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格林-巴利综合征的临床特征、发病机制及治疗

Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome.

作者信息

van Doorn Pieter A, Ruts Liselotte, Jacobs Bart C

机构信息

Department of Neurology, Erasmus Medical Centre, Rotterdam, Netherlands.

出版信息

Lancet Neurol. 2008 Oct;7(10):939-50. doi: 10.1016/S1474-4422(08)70215-1.

Abstract

Guillain-Barré syndrome (GBS) is an important cause of acute neuromuscular paralysis. Molecular mimicry and a cross-reactive immune response play a crucial part in its pathogenesis, at least in those cases with a preceding Campylobacter jejuni infection and with antibodies to gangliosides. The type of preceding infection and patient-related host factors seem to determine the form and severity of the disease. Intravenous immunoglobulin (IVIg) and plasma exchange are effective treatments in GBS; mainly for practical reasons, IVIg is the preferred treatment. Whether mildly affected patients or patients with Miller Fisher syndrome also benefit from IVIg is unclear. Despite medical treatment, GBS often remains a severe disease; 3-10% of patients die and 20% are still unable to walk after 6 months. In addition, many patients have pain and fatigue that can persist for months or years. Advances in prognostic modelling have resulted in the development of a new and simple prognostic outcome scale that might also help to guide new treatment options, particularly in patients with GBS who have a poor prognosis.

摘要

吉兰-巴雷综合征(GBS)是急性神经肌肉麻痹的重要病因。分子模拟和交叉反应性免疫应答在其发病机制中起着关键作用,至少在那些有先前空肠弯曲菌感染且有抗神经节苷脂抗体的病例中如此。先前感染的类型和与患者相关的宿主因素似乎决定了疾病的形式和严重程度。静脉注射免疫球蛋白(IVIg)和血浆置换是治疗GBS的有效方法;主要出于实际原因,IVIg是首选治疗方法。病情轻度的患者或患有米勒-费希尔综合征的患者是否也能从IVIg治疗中获益尚不清楚。尽管进行了医学治疗,GBS通常仍是一种严重疾病;3%至10%的患者死亡,20%的患者在6个月后仍无法行走。此外,许多患者会出现疼痛和疲劳,这些症状可能会持续数月或数年。预后模型的进展导致了一种新的、简单的预后结果量表的开发,该量表可能也有助于指导新的治疗选择,特别是对于预后较差的GBS患者。

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