Autoimmune nodopathies: treatable neuropathies beyond traditional classifications

Precise characterisation of the target antigens and autoantibody isotypes improves patient care in inflammatory neuropathies

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-Barré syndrome. Fehmi et al describe that anti-pan-neurofascin IgG1 antibodies associate with an aggressive (often fatal) neuropathy, leading to profound tetraplegia, intensive care unit admission and,…

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