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

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

Autor(es): Rosch Richard E.; Bamford Alasdair; Hacohen Yael; Wraige Elizabeth; Vincent Angela; Mewasingh Leena; Lim Ming


Resumo: 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.


Palavras-Chave: Guillain-Barré syndrome, contactin-associated protein-like 2, leucine-rich glioma-inactivated 1, voltage-gated potassium channel


Imprenta: Journal of the Peripheral Nervous System, v. 19, n. 3, p. 246-249, 2014


Identificador do objeto digital: 10.1111/jns.12089


Descritores: Guillain-Barre Syndrome - Pathogenesis ; Guillain-Barre Syndrome - Proteins ; Guillain-Barre Syndrome - Antibodies ; Guillain-Barre Syndrome - Immunology


Data de publicação: 2014