以可逆性皮层盲为主要表现的可逆性后部白质脑病综合征三例临床和影像学分析  

Clinical and radiologic findings of in reversible posterior leukoencephalopathy syndrome(RPLS) presenting with reversible cortical blindness:three cases report

作　　者：武力勇[1] 贾建平[1] 车林海[1] 张倩[1] 张津[1] 

机构地区：[1]首都医科大学宣武医院神经内科,北京100053

出　　处：《脑与神经疾病杂志》2009年第4期301-304,共4页Journal of Brain and Nervous Diseases

基　　金：国家自然科学青年基金(30700241);北京市科技新星项目(2007B069)

摘　　要：目的探讨主要表现为皮层盲的可逆性后部白质脑病综合征的临床与影像学表现、早期诊断和治疗。方法回顾性分析主要表现为可逆性皮层盲的慢性肾功能衰竭患者1例、先兆子痫和子痫患者各1例的临床、腰椎穿刺和影像学资料。结果3例患者均出现急性双侧完全性皮层盲,伴有高血压、头痛,1例伴有癫痫发作。腰椎穿刺示轻度颅内压增高。头颅MRI示双侧枕叶皮层或皮层下对称性异常信号,T2WI、FLAIR为高信号,DWI为低或等信号,ADC为高信号,提示血管源性水肿。3例患者均诊断为可逆性后部白质脑病综合征,经控制血压、脱水降颅压等治疗,皮层盲在2天内均完全好转,2周后复查头MRI病灶完全消失。结论可逆性皮层盲是可逆性后部白质脑病综合征最为特征性的症状之一,是由于双侧枕叶皮层或皮层下血管源性水肿所致。Objective The precise nature of the pathogenesis of reversible cortical blindness in reversible posterior leukoencephalopathy syndrome （RPLS） has not previously been understood. This report has important implications concerning the clinical and radiologic features, the early diagnosis and treatment of reversible cortical blindness in RPLS. Methods One patient with chronic renal failure and two patients with eclampsia or preclampsia presented with acute bilateral total blindness, hypertension, headache, and seizures. Magnetic resonance imaging （MRI） and lumbar puncture were performed to make diagnosis. Results Three patients were all diagnosed with RPLS. In all these patients, it is characterized predominately by bilateral symmetric cortical or subcortical vasogenic edema of the occipital lobes on MRI, accompanied by high intracranial pressure demonstrated by lmnbar puncture. After antihypertens!ve and dehydrating treatment, the vision in these three patients was restored completely in two days, and follow-up MRI within 2 weeks showed complete resolution of the lesions. Conclusion Reversible cortical blindness, which is most likely due to bilateral symmetric cortical or subcortical vasogenic edema of the occipital lobes, is one of characteristic symptoms of RPLS.

关 键 词：皮层盲 可逆性后部白质脑病综合征 枕叶 血管源性水肿 

分 类 号：R742[医药卫生—神经病学与精神病学]