可逆性后部白质脑病综合征临床及影像学特点分析（附六例报道）

Clinical and imaging features of reversible posterior leukoenphalopathy syndrome： a report of 6 cases

目的探讨可逆性后部白质脑病综合征的临床及影像学特点。方法收集无锡市人民医院神经内科自2007年12月至2010年8月诊治的6例可逆性后部白质脑病综合征患者的临床资料，回顾性分析其发病原因、临床症状、影像学特点、治疗及预后。结果6例患者中自血病1例，肾病综合征1例，肾移植l例，嗜铬细胞瘤1例，原发性高血压2例。患者临床表现多样．主要为痫性发作、头晕头痛、恶心呕吐、意识障碍、精神行为异常、视觉障碍等。头颅CT检查示4例脑白质低密度病灶，2例正常。头颅MR／检查主要表现为大脑半球后部白质病变，T1WI呈低或等信号，T2WI呈高信号，液体衰减反转恢复序列（FLAIR）呈高信号，弥散加权成像（DWI）呈低信号或等信号，表观弥散系数图（ADC）呈高信号。5例经对因及对症治疗临床症状迅速改善．影像学表现减轻或恢复正常；1例肾移植患者因拒绝停用环孢霉素A及骁悉而最终死亡。结论可逆性后部白质脑病综合征是一组依靠病史、临床特征及影像学特点而诊断的疾病，经积极治疗大多预后较好．但也有小部分患者由于种种原因而预后不良。

Objective To explore the clinical and imaging features of reversible posterior leukoencephalopathy syndrome （RPLS）. Methods The etiology, clinical manifestations, imaging features, treatment and prognosis of 6 patients with RPLS, admitted to our hospital from December 2007 to August 2010, were retrospectively analyzed. Results Among the 6 patients with RPLS, 1 was secondary to leukemia, 1 secondary to nephrotic syndrome, 1 secondary to renal transplantation, 1 secondary to pheochromocytoma, and 2 secondary to primary hypertention. The clinical manifestations of patients included seizure, dizziness, headache, nausea, vomit, conscious disturbance, behavioral and psychological abnormalities, and visual disorder. Cranial CT showed that 4 patients had low-density white matter lesions; cranial MRI mainly indicated posterior cerebral hemisphere white matter lesions, and the lesions showed low or iso-signal in T1WI, high signal in T2W/and FLAIR, low or iso-signal in diffusion-weighted magnetic imaging （DWI） and high signal in apparent diffusion coefficient （ADC） map. Five patients received the right treatment got rapid improvement of clinical symptoms with normal imaging examinations. One patient with renal transplantation who refused to disable cyclosporin A and mycophenolate mofetil eventually died. Conclusion The diagnosis of RPLS relys on history, clinical features and imaging characteristics. Most patients have a better prognosis, but a few patients have poor prognosis due to various reasons.