Clinical characterization of autoimmune LGI1 antibody limbic encephalitis

Epilepsy Behav. 2016 Mar:56:165-9. doi: 10.1016/j.yebeh.2015.12.041. Epub 2016 Feb 17.

Abstract

Objective: Autoimmune encephalitis associated with antibodies to leucine-rich glioma inactivated 1 (LGI1) has recently been identified and is characterized by an acute to subacute onset of cognitive impairment and convulsion, faciobrachial dystonic seizures (FBDSs), and psychiatric disturbances. This study analyzed the clinical characteristics and outcomes of 10 patients with LGI1 antibody encephalitis in order to further understand this disease and to improve its therapeutic strategies.

Methods: Between January 2013 and March 2015, we identified 10 patients with LGI1 antibody encephalitis. We retrospectively analyzed the clinical details, laboratory results, electrophysiological and imaging findings, and the treatment outcomes.

Results: All patients tested had LGI1 antibodies. Immunotherapy was effective in all patients. Seizures in patients with FBDS showed a poor response to antiepileptic drugs. Two patients examined by magnetoencephalogram (MEG) during the acute disease phase showed a small quantity of spike-wave dipoles in the temporal lobe close to the lateral fissure and insular lobe.

Conclusion: Patients with LGI1 antibody encephalitis responded well to immunotherapy. We speculate that FBDS is likely a form of insular epilepsy.

Keywords: Autoimmune encephalitis; LGI1; Limbic encephalitis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Anticonvulsants / therapeutic use
  • Autoantibodies / blood*
  • Autoimmune Diseases / blood*
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / therapy
  • Biomarkers / blood
  • Cohort Studies
  • Female
  • Humans
  • Immunotherapy / trends
  • Intracellular Signaling Peptides and Proteins
  • Limbic Encephalitis / blood*
  • Limbic Encephalitis / diagnosis*
  • Limbic Encephalitis / therapy
  • Magnetoencephalography / methods
  • Male
  • Middle Aged
  • Proteins / metabolism*
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Anticonvulsants
  • Autoantibodies
  • Biomarkers
  • Intracellular Signaling Peptides and Proteins
  • LGI1 protein, human
  • Proteins

Supplementary concepts

  • Autoimmune limbic encephalitis