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Guillain‐Barré syndrome associated with CASPR2 antibodies: two paediatric cases
Author(s) -
Rosch Richard E.,
Bamford Alasdair,
Hacohen Yael,
Wraige Elizabeth,
Vincent Angela,
Mewasingh Leena,
Lim Ming
Publication year - 2014
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1111/jns.12089
Subject(s) - guillain barre syndrome , antibody , autoantibody , neuromyotonia , medicine , pathogenesis , immunology , ganglioside , disease , antigen , neuroimmunology , pathology , biology , immune system , genetics
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.