Fig. 1

Representative animal models for GBS, CIDP, and underlying mechanisms. a Experimental autoimmune neuritis (EAN) is the animal model of acute peripheral neuropathies such as GBS. Induction is achieved by immunizing susceptible rodents (e.g., Lewis rats) with PNS myelin, peripheral myelin homogenates, myelin proteins (P0 or PMP22) or myelin-derived peptides. Immunization leads to a myelin-directed T cell response characterized by T cell and macrophage infiltration. Cytokine production mediates peripheral nerve damage and attraction of further inflammatory cells. b NOD B7-2^−/− and NOD ICAM1^−/− mice develop spontaneous CD4⁺ T cell-mediated neuritis with parallels to CIDP in humans. The neuritis is characterized by P0-specific CD4⁺ T cells that infiltrate into the PNS, IFN-γ dependency, an increased B cell reactivity to P0, expansion of P0-specific plasmablasts and lower frequencies of T_regs/B_regs. In the case of NOD ICAM1^−/− mice, mechanisms are slightly different than depicted here. In this case, the neuritis is characterized by enhanced IL-17 production, macrophage and B cell infiltration without changes in T_reg levels