Stevens A, Weller M, Wiethölter H
Department of Neurology, University of Tübingen, Germany.
J Neurol Neurosurg Psychiatry. 1993 Apr;56(4):361-4. doi: 10.1136/jnnp.56.4.361.
Paired cerebrospinal fluid and serum samples of patients with amyotrophic lateral sclerosis (n = 35) revealed no consistent abnormalities of CSF cell count, CSF albumin, CSF IgG, CSF IgM, IgG or IgM index, or oligoclonal immunoglobulin band formation in the CSF. Determination of IgG and IgM CSF and serum antibodies to gangliosides GM1, GM2, GM3, AGM1, GD1a, GD1b, and GT1b showed a characteristic pattern which allowed the differentiation of amyotrophic lateral sclerosis from controls and from patients with other neurological disorders including multiple sclerosis. Specifically, patients with the disease had elevated CSF IgM antibodies to all gangliosides except AGM1. The lack of correlation between the CSF findings and corresponding serum antibodies suggests a chronic, compartmental, intrathecal immune response of low activity in amyotrophic lateral sclerosis. Whether this immune response is primary and of pathogenetic significance, or an epiphenomenon of neuronal degeneration, remains to be investigated.
对35例肌萎缩侧索硬化症患者的配对脑脊液和血清样本进行检测，结果显示脑脊液细胞计数、脑脊液白蛋白、脑脊液IgG、脑脊液IgM、IgG或IgM指数，以及脑脊液中寡克隆免疫球蛋白带形成均无一致异常。对脑脊液和血清中针对神经节苷脂GM1、GM2、GM3、AGM1、GD1a、GD1b和GT1b的IgG和IgM抗体进行检测，结果呈现出一种特征性模式，可用于区分肌萎缩侧索硬化症患者与对照组以及其他神经系统疾病患者（包括多发性硬化症患者）。具体而言，该病患者除针对AGM1外，针对所有神经节苷脂的脑脊液IgM抗体均升高。脑脊液检测结果与相应血清抗体之间缺乏相关性，提示肌萎缩侧索硬化症存在慢性、局部、鞘内低活性免疫反应。这种免疫反应是原发性的且具有致病意义，还是神经元变性的一种附带现象，仍有待研究。
