Data_Sheet_1_Different Clinicoradiological Characteristics of Posterior Reversible Encephalopathy Syndrome in Pediatric Oncology and Post-Bone Marrow Transplantation Cases: A Retrospective Study.PDF

Posterior reversible encephalopathy syndrome (PRES) is receiving increasing recognition in pediatrics. However, comparisons between PRES in pediatric oncology and post-bone marrow transplantation (BMT) are lacking. Therefore, we aimed to describe the risk factors and clinical and radiological features of PRES and investigate the differences between PRES in pediatric oncology and post-BMT. The PRES data of 13 patients from our center were combined with those of 217 cases from the PubMed, Scopus, and Web of Science databases. The patients were divided into either an oncology or a post-BMT group. We included 230 patients in the analysis, 26.1% of whom belonged to the post-BMT group. Oncology patients developed PRES at a younger age (p = 0.010) and were more likely to develop encephalopathy (p = 0.004). Systemic hypertension (S-HTN) preceding PRES occurred in 43.5% (66/154) of patients. Post-BMT patients were more likely to have S-HTN (p = 0.003). Cyclosporine levels were detected in 37 patients; 40.5% had supra-therapeutic levels. The radiological findings were atypical in 74.3% of patients, and delayed repeated imaging increased the occurrence of resolution (p = 0.004). Sixteen (7%) patients developed PRES recurrence after a median of 8 weeks, with the between-group difference being non-significant. Oncology patients were more likely to develop chronic epilepsy, while BMT patients were more likely to develop rare neurologic abnormalities (p < 0.001). In conclusion, atypical clinical presentation and imaging findings should not hinder the diagnosis of PRES. S-HTN is a risk factor, particularly in post-BMT patients. Supra-therapeutic levels of cyclosporine and previous exposure to immunosuppression did not increase the risk of recurrence.

后部可逆性脑病综合征（Posterior reversible encephalopathy syndrome, PRES）在儿科临床中的受重视程度日益提升。然而，目前针对儿科肿瘤患者与骨髓移植（bone marrow transplantation, BMT）后并发PRES的对比研究仍较为匮乏。为此，本研究旨在阐明PRES的危险因素、临床及影像学特征，并探讨儿科肿瘤相关PRES与BMT后PRES的差异。本研究将本中心13例PRES患者的临床资料，与PubMed、Scopus及Web of Science数据库中收录的217例PRES病例数据进行合并。按照分组规则将患者分为肿瘤组与BMT后组，最终纳入分析的患者共计230例，其中26.1%属于BMT后组。肿瘤组患者确诊PRES时的年龄更小（p=0.010），且更易出现脑病相关症状（p=0.004）。PRES发病前合并系统性高血压（Systemic hypertension, S-HTN）的患者占比为43.5%（66/154）。BMT后组患者出现系统性高血压的比例显著更高（p=0.003）。本研究对37例患者的环孢素（cyclosporine）血药浓度进行了检测，其中40.5%的患者血药浓度超出治疗窗范围。74.3%的患者影像学表现不典型，延迟复查影像学可提升病灶消退的检出率（p=0.004）。16例（7%）患者在中位随访8周后出现PRES复发，两组间复发率差异无统计学意义。肿瘤组患者更易并发慢性癫痫，而BMT后组患者则更易出现罕见神经系统异常表现（p<0.001）。综上，不典型的临床表现与影像学表现不应作为排除PRES诊断的依据。系统性高血压是PRES的危险因素，尤其对于BMT后患者。环孢素血药浓度超出治疗窗范围及既往免疫抑制治疗暴露史，均不会增加PRES的复发风险。