Kanda So, Kanda Takashi
Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.
Brain Nerve. 2024 May;76(5):526-533. doi: 10.11477/mf.1416202639.
Multifocal motor neuropathy (MMN), an acquired chronic progressive immune-mediated motor neuropathy, is characterized by asymmetrical distal upper limb muscle weakness and muscle atrophy without sensory impairment. Differentiation from amyotrophic lateral sclerosis is usually challenging, and electrophysiological studies show multifocal conduction blocks. Immunoglobulin (Ig)M GM1 antibodies are detected in approximately 50% of patients. In contrast to chronic inflammatory demyelinating polyneuropathy, corticosteroids are ineffective for management of MMN, and IVIg is the sole established treatment.
多灶性运动神经病(MMN)是一种获得性慢性进行性免疫介导的运动神经病,其特征为不对称性远端上肢肌肉无力和肌肉萎缩,无感觉障碍。与肌萎缩侧索硬化症进行鉴别通常具有挑战性,电生理研究显示多灶性传导阻滞。约50%的患者可检测到免疫球蛋白(Ig)M GM1抗体。与慢性炎症性脱髓鞘性多发性神经病不同,皮质类固醇对MMN的治疗无效,静脉注射免疫球蛋白(IVIg)是唯一已确立的治疗方法。