Gong Yu
Department of Intensive Care Unit, Fudan University Affiliated Zhongshan Hospital, Shanghai 200032, China.
World J Hepatol. 2024 Sep 27;16(9):1297-1307. doi: 10.4254/wjh.v16.i9.1297.
Posterior reversible encephalopathy syndrome (PRES), characterized by acute neurological deterioration and extensive white matter lesions on T2-fluid attenuated inversion recovery magnetic resonance imaging (MRI), is increasingly associated with calcineurin inhibitors (CNI)-related neurotoxicity. Prompt diagnosis is crucial, as early intervention, including the modification or discontinuation of CNI therapy, strict blood pressure management, corticosteroid treatment, and supportive care can significantly improve patient outcomes and prognosis. The growing clinical recognition of CNI-related PRES underscores the importance of identifying and managing this condition in patients presenting with acute neurological symptoms.
This report describes three cases of liver transplant recipients who developed PRES. The first case involves a 60-year-old woman who experienced seizures, aphasia, and hemiplegia on postoperative day (POD) 9, with MRI revealing ischemic foci followed by extensive white matter lesions. After replacing tacrolimus, her symptoms improved, and no significant MRI abnormalities were observed after three years of follow-up. The second case concerns a 54-year-old woman with autoimmune hepatitis who developed headaches, seizures, and extensive white matter demyelination on MRI on POD24. Following the switch to rapamycin and the initiation of corticosteroids, her symptoms resolved, and she was discharged on POD95. The third case details a 60-year-old woman with hepatocellular carcinoma who developed PRES, evidenced by brain MRI abnormalities on POD11. Transitioning to rapamycin and corticosteroid therapy led to her full recovery, and she was discharged on POD22. These cases highlight the critical importance of early diagnosis, CNI modification, and stringent management in improving outcomes for liver transplant recipients with CNI-related PRES.
Clinical manifestations, combined with characteristic MRI findings, are crucial in diagnosing PRES among organ transplant recipients. However, when standard treatments are ineffective or MRI results are atypical, alternative diagnoses should be taken into considered.
后部可逆性脑病综合征(PRES)以急性神经功能恶化和T2加权液体衰减反转恢复序列磁共振成像(MRI)上广泛的白质病变为特征,越来越多地与钙调神经磷酸酶抑制剂(CNI)相关的神经毒性有关。及时诊断至关重要,因为早期干预,包括调整或停用CNI治疗、严格控制血压、使用皮质类固醇治疗和支持性护理,可显著改善患者的结局和预后。临床上对CNI相关PRES的认识不断增加,凸显了在出现急性神经症状的患者中识别和管理这种疾病的重要性。
本报告描述了3例肝移植受者发生PRES的病例。第一例为一名60岁女性,术后第9天出现癫痫发作、失语和偏瘫,MRI显示缺血灶,随后出现广泛的白质病变。更换他克莫司后,她的症状有所改善,随访三年后MRI未观察到明显异常。第二例是一名54岁的自身免疫性肝炎女性,术后第24天出现头痛、癫痫发作,MRI显示广泛的白质脱髓鞘。改用雷帕霉素并开始使用皮质类固醇后,她的症状消失,术后第95天出院。第三例详细介绍了一名60岁的肝细胞癌女性,术后第11天脑MRI异常,证实发生了PRES。改用雷帕霉素和皮质类固醇治疗后她完全康复,术后第22天出院。这些病例凸显了早期诊断、调整CNI以及严格管理对于改善CNI相关PRES肝移植受者结局的至关重要性。
临床表现结合特征性MRI表现对于诊断器官移植受者的PRES至关重要。然而,当标准治疗无效或MRI结果不典型时,应考虑其他诊断。