Salazar-Grueso E F, Routbort M J, Martin J, Dawson G, Roos R P
Department of Neurology, University of Chicago, IL 60637.
Ann Neurol. 1990 May;27(5):558-63. doi: 10.1002/ana.410270517.
Recent studies reported the presence of anti-ganglioside antibodies in occasional patients with motor neuron disease. We found polyclonal serum IgM anti-GM1 antibodies by an anti-GM1 enzyme-linked immunosorbent assay (ELISA) in 9 (19%) of 48 patients with motor neuron disease. A comparable frequency of IgM anti-GM1 antibodies was found in 4 (10%) of 40 sera from patients with other neurological disease. Three (17%) of 18 sera from the patients with motor neuron disease and 2 (17%) of 12 sera from patients with other neurological diseases had anti-GM1 immunostaining as shown by thin layer chromatography immunoblot. One patient with a lower motor neuron variant of motor neuron disease or motor axonopathy without multifocal conduction block had a markedly elevated polyclonal IgM anti-GM1 ELISA titer (greater than 1:64,000) with prominent immunostaining of GM1, moderate immunostaining of GM2, and weak and inconsistent immunostaining of GD1b by thin layer chromatography immunoblot. Treatment with prednisone resulted in clinical improvement despite increasing anti-GM1 antibody titers. These data indicate that patients with motor neuron disease have measurable levels of anti-ganglioside antibodies as frequently as patients with other neurological diseases. This contrasts with a small subgroup of patients with a lower motor neuron variant of motor neuron disease or motor axonopathy who have markedly elevated levels of serum anti-ganglioside antibodies and a clinical syndrome that is treatable with immunosuppression.
近期研究报道，偶见运动神经元病患者体内存在抗神经节苷脂抗体。我们采用抗GM1酶联免疫吸附测定法（ELISA），在48例运动神经元病患者中的9例（19%）检测到多克隆血清IgM抗GM1抗体。在40例其他神经疾病患者的血清中，有4例（10%）检测到类似频率的IgM抗GM1抗体。运动神经元病患者的18份血清中有3份（17%）、其他神经疾病患者的12份血清中有2份（17%）经薄层色谱免疫印迹法检测显示有抗GM1免疫染色。1例患有运动神经元病下运动神经元变异型或无多灶性传导阻滞的运动轴索性神经病患者，其多克隆IgM抗GM1 ELISA滴度显著升高（大于1:64,000），薄层色谱免疫印迹法显示GM1有明显免疫染色，GM2有中度免疫染色，GD1b有微弱且不一致的免疫染色。尽管抗GM1抗体滴度升高，但泼尼松治疗仍使临床症状改善。这些数据表明，运动神经元病患者体内抗神经节苷脂抗体水平与其他神经疾病患者一样可检测到。这与一小部分患有运动神经元病下运动神经元变异型或运动轴索性神经病的患者形成对比，这些患者血清抗神经节苷脂抗体水平显著升高，且临床综合征可用免疫抑制治疗。
