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Human immunoglobulin treatment of multifocal motor neuropathy and polyneuropathy associated with monoclonal gammopathy.

机译:人免疫球蛋白治疗多发性运动神经病和多发性神经病与单克隆丙种球蛋白病相关。

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摘要

Intravenous human immune globulin (IVIg) has been proposed for the treatment of various peripheral neuropathies that are considered to be immunemediated. The results are reported of an open trial conducted in multifocal motor neuropathy and polyneuropathy associated with monoclonal gammopathy. Six cases with multifocal motor neuropathy, selected on clinical and electrophysiological criteria (four of six patients also had significantly high anti-GM1 titres), received IVIg monthly, at doses varying from 1.6 to 2.5 mg/kg, over three to 13 months. The initial response to treatment was dramatic in 3/6 cases (with improvement of at least two grades on the MRC scale in the five more severely affected muscles). The final evaluation showed a good result in 4/6 cases, but the conduction blocks were not significantly reduced. In 13 other cases with polyneuropathy associated with IgM monoclonal gammopathy of unknown significance, IVIg was of benefit, with improvement of at least one grade on the Prineas score, in 4/7 cases previously treated with immunosuppression and 2/3 cases not treated before IVIg. In the last group of four patients with polyneuropathy and IgG monoclonal gammopathy, IVIg was followed by clinical improvement in the two cases with a chronic demyelinating polyneuropathy.
机译:已提出静脉内人免疫球蛋白(IVIg)用于治疗被认为是免疫介导的各种周围神经病。结果报告了一项针对多灶性运动神经病和与单克隆丙种球蛋白病相关的多发性神经病的公开试验。根据临床和电生理标准选择的6例多灶性运动神经病(6例患者中有4例也具有明显高的抗GM1滴度),在3到13个月内每月接受IVIg,剂量从1.6到2.5 mg / kg不等。对治疗的最初反应在3/6例中是戏剧性的(在五个受更严重影响的肌肉中,MRC评分至少改善了两个等级)。最终评估显示4/6例结果良好,但传导阻滞并未明显减少。在其他13例具有未知意义的IgM单克隆丙种球蛋白病相关性多发性神经病的病例中,IVIg有益,在先前进行过免疫抑制治疗的4/7例患者和在IVIg之前未治疗的2/3例中,Prineas评分至少提高了一个等级。 。在最后一组四名患有多发性神经病和IgG单克隆丙种球蛋白病的患者中,IVIg继之以慢性脱髓鞘性多发性神经病的两例临床改善。

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