Department of Neurology, University of Wuerzburg, Wuerzburg, Germany.
Eur Neurol. 2010;63(4):193-204. doi: 10.1159/000282734. Epub 2010 Feb 11.
Multifocal motor neuropathy (MMN) is an acquired immune-mediated neuropathy characterized by chronic or stepwise progressive asymmetrical limb weakness without sensory deficits. The upper extremities are more often affected than the lower extremities with distal paresis dominating over proximal paresis. Important diagnostic features are persistent multifocal partial conduction blocks (CBs) and the presence of high-titer anti-GM1 serum antibodies. Motor neuron disease, other chronic dysimmune neuropathies, such as chronic inflammatory demyelinating polyneuropathy and the Lewis-Sumner syndrome (MADSAM neuropathy), are important differential diagnoses. While corticosteroids and plasma exchange are largely ineffective, high-dose intravenous immunoglobulins are regarded as first-line treatment. In spite of significant success in elucidating the underlying disease mechanisms in MMN during the past few years, important pathophysiological issues and the optimum long-term therapy remain to be clarified. The present review summarizes the clinical picture and current pathophysiological concepts of MMN with a special focus on the molecular and electrophysiological basis of CBs and highlights established therapies as well as possible novel treatment options.
多灶性运动神经病（MMN）是一种获得性免疫介导的神经病，其特征为慢性或进行性逐步加重的非对称性肢体无力，无感觉缺失。上肢比下肢更容易受累，以远端无力为主，近端无力为辅。重要的诊断特征是持续存在多灶性部分传导阻滞（CB）和高滴度抗 GM1 血清抗体。运动神经元病、其他慢性免疫性神经病，如慢性炎症性脱髓鞘性多发性神经病和 Lewis-Sumner 综合征（MADSAM 神经病），是重要的鉴别诊断。虽然皮质类固醇和血浆置换的效果不大，但是大剂量静脉注射免疫球蛋白被认为是一线治疗。尽管在过去几年中，在阐明 MMN 的潜在疾病机制方面取得了重大进展，但重要的病理生理问题和最佳长期治疗仍有待阐明。本综述总结了 MMN 的临床表现和当前的病理生理概念，特别关注 CB 的分子和电生理基础，并强调了已确立的治疗方法以及可能的新治疗选择。
