Neonatal Guillain-Barré syndrome
Neonatal Guillain-Barré syndrome
复制标题
新生儿吉兰-巴利综合征
DOI：
10.1212/wnl.53.6.1246
复制
发表时间：
1999
期刊：
影响因子：
9.9
通讯作者：
K. Toyka
中科院分区：
文献类型：
--
作者：
B. Buchwald;M. D. de Baets;G. Luijckx;K. Toyka
目的：探讨阻断抗体在患有持续性 GBS 的母亲产下的产后 12 天发生的新生儿吉兰-巴利综合征 (GBS) 中的作用。方法：我们研究了患病母亲的血浆滤液、纯化的 IgG 和单价 Fab 片段、新生儿的血清以及疾病恢复 3 个月后的血清样本。在成年小鼠以及新生和幼年大鼠的半膈上进行了实验。用灌注宏膜片钳电极记录量子终板电流。结果：观察到双重效果。母亲和婴儿的血清使量子含量降低约 90%，并使突触后电流幅度降低 30% 至 40%。阻断的抗体性质可以通过显示单价 Fab 片段与纯化的免疫球蛋白 (Ig) G 具有类似的效果来证实。没有检测到针对神经节苷脂、胎儿或成人烟碱乙酰胆碱受体或电压门控钙通道的 IgG 抗体，但存在针对神经节苷脂 GM1 的 IgM 抗体。 GBS 恢复后，母亲和婴儿的血清中未观察到阻断活性。为了阐明新生儿疾病发作延迟的原因，我们研究了早期发育变化对神经肌肉接头功能特性的可能影响，并将母亲的活性血清应用于出生后的大鼠。尽管在 23 日龄的大鼠中存在阻滞，但在 5 日龄的大鼠中则不存在。结论：经胎盘转移的阻断抗体可能特异性针对成熟的表位，而不是胎儿的神经肌肉接头。
Objective: To investigate the role of blocking antibodies in neonatal Guillain-Barré syndrome (GBS) occurring 12 days postpartum in a child born to a mother with ongoing GBS. Methods: We studied plasma filtrate, purified IgG, and monovalent Fab fragments from the affected mother and serum from the neonate as well as serum samples after recovery from disease 3 months later. Experiments were performed on the hemidiaphragms of adult mice and neonatal and juvenile rats. Quantal endplate currents were recorded with the perfused macro-patch clamp electrode. Results: A dual effect was seen. Serum from mother and infant depressed quantal content by approximately 90% and reduced the amplitude of postsynaptic currents by 30 to 40%. The antibody nature of the blockade could be confirmed by showing that monovalent Fab fragments were similarly effective as purified immunoglobulin (Ig) G. No IgG antibodies to gangliosides, fetal or adult nicotinic acetylcholine receptor, or voltage-gated calcium channels could be detected, but IgM antibodies to the ganglioside GM1 were present. After recovery from GBS no blocking activity was seen in the sera of mother and infant. To elucidate why neonatal disease onset was delayed we examined the possible influence of early developmental changes in functional properties of the neuromuscular junction and applied the mother’s active serum to postnatal rats. Although blockade was present in 23-day-old rats, it was absent in 5-day-old rats. Conclusion: Transplacentally transferred blocking antibodies may be specifically directed at epitopes of the mature but not the fetal neuromuscular junction.