Steck A J, Meier C, Vandevelde M, Regli F
Rev Neurol (Paris). 1984;140(1):28-36.
Two cases of polyneuropathy with IgM gammopathy are reported. Myelin associated glycoprotein is the antigen for the monoclonal antibody in both of these patients. The same antigenic specificity has now been identified in other patients having a paraproteinemia with a polyneuropathy. These findings suggest the existence of a new syndrome characterized by: a) a primary demyelinating neuropathy; b) a monoclonal IgM antibody to a specific glycoprotein component of myelin, referred to as myelin associated glycoprotein (MAG). As in other humorally mediated autoimmune diseases, it is suggested that demyelination is caused by circulating anti-MAG antibodies. The role of immunosuppressive drugs and plasmapheresis is discussed.
报告了两例伴有IgM丙种球蛋白病的多发性神经病。髓鞘相关糖蛋白是这两名患者单克隆抗体的抗原。现在在其他患有副蛋白血症伴多发性神经病的患者中也发现了相同的抗原特异性。这些发现提示存在一种新的综合征,其特征为:a)原发性脱髓鞘性神经病;b)针对髓鞘特定糖蛋白成分的单克隆IgM抗体,即髓鞘相关糖蛋白(MAG)。如同其他体液介导的自身免疫性疾病一样,提示脱髓鞘是由循环中的抗MAG抗体引起的。文中讨论了免疫抑制药物和血浆置换的作用。