Anti ‐nodal/paranodal antibodies in human demyelinating disorders

AbstractAnti ‐nodal/paranodal antibodies have been reported in human demyelinating disorders. Anti‐nodal protein antibodies, namely, anti‐neurofascin (NF) 186 antibodies and antibodies against paranodal proteins, such as NF155, contactin 1 (CNTN1), and contactin‐associated protein 1 (CASPR1), are found i n subsets of chronic inflammatory demyelinating polyneuropathy (CIDP). In particular, CIDP patients with IgG4 anti‐NF155 antibodies and those with IgG4 anti‐CNTN1 antibodies commonly show sensory ataxia, severe demyelination on nerve conduction studies, very high cerebrospinal (CSF) protein leve ls and poor response to intravenous immunoglobulin. However, younger age at onset, higher frequency of tremor, and nerve root hypertrophy are characteristic of anti‐NF155 antibody–positive CIDP, whereas anti‐CNTN1 antibody–positive CIDP is characterized by higher age at onset, early axonal d amage, subacute aggressive course, and concurrence of membranous nephropathy. Interestingly, anti‐NF155 antibody–positive CIDP occasionally accompanies central nervous system lesions suggestive of demyelination. Such cases are termed combined central and peripheral demyelination. Both CIDP with anti‐NF155 antibodies and CIDP with anti‐CNTN1 antibodies usually demonstrate detachment of terminal loops from the axolemma at the node of Ranvier in biopsied sural nerves. IgG4 in vivo is monovalent and bispecific and lacks complement‐binding capability; therefore, IgG4 autoa...

Source: Clinical and Experimental Neuroimmunology - March 31, 2020 Category: Neurology Authors: Hidenori Ogata Tags: REVIEW ARTICLE Source Type: research