Leonhard Sonja E, Papri Nowshin, Querol Luis, Rinaldi Simon, Shahrizaila Nortina, Jacobs Bart C
Department of Clinical Microbiology and Infectious Disease, Erasmus MC, Rotterdam, The Netherlands.
Laboratory of Gut-Brain Axis, Infectious Diseases Division (IDD), icddr,b, Dhaka, Bangladesh.
Nat Rev Dis Primers. 2024 Dec 19;10(1):97. doi: 10.1038/s41572-024-00580-4.
Guillain-Barré syndrome (GBS) is a rare immune-mediated polyradiculoneuropathy. Patients typically develop rapidly progressive weakness and sensory deficits that can result in complete paralysis requiring mechanical ventilation. GBS is usually a monophasic disease in which an aberrant immune response to an infection or other trigger damages the peripheral nerves. For example, in patients with preceding Campylobacter jejuni infection, molecular mimicry causes a cross-reactive antibody response to nerve gangliosides. Diagnosis is based on clinical features, supported by cerebrospinal fluid analysis and nerve conduction studies. Effective treatments include plasma exchange and intravenous immunoglobulins. However, ~20% of patients who received treatment are unable to walk after 6 months and ~5% die as a consequence of GBS. Important knowledge gaps in GBS include its pathogenesis, especially after viral infections. In addition, there is a lack of specific biomarkers to improve the diagnosis, monitor the disease activity, and predict the clinical course and outcome of GBS. Major challenges for the future include finding more effective and personalized treatments, which are affordable in low-income and middle-income countries, and preparation for outbreaks of infections as potential triggers for GBS.
吉兰-巴雷综合征(GBS)是一种罕见的免疫介导性多发性神经根神经病。患者通常会迅速出现进行性肌无力和感觉障碍,可导致完全瘫痪,需要机械通气。GBS通常是单相疾病,其中对感染或其他触发因素的异常免疫反应会损害周围神经。例如,在先前有空肠弯曲菌感染的患者中,分子模拟会导致对神经节苷脂的交叉反应性抗体反应。诊断基于临床特征,并得到脑脊液分析和神经传导研究的支持。有效的治疗方法包括血浆置换和静脉注射免疫球蛋白。然而,接受治疗的患者中约20%在6个月后仍无法行走,约5%因GBS死亡。GBS的重要知识空白包括其发病机制,尤其是病毒感染后的发病机制。此外,缺乏特异性生物标志物来改善GBS的诊断、监测疾病活动以及预测其临床病程和结局。未来的主要挑战包括找到更有效且个性化的治疗方法,这些方法在低收入和中等收入国家也能负担得起,以及为作为GBS潜在触发因素的感染爆发做好准备。