Anti-MAG neuropathy: From biology to clinical management

• Published in: Journal of neuroimmunology 2021-12, Vol.361, p.577725-577725, Article 577725
• Main Author: Steck, Andreas J.
• Format: Article
• Language: English
• Subjects: Adenine - analogs & derivatives; Adenine - therapeutic use; Animals; Antibody; Autoantibodies - blood; Autoantibodies - immunology; Autoantigens - immunology; B-Lymphocyte Subsets - immunology; CD57 Antigens - immunology; Demyelinating; 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 associated glycoprotein; 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; Neuropathy; 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; Therapy
• ISSN: 0165-5728; 1872-8421
• DOI: 10.1016/j.jneuroim.2021.577725

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. •Discovery of antigenic targets: crucial step in understanding autoimmune neuropathies.•Antibodies against MAG characterize a demyelinating neuropathy.•B cells depleting therapies are main therapeutical mode of treatment.•New treatment modalities include inhibitors or antigen specific immunotherapy.