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[免疫介导的神经病]

[Immune-mediated neuropathies].

作者信息

Kusunoki Susumu

机构信息

Department of Neurology, Kinki University School of Medicine.

出版信息

Rinsho Shinkeigaku. 2009 Nov;49(11):956-8. doi: 10.5692/clinicalneurol.49.956.

Abstract

Such neuropathies as Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and IgM paraproteinemic neuropathy are caused by autoimmune mechanisms. IgM paraproteinemic neuropathies are known to be intractable. Rituximab has recently been reported to be effective for IgM paraproteinemic neuropathy with anti-MAG IgM M-protein in a placebo-controlled trial. The effect should be confirmed with a larger trial. The use of this drug also may be tried for other type of IgM paraproteinemic neuropathy and for intractable CIDP in future. Antiganglioside IgG antibodies are frequently present in the acute-phase sera from GBS patients. Recently, presence of the antibodies that recognize a conformational epitope formed by carbohydrate portions of two different gangliosides (ganglioside complex) has been reported. Antibodies against a complex formed by GD1a and GD1b (anti-GD1a/GD1b antibodies) are associated with severe GBS. Anti-GM1/GalNAc-GD1a antibodies are found to be associated with pure motor GBS with frequent conduction block. Anti-ganglioside complex antibodies may be useful diagnostic and prognostic markers of GBS. Future study is necessary to clarify the pathogenetic mechanisms in which those antibodies are specifically involved.

摘要

诸如吉兰 - 巴雷综合征(GBS)、慢性炎症性脱髓鞘性多发性神经病(CIDP)和IgM副蛋白血症性神经病等神经病变是由自身免疫机制引起的。已知IgM副蛋白血症性神经病难以治疗。在一项安慰剂对照试验中,最近有报道称利妥昔单抗对伴有抗MAG IgM M蛋白的IgM副蛋白血症性神经病有效。该效果应通过更大规模的试验加以证实。未来也可尝试将这种药物用于其他类型的IgM副蛋白血症性神经病以及难治性CIDP。抗神经节苷脂IgG抗体在GBS患者的急性期血清中经常出现。最近,有报道称存在识别由两种不同神经节苷脂的碳水化合物部分形成的构象表位(神经节苷脂复合物)的抗体。针对由GD1a和GD1b形成的复合物的抗体(抗GD1a/GD1b抗体)与严重GBS相关。发现抗GM1/ GalNAc - GD1a抗体与伴有频繁传导阻滞的纯运动性GBS相关。抗神经节苷脂复合物抗体可能是GBS有用的诊断和预后标志物。有必要开展进一步研究以阐明这些抗体具体参与的致病机制。

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