Sharma Praveen K, Balachandar Sanjaykanth, Vikram Michael Antony, Sukumar Pujitha Duvooru
Department of Radio-Diagnosis, Saveetha Medical College and Hospital, Saveetha Nagar, Thandalam, Chennai, Tamil Nadu 602105, India.
Radiol Case Rep. 2024 Dec 24;20(3):1538-1547. doi: 10.1016/j.radcr.2024.11.070. eCollection 2025 Mar.
Posterior reversible encephalopathy syndrome (PRES) is an uncommon neurological condition characterized by reversible subcortical vasogenic edema that primarily affects the posterior areas of the brain. Subcortical vasogenic edema resulting from endothelial injury and hypertension is the pathogenesis. Here, we present a 23-year-old female patient with systemic lupus erythematosus (SLE) and lupus nephritis who developed PRES following Rituximab (a monoclonal anti-CD-20 antibody) administration. The patient initially presented with severe headaches, visual disturbances, and an altered mental status. Neurological examination revealed bilateral cortical blindness, hyperreflexia, and seizures. Brain imaging, including MRI, demonstrated characteristic findings of PRES, with symmetric hyperintensities involving the occipital and parietal lobes on T2-weighted and FLAIR sequences, consistent with vasogenic edema. Rituximab is promptly discontinued, and the patient was managed with supportive care, including antiepileptic drugs and blood pressure control. Within days of Rituximab cessation, the patient showed gradual improvement in symptoms, with resolution of cortical blindness and normalization of MRI findings. Follow-up assessments revealed complete neurological recovery without residual deficits. This instance emphasizes how crucial it is to take into account PRES as a possible side effect in patients receiving Rituximab therapy, especially if those individuals have sudden neurological symptoms. The offending agent must be located and eliminated immediately for the best outcomes. Clinicians should maintain a high index of suspicion for PRES in patients receiving monoclonal anti-CD20 antibody therapies, immunosuppressants, and corticosteroids, facilitating timely diagnosis and intervention to prevent potentially life-threatening complications. More studies are necessary to clarify the pathophysiological mechanisms causing the PRES produced by Rituximab and to improve therapeutic approaches.
后部可逆性脑病综合征(PRES)是一种罕见的神经系统疾病,其特征为可逆性皮质下血管源性水肿,主要影响大脑后部区域。由内皮损伤和高血压导致的皮质下血管源性水肿是其发病机制。在此,我们报告一名23岁患有系统性红斑狼疮(SLE)和狼疮性肾炎的女性患者,在使用利妥昔单抗(一种单克隆抗CD - 20抗体)后发生了PRES。患者最初表现为严重头痛、视觉障碍和精神状态改变。神经系统检查发现双侧皮质盲、反射亢进和癫痫发作。包括MRI在内的脑部影像学检查显示了PRES的特征性表现,在T2加权和液体衰减反转恢复(FLAIR)序列上,枕叶和顶叶出现对称的高信号,符合血管源性水肿。立即停用利妥昔单抗,并对患者进行支持性治疗,包括使用抗癫痫药物和控制血压。在停用利妥昔单抗后的几天内,患者症状逐渐改善,皮质盲消失,MRI表现恢复正常。随访评估显示神经功能完全恢复,无残留缺陷。该病例强调了在接受利妥昔单抗治疗的患者中,尤其是那些突然出现神经症状的患者,将PRES视为可能的副作用是多么重要。必须立即找出并消除致病因素以获得最佳治疗效果。临床医生应对接受单克隆抗CD20抗体治疗、免疫抑制剂和皮质类固醇治疗的患者保持高度的PRES怀疑指数,以便及时诊断和干预,预防潜在的危及生命的并发症。需要更多的研究来阐明利妥昔单抗导致PRES的病理生理机制,并改进治疗方法。