Saito T, Irie S, Ito H, Kowa H
Department of Neurology, Kitasato University East Hospital.
Rinsho Shinkeigaku. 1992 Oct;32(10):1146-8.
In some patients, motor neuron disease (MND) is associated with a paraprotein, raising the question that there may be a relationship between MND and monoclonal gammopathy. We analysed the presence of monoclonal immunoglobulin in the serum from 53 patients with MND using immunofixation in cellulose acetate membrane. The incidence of paraproteins in 6 cases (11.3%) of patients with MND exceeds values found with cellulose acetate membrane supports in reported populations (below 1%), even when age was also considered. Monoclonal components were IgG (33%), IgM (33%) and IgA (33%). In six cases, four showed typical changes of amyotrophic lateral sclerosis and other two patients were spinal progressive muscular atrophy (SPMA) in autopsy. No malignancy was detected in all cases. These results corroborate the concept of a probable association between MND and benign monoclonal gammopathy (plasma cell dyscrasia). A patient with SPMA had transient elevation of IgG-GM1 antibodies. Other five patients with M-proteinemia did not show elevated serum anti GM1 antibodies.
在一些患者中，运动神经元病（MND）与副蛋白相关，这引发了MND与单克隆丙种球蛋白病之间可能存在关联的问题。我们使用醋酸纤维素膜免疫固定法分析了53例MND患者血清中是否存在单克隆免疫球蛋白。MND患者中有6例（11.3%）出现副蛋白，这一发生率超过了在已报道人群中使用醋酸纤维素膜检测到的值（低于1%），即便考虑了年龄因素也是如此。单克隆成分有IgG（33%）、IgM（33%）和IgA（33%）。在6例患者中，4例表现出肌萎缩侧索硬化的典型变化，另外2例患者在尸检时被诊断为脊髓性进行性肌萎缩（SPMA）。所有病例均未检测到恶性肿瘤。这些结果证实了MND与良性单克隆丙种球蛋白病（浆细胞发育异常）之间可能存在关联这一概念。1例SPMA患者的IgG - GM1抗体出现短暂升高。其他5例有M蛋白血症的患者血清抗GM1抗体未升高。
