Rudolf Magnus Institute of Neuroscience, Department of Neurology, University Medical Center Utrecht, Heidelberglaan 100, Utrecht, the Netherlands.
J Clin Immunol. 2010 May;30 Suppl 1(Suppl 1):S79-83. doi: 10.1007/s10875-010-9408-3.
Multifocal motor neuropathy (MMN) is characterized by asymmetric weakness of limbs and the electrophysiological finding of conduction block in motor nerves. Conduction block is the inability of nerves to propagate action potentials and is probably caused by immune-mediated dysfunction of the axon at the nodes of Ranvier or the myelin sheath. MMN immune pathogenesis has not been elucidated.
In approximately 50% of all patients, IgM antibodies that bind to the glycolipid GM1, which is abundantly expressed in peripheral motor nerves, can be detected. A recent study showed an association with HLA-DRB1*15, and virtually all patients respond to treatment with intravenous immunoglobulin (IVIG) in at least the early stages of the disease.
This review aims at providing a concise overview of what is known about MMN pathogenesis, and how the beneficial effect of IVIG might be explained.
多灶性运动神经病（MMN）的特征是四肢不对称性无力，以及运动神经的电生理学发现存在传导阻滞。传导阻滞是指神经不能传播动作电位，可能是由于Ranvier 结或髓鞘处的轴突免疫介导功能障碍引起的。MMN 的免疫发病机制尚未阐明。
大约 50%的所有患者都可以检测到针对富含于周围运动神经的糖脂 GM1 的 IgM 抗体。最近的一项研究显示与 HLA-DRB1*15 有关，实际上所有患者在疾病的早期阶段至少对静脉注射免疫球蛋白（IVIG）治疗有反应。
本文旨在简要概述 MMN 发病机制的已知内容，以及 IVIG 的有益作用如何得到解释。
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