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

N2 Year in Review

“…Importantly, more than 80% of the patients did not respond to IVIg or steroids but responded only to rituximab, confirming the original observation by Querol et al 39 In all rituximab-treated patients, the IgG4 antibody titers substantially decreased correlating with clinical response, suggesting that specific antibody titers may serve as a guide for reinfusion decisions. Because NF155 is expressed in the central and peripheral nervous systems, Pegat et al 40 showed in an important case report that anti-NF155 antibodies can be also associated with a demyelinating disease concurrently affecting both the CNS and the PNS. Furthermore, in a large series of patients with nodo-paranodopathy, Appelttshauser et al 41 showed a higher incidence (33%) of diabetes and a 3.4-fold higher risk of diabetes mellitus in these patients compared with the general population.…”