VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy

J Neuroimmunol. 2013 Dec 15;265(1-2):75-81. doi: 10.1016/j.jneuroim.2013.10.005. Epub 2013 Oct 17.

Abstract

Leucine-rich glioma inactivated 1 (LGI1) was recently identified as a target protein in autoimmune synaptic encephalitis, a rare condition associated with autoantibodies against structures in the neuronal synapse. Studies dealing with LGI1 are small in number and the various outcomes of different therapeutic regimens are not well studied. Here, we analyzed clinical characteristics of 14 patients with LGI1 antibodies, and outcomes according to therapeutic strategies. Most patients exhibited abnormal brain positron emission tomography and that patients treated with steroids alone were more likely to relapse and had less favorable outcomes than those treated with steroids and intravenous immunoglobulins.

Keywords: Encephalitis; Immunotherapy; LGI1.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies / blood*
  • Autoantibodies / blood
  • Brain / diagnostic imaging
  • Brain / metabolism
  • Brain / pathology
  • Brain Diseases / blood*
  • Brain Diseases / diagnostic imaging
  • Brain Diseases / immunology
  • Brain Diseases / therapy*
  • Electroencephalography
  • Encephalitis
  • Female
  • Fluorodeoxyglucose F18
  • Hashimoto Disease / blood*
  • Hashimoto Disease / diagnostic imaging
  • Hashimoto Disease / immunology
  • Hashimoto Disease / therapy*
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunotherapy / methods*
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Positron-Emission Tomography
  • Proteins / immunology*
  • Treatment Outcome

Substances

  • Antibodies
  • Autoantibodies
  • Immunoglobulins, Intravenous
  • LGI1 protein, human
  • Proteins
  • Fluorodeoxyglucose F18

Supplementary concepts

  • Hashimoto's encephalitis