Department of Neurosciences, University of Padova, Padova, Italy.
Apheresis Unit, Blood Transfusion Service, University Hospital of Padova, Padova, Italy.
J Clin Apher. 2020 Jun;35(3):231-233. doi: 10.1002/jca.21783. Epub 2020 Apr 14.
Around half of the patients with Guillain-Barré syndrome (GBS) present autonomic dysfunction requiring admission to intensive care unit in up to a quarter of patients. Treatment of GBS consists of plasma exchange (PE) and intravenous immunoglobulins (IVIG). Posterior reversible encephalopathy syndrome (PRES) consists in a reversible subcortical vasogenic brain edema caused by endothelial damage triggered by abrupt blood pressure changes. We report on a woman who presented with PRES in the course of GBS treated first with IVIG, and then with PE. The present report underlines the challenge that the clinicians face when these two rare syndromes concur. The literature is not helpful considering that both blood pressure fluctuations and IVIG are reported to be involved in the pathogenesis of PRES. In the present letter, both pathogenic mechanisms and clinical management considerations are discussed.
约一半的吉兰-巴雷综合征(GBS)患者存在自主神经功能障碍,多达四分之一的患者需要入住重症监护病房。GBS 的治疗包括血浆置换(PE)和静脉注射免疫球蛋白(IVIG)。可逆性后部脑病综合征(PRES)是由内皮损伤引起的突发性血压变化导致的可逆性皮质下血管源性脑水肿。我们报告了一例在接受 IVIG 治疗后又接受 PE 治疗的 GBS 患者并发 PRES。本报告强调了当这两种罕见综合征同时发生时,临床医生所面临的挑战。由于文献报道血压波动和 IVIG 均与 PRES 的发病机制有关,因此文献对此并无帮助。在本信中,我们讨论了这两种发病机制和临床管理注意事项。