Autoimmune Encephalitis: Insights Into Immune-Mediated Central Nervous System Injury

Korean J Radiol. 2024 Sep;25(9):807-823. doi: 10.3348/kjr.2023.1307.

Abstract

Autoimmune encephalitis (AE) is a category of immune-mediated disorders of the central nervous system (CNS) affecting children and adults. It is characterized by the subacute onset of altered mentation, neurocognitive issues, refractory seizures/drug-resistant epilepsy, movement disorders, and/or autonomic dysfunction. AE is mediated by autoantibodies targeting specific surface components or intracytoplasmic antigens in the CNS, leading to functional or structural alterations. Multiple triggers that induce autoimmunity have been described, which are mainly parainfectious and paraneoplastic. The imaging features of AE often overlap with each other and with other common causes of encephalitis/encephalopathy (infections and toxic-metabolic etiologies). Limbic encephalitis is the most common imaging finding shared by most of these entities. Cortical, basal ganglia, diencephalon, and brainstem involvement may also be present. Cerebellar involvement is rare and is often a part of paraneoplastic degeneration. Owing to an improved understanding of AE, their incidence and detection have increased. Hence, in an appropriate setting, a high degree of suspicion is crucial when reporting clinical MRIs to ensure prompt treatment and better patient outcomes. In this review, we discuss the pathophysiology of AE and common etiologies encountered in clinical practice.

Keywords: Autoimmune encephalitis; Limbic encephalitis; Paraneoplastic syndrome.

Publication types

  • Review

MeSH terms

  • Autoantibodies / immunology
  • Encephalitis* / diagnostic imaging
  • Encephalitis* / immunology
  • Hashimoto Disease / diagnostic imaging
  • Hashimoto Disease / immunology
  • Humans
  • Magnetic Resonance Imaging* / methods

Substances

  • Autoantibodies

Supplementary concepts

  • Hashimoto's encephalitis