Neurofascin antibodies in chronic inflammatory demyelinating polyradiculoneuropathy: from intrinsic genetic background to clinical manifestations

Combined Central and Peripheral Demyelination With IgM Anti–Neurofascin 155 Antibodies

“…CIDP associated with IgG4 anti-NF155 Ab is rare (5%–10% of CIDP cases) and presents specific clinical characteristics: young age at onset, distal motor lower limb weakness, sensory ataxia, tremor, poor response to IVIg, and good response to corticosteroids and rituximab. 1 NF155 protein is expressed in both the central and peripheral nervous systems explaining the CCPD due to anti-NF155 Ab (up to half the cases of CCPD reported in a Japanese study). 1 …”

“… 1 NF155 protein is expressed in both the central and peripheral nervous systems explaining the CCPD due to anti-NF155 Ab (up to half the cases of CCPD reported in a Japanese study). 1 …”

“… 8 Anti-CD20 is a classical treatment of multiple sclerosis and is sometimes effectively used in CIDP, especially with IgG4 anti-NF155, and has been found effective in CCPD. 1 …”

“…Immunoglobulin (Ig) G anti–neurofascin 155 antibodies (anti-NF155 Abs) have been described in rare forms of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and in combined central and peripheral demyelination (CCPD). 1 We report herein a case of a patient presenting CCPD and IgM anti-NF155 Ab.…”

Novel Immunological and Therapeutic Insights in Guillain-Barré Syndrome and CIDP

“…In the case of anti-NF155 IgM antibodies, their pathogenic effect is supported by a study of Ng et al that reported enhancement and prolongation of experimental autoimmune neuritis (EAN) in rats after injection of mouse anti-NF155 IgM, but not by injection of an isotype control [ 34 ]. Whether anti-NF IgM presents only at disease onset in anti-NF155 AN and then the isotype switch elicits an IgG4 response or anti-NF IgM persists during the course of the disease in patients with predominant anti-NF IgM needs more research [ 35 ].…”