Autoimmune encephalitis: what the radiologist needs to know

Neuroradiology. 2024 May;66(5):653-675. doi: 10.1007/s00234-024-03318-x. Epub 2024 Mar 20.

Abstract

Autoimmune encephalitis is a relatively novel nosological entity characterized by an immune-mediated damage of the central nervous system. While originally described as a paraneoplastic inflammatory phenomenon affecting limbic structures, numerous instances of non-paraneoplastic pathogenesis, as well as extra-limbic involvement, have been characterized. Given the wide spectrum of insidious clinical presentations ranging from cognitive impairment to psychiatric symptoms or seizures, it is crucial to raise awareness about this disease category. In fact, an early diagnosis can be dramatically beneficial for the prognosis both to achieve an early therapeutic intervention and to detect a potential underlying malignancy. In this scenario, the radiologist can be the first to pose the hypothesis of autoimmune encephalitis and refer the patient to a comprehensive diagnostic work-up - including clinical, serological, and neurophysiological assessments.In this article, we illustrate the main radiological characteristics of autoimmune encephalitis and its subtypes, including the typical limbic presentation, the features of extra-limbic involvement, and also peculiar imaging findings. In addition, we review the most relevant alternative diagnoses that should be considered, ranging from other encephalitides to neoplasms, vascular conditions, and post-seizure alterations. Finally, we discuss the most appropriate imaging diagnostic work-up, also proposing a suggested MRI protocol.

Keywords: Autoimmune diseases of the nervous system; Autoimmune encephalitis; Encephalitis; Limbic encephalitis; Magnetic resonance imaging.

Publication types

  • Review

MeSH terms

  • Autoantibodies
  • Autoimmune Diseases of the Nervous System*
  • Encephalitis* / diagnostic imaging
  • Hashimoto Disease* / diagnostic imaging
  • Humans
  • Limbic Encephalitis* / diagnostic imaging
  • Radiologists
  • Seizures

Substances

  • Autoantibodies

Supplementary concepts

  • Hashimoto's encephalitis