脑海绵状血管畸形的病理学及超微结构特点  

Characteristics of pathological and ultra structure in cerebral cavernous malformation

作　　者：位坤坤[1] 李文娜[1] 乔珊[1] 韩涛[1] 王胜军[1] 赵秀鹤[1] 迟兆富[1] 刘学伍[1] 

机构地区：[1]山东大学齐鲁医院神经内科,济南250012

出　　处：《临床神经病学杂志》2014年第4期241-243,共3页Journal of Clinical Neurology

基　　金：国家自然科学基金(81171071);山东省自然科学基金(ZR2010HM052)

摘　　要：目的探讨脑海绵状血管畸形(CCM)的病理学及超微结构特点。方法回顾性分析12例CCM患者的临床资料。结果在光镜下,CCM呈较大的窦腔状扩张的畸形血管,海绵状窦腔无弹力板及平滑肌,仅由一层血管内皮细胞和胶原纤维构成。窦腔内有不同阶段的瘀滞性静脉血、血栓、机化结构和无机盐。电子显微下见大小不等的多个血窦样扩张结构,窦腔内有大量红细胞,窦壁为一层菲薄的内皮细胞。基膜增厚、疏松、部分分层,见大量胶原纤维。部分基膜呈指状隆起指向窦腔。窦壁细胞由单层血管内皮细胞组成,胞质内可见微丝;部分内皮细胞间紧密连接消失、内皮细胞连续性中断。基膜外散在成纤维细胞,胞质内粗面内质网扩张。结论 CCM由一团大小不等且管腔扩张的异常血管构成,血管间无神经纤维组织。CCM表现为血窦样扩张结构,窦壁为一层菲薄的内皮细胞,细胞间紧密连接异常。Objective To explore the characteristics of pathological and ultra structure in cerebral cavernous malformation. Methods The clinical data of 12 cerebral cavernous malformation patients were analyzed retrospectively. Results In light microscope, cavernous malformation showed large vascular malformations with sinus dilatation, its sinus consists of a layer of endothelial cells and collagen fibers without elastic lamina and smooth muscle. The sinus was filled with venous stasis blood of different stages, thrombus, mechanical structure, and inorganic salts. Electron microscopy presents multiple sinus-like dilatation structures of varying sizes with a large number of red blood cells inside it, while a meager layer of endothelial cells froms the wall. Basement membrane showed thickened, loosen with sectional stratification, forms the wall of sinus, and there was a lot of collagen fibers. Some basement membrane was finger-like uplift pointing sinuses. A single layer of endothelial cells, in which filaments were visible in cytoplasm. Parts of tight junctions between endothelial cells disappeared, and endothelial cell ailed to continue. Fibroblast cells scattered outside the basement membrane, within which rough endoplasmie reticulum expanded in the cytoplasm. Conclusions Cerebral cavernous malformation is a group of abnormal vessels with luminal expansion in varying sizes, among whom no nerve fibers exist. It presents as sinus-like structures, whose wall is a meager layer of endothelial cells with abnormal tight junctions.

关 键 词：脑海绵状血管畸形 病理学特点 超微结构 

分 类 号：R743.4[医药卫生—神经病学与精神病学]