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

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.