Mechanisms and Emerging Therapies for Treatment of Seizures in Pediatric Autoimmune Encephalitis and Autoinflammatory/Autoimmune-Associated Epilepsy

Rheum Dis Clin North Am. 2023 Nov;49(4):875-893. doi: 10.1016/j.rdc.2023.06.010. Epub 2023 Aug 28.

Abstract

There has been increasing understanding of the role of inflammation in seizures and epilepsy, as well as targeted immunomodulatory treatments. In children, immune-mediated seizures often present acutely in the setting of autoimmune encephalitis and are very responsive to immunotherapy with low rates of subsequent epilepsy. Conversely, seizures in autoimmune-associated epilepsies, such as Rasmussen syndrome, can remain refractory to multimodal therapy, including immunomodulation. In this review, the authors discuss the presentations of immune-mediated seizures in children, underlying mechanisms, and emerging therapies.

Keywords: Anti-MOG-associated disease; Anti-NMDAR encephalitis; Autoimmune encephalitis; Autoimmune-associated epilepsy; FIRES; Rasmussen syndrome.

Publication types

  • Review

MeSH terms

  • Autoantibodies
  • Autoimmune Diseases of the Nervous System* / complications
  • Autoimmune Diseases of the Nervous System* / drug therapy
  • Child
  • Encephalitis* / complications
  • Encephalitis* / drug therapy
  • Epilepsy* / drug therapy
  • Epilepsy* / etiology
  • Humans
  • Seizures / drug therapy
  • Seizures / etiology

Substances

  • Autoantibodies

Supplementary concepts

  • Hashimoto's encephalitis