[Pan-neurofascin autoimmune nodopathy - an emerging and treatable differential diagnosis to Guillain-Barré syndrome].

Abstract

Autoimmune nodopathy (AN) is an emerging diagnostic category with antibodies targeting the peripheral node of Ranvier. Clinically, AN overlaps with Guillain-Barré syndrome (GBS) and acute-onset CIDP. However, the immunopathology of AN is different, hence AN is considered as a separate entity. Antibodies targeting all neurofascin isoforms are associated with a phenotype and immunopathology described as pan-neurofascin (panNF) AN. Here, we describe a clinical and serological work-up of two patients with anti-panNF IgG4 AN, as well as treatment response. Due to the high morbidity of panNF and its treatable nature, we suggest early antibody testing in severe cases of GBS, especially when a prolonged need for invasive ventilation occurs. Specialized intensive care rehabilitation promotes autonomy and recovery, and should be considered as an alternative to traditional ICU care.