Leger J M, Younes-Chennoufi A B, Chassande B, Davila G, Bouche P, Baumann N, Brunet P
Service de Neurologie, Hôpital de la Salpêtrière, Paris, France.
J Neurol Neurosurg Psychiatry. 1994 Nov;57 Suppl(Suppl):46-9. doi: 10.1136/jnnp.57.suppl.46.
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.
静脉注射人免疫球蛋白（IVIg）已被提议用于治疗各种被认为是免疫介导的周围神经病。本文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进行的开放试验结果。根据临床和电生理标准选取了6例多灶性运动神经病患者（6例患者中有4例抗GM1滴度也显著升高），每月接受IVIg治疗，剂量为1.6至2.5mg/kg，持续3至13个月。3/6的病例对治疗的初始反应显著（在5块受影响更严重的肌肉中，医学研究委员会（MRC）量表评分至少提高了两个等级）。最终评估显示4/6的病例效果良好，但传导阻滞并未显著减轻。在另外13例与意义未明的IgM单克隆丙种球蛋白病相关的多发性神经病患者中，IVIg有帮助，在7例先前接受免疫抑制治疗的病例中有4例、在3例IVIg治疗前未接受治疗的病例中有2例，普里尼亚斯评分至少提高了一个等级。在最后一组4例患有多发性神经病和IgG单克隆丙种球蛋白病的患者中，2例患有慢性脱髓鞘性多发性神经病的病例在接受IVIg治疗后临床症状得到改善。
