_{AIDP – acute inflammatoric demyelinating polyneuropathy, AMAN – acute motor axonal neuropathy, AMSAN – acute motor and sensory axonal neuropathy, CANOMAD -chronic ataxic neuropathy ophthalmoplegia IgM paraprotein cold agglutinins disialosyl antibodies, CIDP – chronic inflammatoric demyelinating polyneuropathy, CMV -cytomegalovirus, GBS – Guillain-Barré syndrome, MFS – Miller Fisher syndrome, MMN – multifocal motor neuropathy with conduction blocks, MN – motor neuropathy, ONS -acute paralysis of the oropharyngeal neck and shoulder muscles. Adapted from Conrad et al. 2011.}

Gangliosides GM1 and GD1a are strongly expressed at the nodes of Ranvier of myelinated axons, where the voltage-gated sodium (Nav) channels are localized. IgG anti-GM1 or anti-GD1a autoantibodies bind to the nodal axolemma, leading to membrane attack complex (MAC) formation. This results in the disappearance of Nav clusters and the detachment of paranodal myelin, which can lead to nerveconduction failure and muscle weak-ness. Axonal degeneration may follow at a later stage. Macrophages subsequently invade from the nodes into the periaxonal space, scavenging the injured axons.