title: Guillain-Barré syndrome associated with CASPR2 antibodies: two paediatric cases.
abstract: 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.
authors.affiliation: Department of Clinical Neurosciences, Institute of Psychiatry, King's College London, London, UK.
authors.forename: Richard E
authors.initials: RE
authors.lastname: Rosch
authors.forename: Alasdair
authors.initials: A
authors.lastname: Bamford
authors.forename: Yael
authors.initials: Y
authors.lastname: Hacohen
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Wraige
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
authors.forename: Leena
authors.initials: L
authors.lastname: Mewasingh
authors.forename: Ming
authors.initials: M
authors.lastname: Lim
mesh_terms: D001323:Autoantibodies; D002648:Child; D002675:Child, Preschool; D020275:Guillain-Barre Syndrome / Q000276:immunology*; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000276:immunology*; D009419:Nerve Tissue Proteins / Q000276:immunology*
chemical_list: D001323:Autoantibodies; C403049:CNTNAP2 protein, human; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins
keywords: Guillain-Barré syndrome; contactin-associated protein-like 2; leucine-rich glioma-inactivated 1; voltage-gated potassium channel