Autoimmune nodopathies: treatable neuropathies beyond traditional classifications

Antibodies targeting all neurofascin isoforms (pan-neurofascin) have been associated with variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) that include ataxic presentations, cranial nerve involvement, respiratory failure and association with other autoimmune disorders .1 2 Fehmi et al describe a severe, yet treatable, neuropathy associated with anti-pan-neurofascin antibodies of the IgG1 isotype.3 Nodo-paranodal antibodies have been described in neuropathies fulfilling CIDP diagnostic criteria. However, a significant proportion of patients harbouring any nodo-paranodal autoantibody (contactin-1, contactin-associated protein-1, neurofascin-155 or pan-neurofascin) presents as rapidly progressive, aggressive (including the need of intensive care unit admission and need of mechanical ventilation) neuropathies, often leading to an initial diagnosis of Guillain-Barre syndrome. Fehmi et al describe that anti-pan-neurofascin IgG1 antibodies associate with an …