Paranodal lesions in chronic inflammatory demyelinating polyneuropathy associated with anti-neurofascin 155 antibodies

Highlights • Anti-Neurofascin 155 and anti-Contactin-1 IgG4 antibodies have recently been associated with subgroups of CIDP. • These patients presented ataxia, tremor, and a poor response to intravenous immunoglobulin. • Antibodies to Contactin-1 appear to induce paranodal alterations in skin biopsies from CIDP patients and in animal models. • Characteristic ultrastructural lesions are observed on sural nerves of these patients. • Multiple mechanisms can be responsible for the physiopathology of CIDP, including IgG4-mediated demyelination.