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吉兰-巴雷综合征的新概念。

New concepts of Guillain-Barré syndrome.

作者信息

Asbury A K

机构信息

Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, USA.

出版信息

J Child Neurol. 2000 Mar;15(3):183-91. doi: 10.1177/088307380001500308.

Abstract

Guillain-Barré syndrome is an acute autoimmune polyradiculoneuropathy with a clinical presentation of flaccid paralysis with areflexia, variable sensory disturbance, and elevated cerebrospinal fluid protein without pleocytosis. Although Guillain-Barré syndrome previously had been viewed as a unitary disorder with variations, it currently is viewed as a group of syndromes with several distinctive subtypes. These include the principal subtype prevalent in the Western world (acute inflammatory demyelinating polyradiculoneuropathy, and others, each with distinctive electrodiagnostic and pathologic features, including acute motor axonal neuropathy), acute motor-sensory axonal neuropathy, Miller Fisher syndrome, and perhaps others. The clinical and pathologic features of these Guillain-Barré syndrome subtypes are reviewed, and the role of antecedent infections, particularly Campylobacter jejuni gastroenteritis, and the role of antiganglioside antibody responses are reviewed with respect to pathogenesis. Treatment of Guillain-Barré syndrome includes both important supportive measures and immunotherapies, specifically high-dose intravenous immunoglobulin and plasma exchange.

摘要

吉兰-巴雷综合征是一种急性自身免疫性多发性神经根神经病,临床表现为弛缓性麻痹伴反射消失、感觉障碍程度不一,以及脑脊液蛋白升高而无细胞增多。尽管吉兰-巴雷综合征以前被视为一种单一疾病,只是存在一些变异情况,但目前它被看作是一组具有几种独特亚型的综合征。这些亚型包括在西方世界流行的主要亚型(急性炎症性脱髓鞘性多发性神经根神经病,以及其他各有独特电诊断和病理特征的亚型,包括急性运动轴索性神经病)、急性运动感觉轴索性神经病、米勒-费雪综合征,或许还有其他亚型。本文综述了这些吉兰-巴雷综合征亚型的临床和病理特征,并就发病机制探讨了前驱感染(尤其是空肠弯曲菌性胃肠炎)的作用以及抗神经节苷脂抗体反应的作用。吉兰-巴雷综合征的治疗包括重要的支持措施和免疫疗法,特别是大剂量静脉注射免疫球蛋白和血浆置换。

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