Kelly J J, Adelman L S, Berkman E, Bhan I
Department of Neurology, Tufts Medical School, Boston.
Arch Neurol. 1988 Dec;45(12):1355-9. doi: 10.1001/archneur.1988.00520360073015.
We studied ten patients with IgM monoclonal gammopathies. Five had M proteins that reacted with myelin-associated glycoprotein (MAG) and five had no recognizable antinerve activity. The neuropathy in the MAG-reactive patients was homogeneous by clinical and laboratory analysis, while the neuropathy in the MAG-nonreactive patients varied considerably. Both groups responded well to immunosuppressive therapy, which lowered the concentration of the serum M protein. The homogeneity of the MAG-reactive patients and their response to sustained lowering of the M protein levels support the concept that the IgM M protein directly damages nerve fibers and is the proximate cause of the polyneuropathy.
我们研究了10例IgM单克隆丙种球蛋白病患者。其中5例患者的M蛋白与髓鞘相关糖蛋白(MAG)发生反应,另外5例患者未表现出可识别的抗神经活性。通过临床和实验室分析,MAG反应性患者的神经病变具有同质性,而MAG无反应性患者的神经病变差异很大。两组患者对免疫抑制治疗均反应良好,血清M蛋白浓度降低。MAG反应性患者的同质性及其对M蛋白水平持续降低的反应支持了IgM M蛋白直接损害神经纤维并是多发性神经病的直接原因这一概念。