12例可逆性后部白质脑病综合征的临床特点

Clinical features of 12 patients with reversible posterior leukoencephalopathy syndrome

目的探讨可逆性后部白质脑病综合征(RPLS)的临床特点。方法回顾性分析2015-06—2016-09于河南省人民医院诊治的12例可逆性后部白质脑病综合征患者的临床特征、影像学特点、实验室检查并复习相关文献。结果临床特征:本组女11例,男1例。子痫10例,合并血小板减少7例;原发性高血压2例。10例出现癫痫发作,7例出现头痛,6例视物障碍。影像学特点:病灶大多为对称性,主要位于双侧大脑后部白质,少数位于额顶叶、基底节区、胼胝体、小脑及其他部位。在CT呈低密度影,MRI呈长T_1、长T_2,FLAIR呈高信号,DWI大部分呈低信号,ADC大部分呈高信号。4例复查后病灶完全恢复,呈完全可逆性。实验室检查:LDH升高11例,其中血小板减少伴白细胞升高、肌酸激酶升高7例。结论 RPLS是一种女性好发,以头痛、癫痫发作、视觉障碍、意识障碍为主要表现,影像学表现为可逆的血管源性水肿的神经系统疾病,血小板减少可能为其病因之一,经积极治疗,大部分预后良好。

Objective To analyze the clinical features of reversible posterior leukoencephalopathy syndrome（RPLS）.Methods Clinical data including the clinical presentation,laboratory finding and imaging features of twelve patients diagnosed of RPLS by People＆#39;s Hospital of Henan Province from June 2015 to September 2016 were analyzed.Results Eleven cases of female and one case of male were identified.Ten cases presented with eclampsia,seven of which combined a low level blood platelet.Seizure（10/12）,headache（7/12）and visual disturbance（6/12）were observed.Radiological imaging showed symmetrical lesions in posterior brain matter,low density on brain CT and vasogenic edema on MRI,with fronto-parietal lobe,basal ganglia region,callosum,cerebellum involved accasionally.Reexamination after treatment showed completely remission in four cases.Elevated level of LDH was detected in 11/12 cases,7 of which accompanied by reduced platelet level,elevated white blood cell and creatine kinase（CK）.Conclusion RPLS is a central nervous system disease featured by headache,seizure,blurred vision and consciousness disturbance.Brain MRI shows vasogenic edema.Lower level of platelet is a possible cause of the disease.Positive treatment is correlated with a better prognosis.