A case with Neurofascin-155 IgG antibody-associated combined central and peripheral demyelination: Successfully treated with anti-CD20 monoclonal antibody

Combined central and peripheral demyelination (CCPD) is an infrequent entity in which demyelination is observed in central (CNS) and peripheral nervous systems (PNS). Potentially, it may develop due to a shared immune mechanism or possible co-occurrence between two unrelated demyelinating diseases such as multiple sclerosis (MS) and chronic inflammatory demyelination polyneuropathy (CIDP). A small number of CIDP patients have autoantibodies against nodal and paranodal proteins such as neurofascin155 (NF155). NF acts as a cell adhesion molecule between nodal and paranodal proteins. Glial NF 155 coexists in the PNS and CNS and can lead to combined demyelination. Although NF antibody-positive CIDP cases and case series have been reported, the number of patients with overt manifestations of central nervous system demyelination is very low in this group. The response to intravenous immunoglobulin (IVIg) in anti NF155 antibody-positive (NF155 +) CIDP is known to be poor. Rituximab, a B-cell-targeted anti-CD20 monoclonal antibody, has made good progress in therapy. Here, we report a case with Neurofascin-155 IgG antibodies related to CCPD who responded well to Rituximab. NF155+ CIDP usually affects young adults, and early administration of appropriately combined immunotherapy can prevent severe disability. NF antibody testing should be performed in unresponsive patients to IVIg therapy.