血管内皮细胞病的临床、影像学和病理学研究  

Clinical Features,Neurological Imaging and Pathological Changes of Endotheliopathy

作　　者：张在强[1] 崔涛[1] 董可辉[1] 曲辉[1] 王拥军[1] 曹世俭[2] 孙异临[3] 

机构地区：[1]首都医科大学附属北京天坛医院神经内科,北京市100050 [2]首都医科大学附属北京天坛医院病理科 [3]北京神经外科研究所超微形态室

出　　处：《中国卒中杂志》2007年第1期8-12,共5页Chinese Journal of Stroke

摘　　要：目的探讨血管内皮细胞病的临床特征、神经影像学和病理学改变。方法分析1例血管内皮细胞病患者的临床表现、实验室检查、神经影像学和病理学资料。结果该例患者反复发生脑卒中,并出现视网膜血管异常、蛋白尿和血尿,眼科检查提示眼底动脉节段性狭窄;头颅MRI显示额顶区和胼胝体长T_1、长T_2信号,注药后增强;数字减影血管造成检查未见颅内血管异常,超微结构检查发现神经、肌肉和皮肤血管出现独特的多层化基底膜。结论血管内皮细胞病易累及大脑、视网膜和肾脏。病理学检查可发现独特的多层化血管基底膜。Objective To study clinical features,neurological imaging and pathological changes of endotheliopathy. Methods We analyzed a case of endotheliopathy with its clinical,experimental investigation,neuroimaging and pathological investigation. Results The case had recurrent stroke,retinal vessel abnormality,proteinuria and hematuria.Ophthalmo- logic evaluations revealed arterial segmental stenosis,and brain magnetic resonance imaging(MRI)dem- onstrated high T_1,T_2 signal intensity and gadolinium-enhancing lesions in the right fronto-parietal region and corpus collosum.The digital subtraction arteriography(DSA)revealed normal.Ultrastructural studies showed distinctive multilaminated vascular basement membranes in superficial peroneal nerve,muscle and skin. Conclusion Endotheliopathy is a characteristic entity prone to involve in brain,retina and kidney.Patho- logic studies reveal distinctive multilaminated vascular basement membranes.

关 键 词：血管内皮细胞病 神经影像 病理学 

分 类 号：R774.1[医药卫生—眼科]