Immunotherapy-responsive Non-paraneoplastic Encephalitis with Antibodies against GAD, LGI1, and GABAA Receptor

Intern Med. 2022 Feb 1;61(3):419-423. doi: 10.2169/internalmedicine.7846-21. Epub 2021 Jul 30.

Abstract

A 62-year-old man showed abnormal behavior. Brain magnetic resonance imaging revealed multifocal lesions on T2-weighted images. Initial screening revealed that he was seropositive for antibodies against glutamate decarboxylase, which usually indicates treatment resistance to autoimmune encephalitis (AE). Intensive immunosuppressive therapies, however, improved the neurological symptoms. In line with this, we also detected seropositivity for antibodies against leucine-rich glioma-inactivated 1 and gamma-aminobutyric acid A receptor (GABAAR). Brain imaging and treatment responsiveness suggested that antibodies against GABAAR were the main cause of symptoms. Furthermore, the patient showed the presence of triple anti-neural antibodies in the absence of malignancy and had a favorable clinical course.

Keywords: anti-GABAAR antibody; anti-GAD antibody; anti-LGI1 antibody; autoimmune encephalitis; plasma exchange.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies
  • Encephalitis* / therapy
  • Hashimoto Disease*
  • Humans
  • Immunotherapy
  • Intracellular Signaling Peptides and Proteins
  • Limbic Encephalitis* / therapy
  • Male
  • Middle Aged
  • Receptors, GABA-A

Substances

  • Autoantibodies
  • Intracellular Signaling Peptides and Proteins
  • LGI1 protein, human
  • Receptors, GABA-A