静脉注射免疫球蛋白治疗伴有IgM型丙种球蛋白病的脱髓鞘性神经病的对照研究。

A controlled study of intravenous immunoglobulin in demyelinating neuropathy with IgM gammopathy.

作者信息

Dalakas M C, Quarles R H, Farrer R G, Dambrosia J, Soueidan S, Stein D P, Cupler E, Sekul E A, Otero C

机构信息

Neuromuscular Diseases Section, Laboratory of Molecular and Cellular Neurobiology, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892-1382, USA.

出版信息

Ann Neurol. 1996 Nov;40(5):792-5. doi: 10.1002/ana.410400516.

DOI:10.1002/ana.410400516

PMID:8957021

Abstract

Eleven patients with demyelinating polyneuropathy associated with monoclonal IgM antibodies were randomized to receive IVIg or placebo, monthly, for 3 months in a double-blind study. After a washout period, they crossed over to the alternate therapy. Response was gauged by evaluating muscle strength, sensation, and neuromuscular symptoms at baseline, after 3 months, and at treatment's end. After IVIg therapy, the strength improved in only 2 of 11 patients, by 28 and 38.5 points from baseline, and declined after placebo. In 1 other patient, the sensory score improved by 13 points. Antibody titers to MAG/SGPG or gangliosides did not appreciably change. We conclude that IVIg has only a modest benefit to not more than 18% of patients with IgM paraproteinemic demyelinating neuropathy.

摘要

在一项双盲研究中，11例与单克隆IgM抗体相关的脱髓鞘性多发性神经病患者被随机分组，每月接受静脉注射免疫球蛋白（IVIg）或安慰剂治疗，为期3个月。经过洗脱期后，他们交叉接受替代治疗。通过在基线、3个月后和治疗结束时评估肌肉力量、感觉和神经肌肉症状来衡量反应。IVIg治疗后，11例患者中只有2例力量有所改善，较基线分别提高了28分和38.5分，而在接受安慰剂治疗后力量下降。在另1例患者中，感觉评分提高了13分。针对髓鞘相关糖蛋白/硫酸糖蛋白聚糖（MAG/SGPG）或神经节苷脂的抗体滴度没有明显变化。我们得出结论，IVIg对不超过18%的IgM副蛋白血症性脱髓鞘性神经病患者只有适度益处。