Anti-MAG neuropathy: From biology to clinical management

J Neuroimmunol. 2021 Dec 15:361:577725. doi: 10.1016/j.jneuroim.2021.577725. Epub 2021 Sep 28.

Abstract

The acquired chronic demyelinating neuropathies include a growing number of disease entities that have characteristic, often overlapping, clinical presentations, mediated by distinct immune mechanisms, and responding to different therapies. After the discovery in the early 1980s, that the myelin associated glycoprotein (MAG) is a target antigen in an autoimmune demyelinating neuropathy, assays to measure the presence of anti-MAG antibodies were used as the basis to diagnose the anti-MAG neuropathy. The route was open for describing the clinical characteristics of this new entity as a chronic distal large fiber sensorimotor neuropathy, for studying its pathogenesis and devising specific treatment strategies. The initial use of chemotherapeutic agents was replaced by the introduction in the late 1990s of rituximab, a monoclonal antibody against CD20+ B-cells. Since then, other anti-B cells agents have been introduced. Recently a novel antigen-specific immunotherapy neutralizing the anti-MAG antibodies with a carbohydrate-based ligand mimicking the natural HNK-1 glycoepitope has been described.

Keywords: Antibody; Demyelinating; Myelin associated glycoprotein; Neuropathy; Therapy.

Publication types

  • Review

MeSH terms

  • Adenine / analogs & derivatives
  • Adenine / therapeutic use
  • Animals
  • Autoantibodies / blood
  • Autoantibodies / immunology
  • Autoantigens / immunology*
  • B-Lymphocyte Subsets / immunology
  • CD57 Antigens / immunology
  • Demyelinating Autoimmune Diseases, CNS / diagnosis
  • Demyelinating Autoimmune Diseases, CNS / immunology*
  • Demyelinating Autoimmune Diseases, CNS / therapy
  • Epitopes / immunology
  • Gait Disorders, Neurologic / immunology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Immunotherapy
  • Lenalidomide / therapeutic use
  • Mammals
  • Mice
  • Molecular Mimicry
  • Myelin Sheath / chemistry
  • Myelin Sheath / immunology
  • Myelin Sheath / ultrastructure
  • Myelin-Associated Glycoprotein / immunology*
  • Nerve Fibers, Myelinated / immunology
  • Nerve Fibers, Myelinated / pathology
  • Nervous System Autoimmune Disease, Experimental / immunology
  • Paraproteinemias / immunology
  • Paraproteins / immunology
  • Piperidines / therapeutic use
  • Plasma Exchange
  • Polyradiculoneuropathy / diagnosis
  • Polyradiculoneuropathy / immunology*
  • Polyradiculoneuropathy / therapy
  • Ranvier's Nodes / chemistry
  • Ranvier's Nodes / immunology
  • Rats
  • Rituximab / therapeutic use

Substances

  • Autoantibodies
  • Autoantigens
  • CD57 Antigens
  • Epitopes
  • Immunosuppressive Agents
  • MAG protein, human
  • Myelin-Associated Glycoprotein
  • Paraproteins
  • Piperidines
  • ibrutinib
  • Rituximab
  • Lenalidomide
  • Adenine