Guillain-Barre syndrome: a century of progress
Guillain-Barre syndrome: a century of progress
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DOI：
10.1038/nrneurol.2016.172
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发表时间：
2016-12-01
影响因子：
38.1
通讯作者：
Willison, Hugh J.
中科院分区：
文献类型：
--
作者：
Goodfellow, John A.;Willison, Hugh J.
1916 年，Guillain、Barre 和 Strohl 报道了两例急性弛缓性麻痹，脑脊液蛋白水平高且细胞计数正常，这一新发现确定了我们现在称为格林巴利综合征 (GBS) 的疾病。 100 年来，我们在 GBS 的临床和病理特征方面取得了巨大进展。早期临床病理学和动物研究表明GBS是一种免疫介导的脱髓鞘疾病，严重的GBS可导致继发性轴突损伤；目前20世纪80年代开发的血浆置换和静脉注射免疫球蛋白疗法就是基于这一前提。然而，随后的研究表明，原发性轴突损伤可能是潜在的疾病。空肠弯曲杆菌菌株的关联已证实抗神经节苷脂抗体具有致病性，并且轴突 GBS 涉及抗体和补体介导的 Ranvier 节点、神经肌肉接头以及其他神经元和神经胶质膜的破坏。现在，正在进行的补体抑制剂依库丽单抗的临床试验是 GBS 的第一个靶向免疫疗法。
In 1916, Guillain, Barre and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts novel findings that identified the disease we now know as Guillain Barre syndrome (GBS). 100 years on, we have made great progress with the clinical and pathological characterization of GBS. Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder, and that severe GBS could result in secondary axonal injury; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise. Subsequent work has, however, shown that primary axonal injury can be the underlying disease. The association of Campylobacter jejuni strains has led to confirmation that anti-ganglioside antibodies are pathogenic and that axonal GBS involves an antibody and complement-mediated disruption of nodes of Ranvier, neuromuscularjunctions and other neuronal and glial membranes. Now, ongoing clinical trials of the complement inhibitor eculizumab are the first targeted immunotherapy in GBS.