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Guillain–Barré syndrome: pathogenesis, diagnosis, treatment and prognosis

医学 格林-巴利综合征 急性运动性轴索神经病 发病机制 弱点 疾病 慢性炎症性脱髓鞘性多发性神经病 血浆置换术 多神经根神经病 分子模拟 重症监护医学 免疫学 内科学 抗体 外科
作者
Bianca van den Berg,Christa Walgaard,Judith Drenthen,C. Fokke,Bart C. Jacobs,Pieter A. van Doorn
出处
期刊:Nature Reviews Neurology [Nature Portfolio]
卷期号:10 (8): 469-482 被引量:1077
标识
DOI:10.1038/nrneurol.2014.121
摘要

Guillain–Barré syndrome (GBS) is a postinfectious disease characterized by rapidly progressive, symmetrical weakness of the extremities, and can lead to life-threatening complications such as respiratory insufficiency and autonomic dysfunction. Here, van den Berg et al. describe the immune pathogenesis and clinical characteristics of GBS and its subtypes, and highlight the importance of careful diagnostic assessment of patients and the possible additional diagnostic value of cerebrospinal fluid examinations and nerve conduction tests. The authors review treatment options and prognosis, including novel predictive models, for patients with GBS. Guillain–Barré syndrome (GBS) is a potentially life-threatening postinfectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. About 25% of patients develop respiratory insufficiency and many show signs of autonomic dysfunction. Diagnosis can usually be made on clinical grounds, but lumbar puncture and electrophysiological studies can help to substantiate the diagnosis and to differentiate demyelinating from axonal subtypes of GBS. Molecular mimicry of pathogen-borne antigens, leading to generation of crossreactive antibodies that also target gangliosides, is part of the pathogenesis of GBS; the subtype and severity of the syndrome are partly determined by the nature of the antecedent infection and specificity of such antibodies. Intravenous immunoglobulin and plasma exchange are proven effective treatments but many patients have considerable residual deficits. Discrimination of patients with treatment-related fluctuations from those with acute-onset chronic inflammatory demyelinating polyneuropathy is important, as these conditions may require different treatments. Novel prognostic models can accurately predict outcome and the need for artificial ventilation, which could aid the selection of patients with a poor prognosis for more-individualized care. This Review summarizes the clinical features of and diagnostic criteria for GBS, and discusses its pathogenesis, treatment and prognosis.
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