慢性自己免疫性脱髄性ニューロパチー

Autoantibodies in Chronic Immune-Mediated Demyelinating Polyneuropathy

過去10年でランヴィエ絞輪部，傍絞輪部に局在する膜蛋白に対する自己抗体の存在が明らかとなり，自己免疫性ノドパチーの概念が提唱された。免疫グロブリンM（immunoglobulin M：IgM）単クローン血症に伴うミエリン関連糖蛋白質抗体は難治性脱髄性ニューロパチーを引き起こす。ジシアロシル基を有するガングリオシドに対するIgM自己抗体，IgM GM1抗体，IgG LM1抗体も各種慢性自己免疫性脱髄性ニューロパチーの診断・治療方針決定に役立つ。

Abstract

Autoantibodies against nodal and paranodal proteins, such as neurofascin 140/186, neurofascin 155, contactin 1, and contactin-associated protein 1, have been identified in subsets of patients with chronic inflammatory demyelinating polyneuropathy. Their distinctive characteristics including poor response to immunoglobulin led to the establishment of a new disease entity called “autoimmune nodopathies.” IgM monoclonal antibodies against myelin-associated glycoproteins cause intractable sensory-dominant demyelinating polyneuropathy. IgM anti-GM1 and IgG anti-LM1 antibodies are associated with multifocal motor neuropathy and chronic inflammatory demyelinating polyneuropathy, respectively. Monoclonal IgM against disialosyl ganglioside epitopes induces chronic ataxic neuropathy with ophthalmoplegia and cold agglutinin.