Guillain–Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/ Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal–paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2–4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12–15 L in four to five exchanges over 1–2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.

格林巴利综合征 (GBS) 是一种急性多发性神经根神经病。症状的表现和严重程度可能差异很大。除无力和感觉障碍外，患者还可能有颅神经受累、呼吸功能不全、自主神经功能障碍和疼痛。为制定 GBS 诊断和治疗的循证指南，采用推荐、评估、发展和评价分级 (GRADE) 方法欧洲神经病学学会 (EAN) 和周围神经学会 (PNS) 的工作组 (TF) 构建了14个覆盖 GBS 诊断、治疗和预后的人群/干预/比较/结局问题 (PICOs)，指导文献检索。提取数据并总结在结果等级总结（对于治疗PICO）或证据表（对于诊断和预后PICO）中。根据循证决策等级 (EtD) 框架编写了声明。对于6个干预PICO，提出了基于证据的建议。对于其他PICO，制定了良好实践要点 (GPP)。对于诊断，主要的 GPPs 是：如果有近期腹泻或呼吸道感染史，GBS的可能性更大；CSF检查有价值，特别是当诊断不太确定时；建议进行电诊断检测以支持诊断；抗神经节苷脂抗体检测在大多数典型运动感觉型 GBS 患者中的临床价值有限，但当怀疑 Miller Fisher 综合征 (MFS) 时，应考虑进行抗 GQ1b 抗体检测；当怀疑自身免疫性结节病时，应检测淋巴结-结旁抗体；在不典型病例中应考虑 MRI 或超声成像；如果在发病后8周后继续进展，应考虑将诊断改为急性发作的慢性炎性脱髓鞘性多发性神经根神经病 (A-CIDP)，这在最初诊断为 GBS 的患者中的发生率约为5%。对于治疗，TF建议无力发作后2周内（GPP也在2-4周内）的患者在无辅助行走时静脉注射免疫球蛋白 (IVIg)0.4 g/kg，持续5天，或无力发作后4周内无辅助行走患者在1-2周内4-5次交换一个疗程的血浆置换 (PE)12-15 L。TF 建议对预后不良的 GBS 患者不要进行第2个疗程的 IVIg 治疗；建议不要使用口服皮质类固醇，弱推荐不要使用 IV 皮质类固醇；不推荐 PE 后立即使用IVIg；弱推荐使用加巴喷丁类、三环类抗抑郁药或卡马西平治疗疼痛；不推荐疲乏的特异性治疗。为了估计个体患者的预后，TF建议使用改良 Erasmus GBS 结局评分 (mEGOS) 评估结局，使用改良 Erasmus GBS 呼吸功能不全评分 (mEGRIS) 评估需要人工通气的风险。基于PICO、现有文献和额外讨论，我们提供了流程图，以帮助做出诊断、治疗和入住重症监护室需求的临床决策。