Immune-mediated insights into clinical and specific autoantibodies in acute and chronic immune-mediated nodo-paranodopathies

Abstract

The recognition of the molecular structures, namely the node of Ranvier and the axonal regions surrounding it (the paranode and juxtaparanode), as the primary target for specific autoantibodies has introduced a new site for neurological location (microtopographic structures), in contrast to the prevailing understanding, in which lesions to neural macrostructures (roots, nerves, and/or plexus) were the focus of semiologists and electrophysiologists for topographic, syndromic, and nosological diagnoses. Therefore, there was a need to understand and characterize the components of these neural microstructures that are grouped in small regions within the nerve to optimize clinical and therapeutic reasoning.

Figure 1

Nodal, paranodal, and juxtaparanodal regions with their main antigens, the presence of sodium channels in the nodal region, and of potassium channels in the juxtaparanodal regions. Predominance of immunoglobulin G1 (IgG1) to IG3 and IgM antibodies in the nodal region, and of IgG4 and 3 antibodies in the paranodal and juxtaparanodal regions.