Willison H J, Chancellor A M, Paterson G, Veitch J, Singh S, Whitelaw J, Kennedy P G, Warlow C P
University Department of Neurology, Southern General Hospital, Glasgow, UK.
J Neurol Sci. 1993 Feb;114(2):209-15. doi: 10.1016/0022-510x(93)90300-n.
The role of humoral autoimmune factors in the pathogenesis of motor neuron disease (MND) is currently under considerable scrutiny. In particular, there have been many reports of abnormal serum immunoglobulin patterns and elevated titres of anti-ganglioside antibodies in patients with MND. However, many of these studies may be biased by the selection criteria for patients and controls. In order to carefully address this issue we obtained 82 blood samples from consecutive MND patients identified through a national MND register in combination with 82 community controls matched for age, sex and geographical area. We used these samples to determine the frequency of monoclonal immunoglobulins (mIgs) and measure the levels of serum immunoglobulins and anti-GM1 ganglioside antibodies in sporadic cases of MND in comparison with normal controls. Serum mIgs detected using high resolution and immunofixation agarose electrophoresis were present in 1.2% of MND patients and 2.4% of controls. Using a highly sensitive isoelectric focusing and immunoblotting method, monoclonal or oligoclonal immunoglobulins were found in 28% of MND patients and 27% of controls. Anti-GM1 antibodies were present in 26% of MND patients and 18% of controls (odds ratio = 1.5, 95%, CI 0.7-3.6) with no significant differences in titres between the 2 groups. Mean immunoglobulin G, A and M levels were equal in 2 groups. Thus, although alterations in these parameters were identified, we were unable to demonstrate any significant difference between MND patients and controls. We conclude that the majority of sporadic cases of MND are unlikely to have an autoimmune basis as judged by the lack of abnormalities in these parameters.
体液自身免疫因素在运动神经元病(MND)发病机制中的作用目前受到广泛审视。特别是,有许多关于MND患者血清免疫球蛋白模式异常和抗神经节苷脂抗体滴度升高的报道。然而,这些研究中的许多可能因患者和对照的选择标准而存在偏差。为了谨慎地解决这个问题,我们从通过全国MND登记册确定的连续MND患者中获取了82份血样,并与82名年龄、性别和地理区域匹配的社区对照进行了比较。我们使用这些样本确定单克隆免疫球蛋白(mIgs)的频率,并测量MND散发病例与正常对照相比的血清免疫球蛋白和抗GM1神经节苷脂抗体水平。使用高分辨率和免疫固定琼脂糖电泳检测到的血清mIgs在1.2%的MND患者和2.4%的对照中存在。使用高灵敏度的等电聚焦和免疫印迹方法,在28%的MND患者和27%的对照中发现了单克隆或寡克隆免疫球蛋白。26%的MND患者和18%的对照中存在抗GM1抗体(优势比 = 1.5,95%,CI 0.7 - 3.6),两组之间的滴度无显著差异。两组的免疫球蛋白G、A和M平均水平相等。因此,尽管确定了这些参数的改变,但我们未能证明MND患者与对照之间存在任何显著差异。我们得出结论,根据这些参数缺乏异常判断,大多数MND散发病例不太可能有自身免疫基础。