Hospital Británico, Buenos Aires, Argentina.
Rev Neurol. 2023 Mar 16;76(6):209-211. doi: 10.33588/rn.7606.2021287.
Multifocal motor neuropathy (MMN) is a chronic progressive immune-mediated neuropathy, predominantly involving upper limbs asymmetrically with electrophysiologic evidence of motor conduction block. The treatment of choice is immunoglobulin (Ig). Nevertheless, some patients may become resistant to treatment. We describe a patient with history of MMN who became resistant to gammaglobulin treatment but markedly improved using ultra-high doses of intravenous immunoglobulin.
A 36-year-old woman with diagnosis of MMN. After 5 years of clinical stability under subcutaneous Ig (2g/kg/month) the patient developed bilateral weakness involving both hands. Treatment was switched to intravenous Ig 2g/kg/month, nevertheless, she progressed and became totally dependent for activities of daily living. We started ultra-high dose intravenous immunoglobulin 5 g/kg/month, with good response. She became independent for activities of daily living and returned to work. The only treatment related adverse event was headache during infusion.
Ultra-high dose intravenous Ig seems to be a useful therapy in aggressive MMN with severe disability despite conventional treatment. A low cardiovascular risk score (QRISK2 less than 10%) and a daily intravenous Ig lower than 35 g reduce the risk of severe complications related to intravenous Ig.
多灶性运动神经病（MMN）是一种慢性进行性免疫介导的神经病，主要累及上肢不对称，伴有运动传导阻滞的电生理证据。首选治疗方法是免疫球蛋白（Ig）。然而，一些患者可能对治疗产生耐药。我们描述了一位患有 MMN 病史的患者，该患者对丙种球蛋白治疗产生耐药，但使用超高剂量静脉注射免疫球蛋白治疗后明显改善。
一名 36 岁女性，诊断为 MMN。在接受皮下免疫球蛋白（2g/kg/月）治疗 5 年后，患者出现双侧手部无力。治疗改为静脉注射免疫球蛋白 2g/kg/月，但病情仍在进展，患者完全依赖日常生活活动。我们开始使用超高剂量静脉注射免疫球蛋白 5g/kg/月，效果良好。她恢复了日常生活活动能力并重返工作岗位。唯一与治疗相关的不良事件是输注期间头痛。
超高剂量静脉注射免疫球蛋白似乎是一种有用的治疗方法，适用于常规治疗无效、病情严重的侵袭性 MMN。心血管风险评分低（QRISK2<10%）和每日静脉注射免疫球蛋白低于 35g 可降低与静脉注射免疫球蛋白相关的严重并发症风险。
