欧洲神经病学学会/外周神经学会格林-巴利综合征诊断和治疗指南。

IF 3.9 3区 医学 Q1 CLINICAL NEUROLOGY Journal of the Peripheral Nervous System Pub Date : 2023-10-10 DOI:10.1111/jns.12594
Pieter A. van Doorn, Peter Y. K. Van den Bergh, Robert D. M. Hadden, Bert Avau, Patrik Vankrunkelsven, Shahram Attarian, Patricia H. Blomkwist-Markens, David R. Cornblath, H. Stephan Goedee, Thomas Harbo, Bart C. Jacobs, Susumu Kusunoki, Helmar C. Lehmann, Richard A. Lewis, Michael P. Lunn, Eduardo Nobile-Orazio, Luis Querol, Yusuf A. Rajabally, Thirugnanam Umapathi, Haluk A. Topaloglu, Hugh J. Willison
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引用次数: 0

摘要

格林-巴利综合征(GBS)是一种急性多神经根性神经病。症状的表现和严重程度可能有很大差异。除了虚弱和感觉障碍外,患者还可能有脑神经受累、呼吸功能不全、自主神经功能障碍和疼痛。为了制定GBS诊断和治疗的循证指南,使用建议、评估、发展和评估分级(GRADE)方法,欧洲神经病学学会(EAN)和周围神经学会(PNS)的一个工作组(TF)构建了14个群体/干预/比较/结果问题(PICO),涵盖GBS的诊断、治疗和预后,指导了文献检索。数据被提取并汇总在GRADE发现总结(用于治疗PICO)或证据表(用于诊断和预后PICO)中。根据GRADE决策证据(EtD)框架编制报表。对于六个干预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。就诊断而言,主要的GP是:如果最近有腹泻或呼吸道感染史,GBS更有可能发生;脑脊液检查是有价值的,尤其是当诊断不太确定时;建议使用电诊断测试来支持诊断;抗神经节苷脂抗体检测在大多数典型运动感觉GBS患者中的临床价值有限,但当怀疑Miller Fisher综合征(MFS)时,应考虑抗GQ1b抗体检测;当怀疑自身免疫性多巴胺时,应检测淋巴结旁抗体;非典型病例应考虑MRI或超声成像;如果8年后病情继续恶化,应考虑将诊断改为急性发作的慢性炎症性脱髓鞘多神经根病变(A-CID) 发病数周后,约5%的最初诊断为GBS的患者出现这种情况。对于治疗,TF建议静脉注射免疫球蛋白(IVIg)0.4 g/kg,适用于5 天,患者在2天内 周(GPP也在2-4内 数周),如果无法独立行走,或进行血浆置换(PE)12-15 L在四到五次交换中超过1-2 周,患者在4周内 虚弱发作后数周,如果无法独立行走。TF建议在预后不良的GBS患者中不要进行第二次IVIg疗程;建议不要使用口服皮质类固醇,并强烈建议不要使用静脉注射皮质类固醇;不建议PE后立即IVIg;弱推荐加巴喷丁类、三环类抗抑郁药或卡马西平用于治疗疼痛;不建议对疲劳进行特殊治疗。为了评估个别患者的预后,TF建议使用改良的伊拉斯谟GBS结果评分(mEGOS)来评估结果,并使用改良的埃拉斯谟-GBS呼吸功能不全评分(mEG RIS)来评估需要人工通气的风险。根据PICO、现有文献和其他讨论,我们提供了流程图,以帮助做出诊断、治疗和重症监护室入院需求的临床决策。
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European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain–Barré syndrome

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.

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来源期刊
CiteScore
6.10
自引率
7.90%
发文量
45
审稿时长
>12 weeks
期刊介绍: The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders. The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies. Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials. The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.
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