Guillain-Barré syndrome associated with CASPR2 antibodies: two paediatric cases

J Peripher Nerv Syst. 2014 Sep;19(3):246-9. doi: 10.1111/jns.12089.


The pathogenesis of Guillain-Barré syndrome (GBS) is considered to be, at least in part, mediated by autoantibodies directed against neuronal antigens. Antibodies to contactin-associated protein-like 2 (CASPR2), part of the voltage-gated potassium channel complex (VGKC-complex), are associated with neurological disease predominantly affecting the peripheral nervous system but are not known to be associated with GBS. We report two cases of ganglioside antibody-negative paediatric GBS associated with CASPR2 antibodies. Both patients made a complete clinical recovery. The tissue distribution and function of CASPR2 make it a biologically plausible autoimmune target in GBS and its clinical relevance in GBS should be determined in further studies.

Keywords: Guillain-Barré syndrome; contactin-associated protein-like 2; leucine-rich glioma-inactivated 1; voltage-gated potassium channel.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies
  • Child
  • Child, Preschool
  • Guillain-Barre Syndrome / immunology*
  • Humans
  • Male
  • Membrane Proteins / immunology*
  • Nerve Tissue Proteins / immunology*


  • Autoantibodies
  • CNTNAP2 protein, human
  • Membrane Proteins
  • Nerve Tissue Proteins