Combined Central and Peripheral Demyelination with Anti-Neurofascin155 IgG Following COVID-19 Vaccination

ADEM = acute demyelinating encephalomyelitis; CE = contrast enhancement; CSF = cerebrospinal fluid; CNS = central nervous system; DADS = distal acquired demyelinating symmetric neuropathy; EMG = electromyography and nerve conduction study; IL = interleukin; IVIg = intravenous immunoglobulin; PNS = peripheral nervous system. [...]finding seems to replicate the mechanisms of autoimmune-mediated complications of vaccination2 and is in accordance with data from a large CCPD case series, where recent vaccination/infection triggered two-thirds of cases.1 Peripheral nervous system (PNS) involvement can happen before, hand-in-hand with, or after central nervous system (CNS) involvement1,3,4 and influences the first treatment choice, as those with early PNS involvement might receive IVIg/plasmapheresis rather than steroids.1,4 CCPD has also been reported with anti-NF155 autoantibodies.3,4 This case exhibits some of the characteristic features of CCPD with anti-NF155 antibodies: the MS-like CNS involvement, with perivenular inflammation at periventricular sites (Figure 1D); absence of oligoclonal bands in CSF; damage in nerve roots and DADS.4 An attempt to better characterize the patient’s immune response has been made by measuring cytokines levels both on serum and on CSF across the stay. Here, although the temporal association of CCPD and vaccination does not prove any causal link, a connection with vaccine exposure must be considered, given the very rare nature of the syndrome and the negative findings emerging from all investigations.