Limbic encephalitis with LGI1 antibodies in a 14-year-old boy

Eur J Paediatr Neurol. 2018 Jan;22(1):190-193. doi: 10.1016/j.ejpn.2017.08.004. Epub 2017 Sep 2.

Abstract

Limbic encephalitis (LE) with antibodies against leucine-rich glioma inactivated protein 1 (LGI1) is an auto-antibody mediated disorder with characteristic symptoms as dysfunction of memory, faciobrachial dystonic seizures and neuropsychiatric symptoms as emotional lability. Limbic encephalitis with LGI1 antibodies has been known so far as a disease of adults. We describe the case of a 14-year-old boy presenting with typical dysfunction of memory and LGI1 antibodies. To the best of our knowledge, this is the youngest patient with LGI1 antibody mediated limbic encephalitis described so far. Improved knowledge of this autoimmune syndrome in children and adolescents permit rapid immunomodulatory treatment, which could help to prevent irreversible lesions, such as hippocampal atrophy.

Keywords: Hippocampal atrophy; Immunotherapy; LGI1 antibodies; Limbic encephalitis; Memory dysfunction.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Autoantibodies / blood
  • Autoantibodies / cerebrospinal fluid
  • Autoantibodies / immunology*
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Limbic Encephalitis / blood
  • Limbic Encephalitis / cerebrospinal fluid
  • Limbic Encephalitis / immunology*
  • Male
  • Proteins / immunology*

Substances

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