Nobile-Orazio Eduardo, Cappellari Alberto, Priori Alberto
Dino Ferrari Centre and Centre of Excellence for Neurodegenerative Diseases, Department of Neurological Sciences, Milan University, IRCCS Ospedale Maggiore Policlinico, and Humanitas Clinical Institute, Milan, Italy.
Muscle Nerve. 2005 Jun;31(6):663-80. doi: 10.1002/mus.20296.
Multifocal motor neuropathy (MMN) is now a well-defined purely motor multineuropathy characterized by the presence of multifocal partial motor conduction blocks (CB), frequent association with anti-GM1 IgM antibodies, and usually a good response to high-dose intravenous immunoglobulin (IVIg) therapy. However, several issues remain to be clarified in the diagnosis, pathogenesis, and therapy of this condition including its nosological position and its relation to other chronic dysimmune neuropathies; the degree of CB necessary for the diagnosis of MMN; the existence of an axonal form of MMN; the pathophysiological basis of CB; the pathogenetic role of antiganglioside antibodies; the mechanism of action of IVIg treatments in MMN and the most effective regimen; and the treatment to be used in unresponsive patients. These issues are addressed in this review of the main clinical, electrophysiological, immunological, and therapeutic features of this neuropathy.
多灶性运动神经病（MMN）现在是一种明确的纯运动性多神经病，其特征为存在多灶性部分运动传导阻滞（CB），常与抗GM1 IgM抗体相关，且通常对大剂量静脉注射免疫球蛋白（IVIg）治疗反应良好。然而，在这种疾病的诊断、发病机制和治疗方面仍有几个问题有待阐明，包括其疾病分类位置及其与其他慢性免疫性神经病的关系；诊断MMN所需的CB程度；MMN轴索性形式的存在；CB的病理生理基础；抗神经节苷脂抗体的致病作用；IVIg治疗MMN的作用机制和最有效的治疗方案；以及对无反应患者应采用的治疗方法。本文对这种神经病的主要临床、电生理、免疫学和治疗特征进行综述，探讨了这些问题。
