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高剂量静脉注射免疫球蛋白对多灶性运动神经病患者耐受性良好且疗效显著。

High-dose Ig VENA is well tolerated and efficacious in patients with multifocal motor neuropathy.

作者信息

Nobile-Orazio Eduardo, Cocito Dario, Briani Chiara, Plasmati Rosaria, Schenone Angelo, Gallia Francesca, Marjanovic Ivan, Suffredini Anna Lotti

机构信息

Department of Medical Biotechnology and Translational Medicine (BIOMETRA), Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Centre, Milan University, Rozzano, Milan, Italy.

Department of Medical Biotechnology and Translational Medicine, Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Institute, Milan University, Via Manzoni 56, 20089, Rozzano, Milan, Italy.

出版信息

Neurol Sci. 2017 May;38(5):899-902. doi: 10.1007/s10072-017-2826-8. Epub 2017 Jan 31.

Abstract

Multifocal motor neuropathy (MMN) is a rare, chronic, motor neuropathy that progressively impairs physical functioning and quality of life. Randomised controlled trials have shown that high-dose intravenous immunoglobulin (IVIg) is superior to placebo in improving muscle strength and disability, but many patients require periodic infusions to maintain long-term improvement. This observational, multicentre, retrospective study investigated the efficacy and tolerability of human normal immunoglobulin (Ig VENA) at high intravenous infusion rates in 20 MMN patients (14 male, 6 female). Thirty days after the first infusion, there was an improvement of at least 1 point in two muscles compared to baseline on the Medical Research Council (MRC) scale and of 1 point in the ONLS (Overall Neuropathy Limitation Scale) scale in 15 patients (75%) and 10 patients (50%), respectively; 45% improved on both scales. At 6 months, 100% of 12 patients had improved on the MRC and 79% of 14 patients had improved on the ONLS scale; 83% improved on both scales. All reported adverse drug reactions (ADR) were mild, transient and possibly related to the study drug. Four patients (20%) reported ADRs, three reported headache and one fever. There were no serious or unexpected ADRs. By confirming that high-dose Ig VENA is efficacious and well tolerated, this study adds to the evidence base for IVIg in MMN and potentially increases clinicians' and patients' choice of therapy.

摘要

多灶性运动神经病(MMN)是一种罕见的慢性运动神经病,会逐渐损害身体功能和生活质量。随机对照试验表明,高剂量静脉注射免疫球蛋白(IVIg)在改善肌肉力量和功能障碍方面优于安慰剂,但许多患者需要定期输注以维持长期改善效果。这项观察性、多中心、回顾性研究调查了20例MMN患者(14例男性,6例女性)以高静脉输注速率使用人正常免疫球蛋白(Ig VENA)的疗效和耐受性。首次输注后30天,与基线相比,15例患者(75%)的医学研究委员会(MRC)量表上至少有两块肌肉改善了1分,10例患者(50%)的总体神经病变限制量表(ONLS)上改善了1分;45%的患者在两个量表上均有改善。在6个月时,12例患者中有100%在MRC量表上有所改善,14例患者中有79%在ONLS量表上有所改善;83%的患者在两个量表上均有改善。所有报告的药物不良反应(ADR)均为轻度、短暂性,可能与研究药物有关。4例患者(20%)报告了ADR,3例报告头痛,1例报告发热。没有严重或意外的ADR。通过证实高剂量Ig VENA有效且耐受性良好,本研究为MMN中IVIg的证据库增添了内容,并可能增加临床医生和患者的治疗选择。

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