Raphaël J C, Sharshar T
Service de Réanimation Médicale, Hôpital Raymond-Poincaré, 92380 Garches.
Ann Med Interne (Paris). 2000 May;151 Suppl 1:1S35-40.
The average annual incidence of Guillain-Barré syndrome is 1.5 per 100 000. Mortality was about 5% in a recent clinical trial. Ten percent of patients have severe neurological sequelae one year after onset. For these patients, general care is essential and should be provided in appropriate hospital units. Corticosteroids, administered orally or intravenously are ineffective. Plasma exchange (PE) was the first treatment to demonstrate efficacy in randomized clinical trials. Indications have been recently specified. Patients who can walk must be given two PEs and two additional PEs in case of aggravation. Four plasma exchanges are sufficient in patients unable to walk unaided (intermediate form) or who are mechanically ventilated (severe form). No further PE is required if the patient fails to improve. High-dose intravenous immunoglobulins (0.4 g /kg daily for 5 days) and PE have equivalent efficacy in intermediate and severe forms. The optimal dose of IVIg and the number of Pes in the different severity forms are being assessed in an ongoing study.
吉兰 - 巴雷综合征的年平均发病率为每10万人中1.5例。在最近的一项临床试验中，死亡率约为5%。10%的患者在发病一年后有严重的神经后遗症。对于这些患者，一般护理至关重要，应在合适的医院科室提供。口服或静脉注射皮质类固醇均无效。血浆置换（PE）是在随机临床试验中首个证明有效的治疗方法。最近已明确了其适应证。能行走的患者必须进行两次血浆置换，病情加重时再增加两次。对于无法独立行走（中度形式）或需要机械通气（重度形式）的患者，四次血浆置换就足够了。如果患者没有改善，则无需进一步进行血浆置换。高剂量静脉注射免疫球蛋白（每日0.4 g/kg，共5天）与血浆置换在中度和重度形式中疗效相当。一项正在进行的研究正在评估不同严重程度形式下静脉注射免疫球蛋白的最佳剂量和血浆置换的次数。
