Autoimmune encephalitis associated with Ma2 antibodies and immune checkpoint inhibitor therapy

Pract Neurol. 2020 May;20(3):256-259. doi: 10.1136/practneurol-2019-002464. Epub 2020 Apr 17.

Abstract

Immune checkpoint inhibitors have transformed the treatment of advanced malignancy, while increasing the risk of immune-related adverse events. A 56-year-old woman who had received nivolumab for stage 4 renal cell carcinoma subsequently developed altered behaviour, memory deficits and worsening of previously stable epilepsy. MR scan of the brain showed bilateral FLAIR (fluid-attenuated inversion recovery) hyperintensity of the mesial temporal lobes, and there were anti-Ma2 antibodies in both serum and cerebrospinal fluid. She was treated with corticosteroids but developed further clinical relapses requiring immunoglobulin and rituximab. The immune-related adverse events relating to immune checkpoint inhibitors are an emerging challenge for the neurologist. Some cases are refractory and require serial immunosuppression.

Keywords: anti-PD1; encephalitis; immune checkpoint inhibitor.

Publication types

  • Case Reports

MeSH terms

  • Antigens, Neoplasm / metabolism*
  • Autoantibodies / drug effects
  • Autoantibodies / metabolism*
  • Encephalitis / diagnostic imaging
  • Encephalitis / drug therapy*
  • Encephalitis / metabolism*
  • Female
  • Hashimoto Disease / diagnostic imaging
  • Hashimoto Disease / drug therapy*
  • Hashimoto Disease / metabolism*
  • Humans
  • Immune Checkpoint Inhibitors / pharmacology
  • Immune Checkpoint Inhibitors / therapeutic use*
  • Middle Aged
  • Nerve Tissue Proteins / metabolism*

Substances

  • Antigens, Neoplasm
  • Autoantibodies
  • Immune Checkpoint Inhibitors
  • Ma2 antigen
  • Nerve Tissue Proteins

Supplementary concepts

  • Hashimoto's encephalitis