Tagaya N, Saito T, Fujioka T, Honda M, Kinoshita M
Fourth Department of Internal Medicine, School of Medicine, Toho University.
Rinsho Shinkeigaku. 1993 Mar;33(3):322-6.
A 57-year-old man was admitted to our hospital complaining of slowly progressive numbness over the hands and legs. Neurological examinations revealed marked loss of superficial sensation of glove-stocking type, mild distal weakness over the extremities and diffuse hyporeflexia. Laboratory examinations disclosed marked increase of serum IgM level and significantly increased kappa-light chain of IgM immunoelectrophoresis. Bone marrow aspiration showed no evidence of malignancy. Motor and sensory nerve conduction velocities were decreased. Sural nerve biopsy was characterized by marked loss of large myelinated fibers, onion bulb formation and widely spaced major dense lines of myelin. The immunological examinations revealed two different antibody activities of IgM against myelin-associated glycoprotein (MAG) and GM1 in serum. Treatment with prednisolone (PSL) 60 mg daily did not improve his neurologic signs and symptoms. Then double-filtration plasmapheresis was performed, which improved the neurologic symptoms and decreased both anti-MAG and anti-GM1 antibody titers. This is the first Japanese case presenting IgM paraproteinemic polyneuropathy with simultaneous occurrence of anti-MAG and anti-GM1 antibodies.
一名57岁男性因双手和双腿逐渐出现麻木感而入住我院。神经学检查发现呈手套 - 袜套型的浅感觉明显丧失、四肢远端轻度无力及弥漫性反射减弱。实验室检查显示血清IgM水平显著升高，IgM免疫电泳的κ轻链明显增加。骨髓穿刺未发现恶性肿瘤迹象。运动和感觉神经传导速度降低。腓肠神经活检的特征是大的有髓纤维明显丧失、洋葱球形成以及髓鞘主要致密线间距增宽。免疫学检查发现血清中IgM针对髓鞘相关糖蛋白（MAG）和GM1有两种不同的抗体活性。每日使用60毫克泼尼松龙（PSL）治疗并未改善其神经症状和体征。随后进行了双重过滤血浆置换，改善了神经症状并降低了抗MAG和抗GM1抗体滴度。这是日本首例同时出现抗MAG和抗GM1抗体的IgM副蛋白血症性多发性神经病病例。
