Leys K, Lang B, Johnston I, Newsom-Davis J
Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, UK.
Ann Neurol. 1991 Mar;29(3):307-14. doi: 10.1002/ana.410290313.
We have tested 36 patients with the Lambert-Eaton myasthenic syndrome for serum antibodies to voltage-gated calcium channels by using an immunoprecipitation assay with [125I] omega-conotoxin-labeled voltage-gated calcium channels extracted from a human neuroblastoma cell line, SKN-SH. Forty-four percent of these patients had significant levels of antibody (30-1,466 pM) compared with healthy control individuals (less than 15 pM). The incidence of positive sera in patients without associated small cell lung carcinoma (61%) was greater than in those patients with small cell lung carcinoma (28%). Results correlated strongly with results obtained using voltage-gated calcium channels extracted from the small cell lung carcinoma line, MAR5. Anti-voltage-gated calcium channel antibody titers did not correlate with disease severity across individuals, but longitudinal studies in 2 patients receiving immunosuppressive therapy showed a clear inverse relation between antibody titer and an electromyographic index of disease severity. The incidence of positive sera among patients with other neurological disorders was not significant, but 8 of 12 patients with rheumatoid arthritis or systemic lupus erythematosus had raised titers (30-82 pM). We conclude that the antibodies detected in this assay are heterogeneous and that some of them are likely to be implicated in this disorder of neuromuscular transmission. The assay should prove useful as an additional diagnostic aid in patients with Lambert-Eaton myasthenic syndrome.
我们采用免疫沉淀试验，利用从人神经母细胞瘤细胞系SKN-SH中提取的经[125I]ω-芋螺毒素标记的电压门控钙通道，对36例兰伯特-伊顿肌无力综合征患者检测了血清中针对电压门控钙通道的抗体。与健康对照个体（低于15 pM）相比，这些患者中有44%具有显著水平的抗体（30 - 1466 pM）。无相关小细胞肺癌的患者中阳性血清的发生率（61%）高于有小细胞肺癌的患者（28%）。结果与使用从小细胞肺癌细胞系MAR5中提取的电压门控钙通道所获得的结果密切相关。抗电压门控钙通道抗体滴度在个体间与疾病严重程度无关，但对2例接受免疫抑制治疗的患者进行的纵向研究显示，抗体滴度与疾病严重程度的肌电图指标之间存在明显的负相关。其他神经系统疾病患者中阳性血清的发生率不显著，但12例类风湿关节炎或系统性红斑狼疮患者中有8例滴度升高（30 - 82 pM）。我们得出结论，该检测中检测到的抗体具有异质性，其中一些可能与这种神经肌肉传递障碍有关。该检测作为兰伯特-伊顿肌无力综合征患者的一种辅助诊断方法应是有用的。
