Lange D J, Trojaborg W
Neurological Institute Columbia-Presbyterian Medical Center, New York, New York 10032.
Muscle Nerve. 1994 Jan;17(1):105-7. doi: 10.1002/mus.880170116.
We review clinical, neurophysiological, immunological, and experimental data concerning multifocal motor neuropathy (MMN), a newly recognized disorder that mimics MND. It is separated from MND by the presence of multifocal conduction block (CB) demonstrated electrophysiologically, and in some instances by the association of high titers of GM1 antibodies. The possible immunopathogenetic effect of GM1 antibodies is discussed. However, 70% of patients with MMNCB do not have elevated titers of GM1 antibodies, but may respond nevertheless to immunosuppressive treatment. Thus, so far unrecognized antibodies may react against some other epitopes in the paranodal region than those attacked by GM1 antibodies to cause CB.
我们回顾了有关多灶性运动神经病(MMN)的临床、神经生理学、免疫学及实验数据,MMN是一种新认识的类似运动神经元病(MND)的疾病。通过电生理证实的多灶性传导阻滞(CB)可将其与MND区分开来,在某些情况下,还可通过高滴度GM1抗体的存在来区分。文中讨论了GM1抗体可能的免疫致病作用。然而,70%的多灶性运动神经病伴传导阻滞(MMNCB)患者GM1抗体滴度并未升高,但仍可能对免疫抑制治疗有反应。因此,迄今未被识别的抗体可能针对结旁区中某些不同于GM1抗体攻击的表位发生反应,从而导致传导阻滞。