Buchwald B, Toyka K V, Zielasek J, Weishaupt A, Schweiger S, Dudel J
Neurologische Klinik der Bayerischen Julius-Maximilians-Universität, Würzburg, Germany.
Ann Neurol. 1998 Dec;44(6):913-22. doi: 10.1002/ana.410440610.
Guillain-Barré syndrome (GBS) is often associated with serum antibodies to glycoconjugates such as GM1 and GQ1b. The pathogenic role of these antibodies and other serum factors has not yet been clarified. We have investigated the effect of serum, plasma filtrate, and highly purified IgG and IgM from 10 patients with typical GBS on motor nerve terminals in the mouse hemidiaphragm. Quantal endplate currents were recorded by means of a perfused macro-patch-clamp electrode. The plasma filtrate of all GBS patients led to a 5- to 20-fold reduction of evoked quantal release within 7 to 15 minutes of continuous superfusion. In 4 patients, the amplitudes of single quanta were clearly reduced (by 10-66% of control values), indicating an additional postsynaptic action. Blocking effects could be reversed to a variable degree within 15 to 18 minutes after washout. Purified IgG was as effective as native serum, whereas a purified GBS IgM fraction did not block transmission. Sera from convalescent patients and IgG from healthy subjects were without blocking effect. The effects were complement independent and there was no link to the presence (in 6 patients) or absence (in 4 patients) of detectable antibodies to GM1 or GQ1b. In GBS, antibodies to an undetermined antigen depress the presynaptic transmitter release and, in some cases, the activation of postsynaptic channels. We suggest that weakness in the acute stage of GBS may be caused in part by circulating antibodies.
吉兰-巴雷综合征（GBS）常与针对糖缀合物（如GM1和GQ1b）的血清抗体相关。这些抗体及其他血清因子的致病作用尚未明确。我们研究了10例典型GBS患者的血清、血浆滤液以及高度纯化的IgG和IgM对小鼠半膈肌运动神经末梢的影响。通过灌注式宏观膜片钳电极记录量子化终板电流。所有GBS患者的血浆滤液在持续灌注7至15分钟内可使诱发的量子释放减少5至20倍。在4例患者中，单个量子的幅度明显降低（降至对照值的10% - 66%），表明存在额外的突触后作用。洗脱后15至18分钟内，阻断作用可不同程度地逆转。纯化的IgG与天然血清效果相同，而纯化的GBS IgM组分不阻断传递。恢复期患者的血清和健康受试者的IgG无阻断作用。这些作用不依赖补体，且与是否存在可检测到的抗GM1或GQ1b抗体（6例患者存在，4例患者不存在）无关。在GBS中，针对未确定抗原的抗体可抑制突触前递质释放，在某些情况下还可抑制突触后通道的激活。我们认为GBS急性期的肌无力可能部分由循环抗体引起。
