一名单克隆IgM κ与施密特-兰特尔曼切迹反应阳性患者的轴索性神经病

Axonal neuropathy in a patient with monoclonal IgM kappa reactive with Schmidt-Lantermann incisures.

作者信息

Quattrini A, Nemni R, Fazio R, Iannaccone S, Lorenzetti I, Grassi F, Canal N

机构信息

Department of Neurology, University of Milan, Istituto Scientifico S. Raffaele, Italy.

出版信息

J Neuroimmunol. 1991 Jul;33(1):73-9. doi: 10.1016/0165-5728(91)90036-7.

DOI:10.1016/0165-5728(91)90036-7

PMID:1647411

Abstract

We report a patient with a progressive, predominantly sensory neuropathy and a IgM kappa M-protein that binds to Schmidt-Lantermann incisures. A sural nerve biopsy showed primary axonal damage and IgM deposits at Schmidt-Lantermann incisures were seen by direct immunoperoxidase. Serum from the patient injected into rat sciatic nerve reacts with the incisures as with those in the patient's nerve. The IgM kappa M-protein reacts with chondroitin sulfate C and binds to a broad nerve protein band with a mobility of between 170 and 118 kDa. Peripheral neuropathy may be related to the M-protein, which had immunocytochemical reactivity not previously described for patients with polyneuropathy and IgM monoclonal gammopathy.

摘要

我们报告了一名患有进行性、以感觉神经病变为主且伴有与施密特-兰特曼切迹结合的IgM κ型M蛋白的患者。腓肠神经活检显示原发性轴索损伤，直接免疫过氧化物酶法可见施密特-兰特曼切迹处有IgM沉积。将患者的血清注射到大鼠坐骨神经中，其与切迹的反应与患者神经中的反应相同。该IgM κ型M蛋白与硫酸软骨素C反应，并与迁移率在170至118 kDa之间的一条宽神经蛋白带结合。周围神经病变可能与该M蛋白有关，该M蛋白具有免疫细胞化学反应性，这在多神经病和IgM单克隆丙种球蛋白病患者中此前未曾描述过。