (AE) is an increasingly recognized inflammatory disorder of the central nervous system and is most often characterized by antibodies against intracellular and neuronal surface antigens. AE is a devastating disease that may result in developmental delay or regression in children. However, the pathogenesis of AE is not clear, and immune system disorders after infection likely play an important role in AE. Many studies have reported that patients with herpes simplex virus encephalitis develop anti-N-methyl-d-aspartate receptor encephalitis after antiviral treatment. It is critical to recognize pediatric AE early and to distinguish it from infectious forms because AE is treatable and responsive to immunotherapies. In this review, we discuss the clinical features of pediatric AE and focus on the relationship between AE and postinfection status. In addition, we review the probable mechanisms underlying infection-triggered AE, which include molecular mimicry, bystander activation, epitope spreading, immune system disorder, and genetic susceptibility.

Keywords: Autoimmune encephalitis; Epilepsy; Genetic susceptibility; Immune system disorder; Infection.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Autoimmune Diseases of the Nervous System* / etiology
  • Autoimmune Diseases of the Nervous System* / genetics
  • Autoimmune Diseases of the Nervous System* / immunology
  • Child
  • Encephalitis* / etiology
  • Encephalitis* / genetics
  • Encephalitis* / immunology
  • Genetic Predisposition to Disease*
  • Humans
  • Infectious Encephalitis* / complications