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吉兰-巴雷综合征

Guillain-Barré syndrome.

作者信息

Cosi V, Versino M

机构信息

Dipartimento Scienze Neurologiche, Università di Pavia, Fondazione Istituto Neurologico C. Mondino, I.R.C.C.S., Via Mondino 2, I-27100 Pavia, Italy.

出版信息

Neurol Sci. 2006 Mar;27 Suppl 1:S47-51. doi: 10.1007/s10072-006-0548-4.

Abstract

Guillain-Barré syndrome (GBS) is an autoimmune acute peripheral neuropathy. Frequently a flu-like episode or a gastroenteritis precede GBS, and the cross-reactivity between microbial and neural antigens partly explains the pathophysiology of the disease and the possible detection of antiganglioside antibodies. The weakness reaches its nadir in 2-4 weeks: the patients may be chair- or bed-bound, may need artificial ventilation and frequently experience dysautonomic dysfunction; 5-15% of the patients die and more patients are left with a disabling motor deficit and/or fatigue. Electrophysiology and cerebrospinal fluid evaluation support the diagnosis. The treatment of GBS is multidisciplinary, and both plasma exchange and high dose immunoglobulin (IVIg) are effective in reducing both the severity of the disease and the residual deficits. Finally, steroids are not effective in GBS.

摘要

格林-巴利综合征(GBS)是一种自身免疫性急性周围神经病。GBS发作前常先有类似流感的症状或肠胃炎,微生物抗原与神经抗原之间的交叉反应部分解释了该疾病的病理生理学以及抗神经节苷脂抗体的可能检测情况。肌无力在2至4周内达到高峰:患者可能需要坐轮椅或卧床,可能需要人工通气,且常伴有自主神经功能障碍;5%至15%的患者死亡,更多患者会遗留致残性运动功能障碍和/或疲劳。电生理学和脑脊液评估有助于诊断。GBS的治疗是多学科的,血浆置换和大剂量免疫球蛋白(IVIg)在减轻疾病严重程度和残留缺陷方面均有效。最后,类固醇对GBS无效。

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