Barrow Neurological Institute, Department of Neurology, Phoenix, AZ 85013, USA.
J Clin Neurosci. 2012 Sep;19(9):1201-9. doi: 10.1016/j.jocn.2012.02.011. Epub 2012 Jun 27.
Multifocal motor neuropathy (MMN) was first described in 1988 as a purely motor neuropathy affecting multiple motor nerves. The diagnosis was based entirely on demonstrating electrophysiological evidence of a conduction block (CB) that selectively affected motor axons, with sparing of sensory axons even through the site of motor CB. Subsequently, a similar disorder was reported but with absence of demonstrable CB on routine nerve conduction studies and there is still some debate as to whether MMN without CB is related to MMN. MMN is thought to be an inflammatory neuropathy related to an immune attack on motor nerves. The conventional hypothesis is that the primary pathology is segmental demyelination, but recent research raises the possibility of a primary axonopathy. Anti-GM1 antibodies can be found in some patients but it is unclear whether these antibodies are pathogenic. Intravenous immunoglobulin is the mainstay of treatment but other immunosuppressive treatments can also be effective.
多灶性运动神经病（MMN）于 1988 年首次被描述为一种纯粹影响多个运动神经的运动神经病。该诊断完全基于证明存在选择性影响运动轴突的电生理传导阻滞（CB）的证据，即使在运动 CB 部位，感觉轴突也不受影响。随后，报道了一种类似的疾病，但在常规神经传导研究中没有发现可证明的 CB，并且对于没有 CB 的 MMN 是否与 MMN 相关仍存在一些争议。MMN 被认为是一种与运动神经免疫攻击有关的炎症性神经病。传统假说认为主要病理学是节段性脱髓鞘，但最近的研究提出了原发性轴索病的可能性。一些患者可发现抗 GM1 抗体，但这些抗体是否具有致病性尚不清楚。静脉注射免疫球蛋白是主要的治疗方法，但其他免疫抑制治疗也可能有效。
