Rafalowska J, Dziewulska D, Podlecka A, Zakrzewska-Pniewska B
Department of Experimental and Clinical Neuropathology, Medical Research Centre, Polish Academy of Sciences, Pawinskiego str. 5, 02-106 Warsaw, Poland.
Clin Neuropathol. 2006 Sep-Oct;25(5):237-42.
Vascular malformations usually develop as a result of influence of teratogenic factor(s) acting in the defined embryonic/fetal period. However, in the case examined by us, various types of vascular malformations formed in different periods of the ontogenic development were found. They were seen in all parts of the central nervous system and clinically mimicked multiple sclerosis. On the background of generalized ischemic lesions of the CNS, certain kinds of vascular malformations were seen: cavernous or fetallike vessels within meninges, superficially located capillary angioma penetrating into the brain and spinal cord white matter, and arterio-venous pathological conglomerates forming meningeal angiomatosis. In pathological vessels, immunocytochemical assessment of vascular endothelium with antibodies against antigens CD31, CD34, von Willebrand factor and lectin Ulex europaeus was normal but examination of the vascular basal membrane compounds revealed poor immunoreactivity to laminin and fibronectin. There were no disturbances in expression of angiopoietin, platelet-derived growth factor, transforming growth factor beta and vascular endothelial growth factor receptors Tie-1/2, PDGFR-alpha/beta, endoglin and Flk-1, respectively. The presence of various types of pathological vessels originating from different ontogenic periods indicates remittent or prolonged influence of teratogenic factor(s) in all periods of fetal vessel development.
血管畸形通常是由于致畸因素在特定胚胎/胎儿期起作用而形成的。然而，在我们所检查的病例中，发现了在个体发育不同阶段形成的各种类型的血管畸形。它们见于中枢神经系统的所有部位，临床上酷似多发性硬化症。在中枢神经系统广泛性缺血性病变的背景下，可见到某些类型的血管畸形：脑膜内的海绵状或胎儿样血管、穿透脑和脊髓白质的浅表性毛细血管血管瘤，以及形成脑膜血管瘤病的动静脉病理性团块。在病理性血管中，用抗CD31、CD34、血管性血友病因子抗原和欧洲荆豆凝集素的抗体对血管内皮进行免疫细胞化学评估结果正常，但对血管基底膜成分的检查显示，其对层粘连蛋白和纤连蛋白的免疫反应性较差。血管生成素、血小板衍生生长因子、转化生长因子β以及血管内皮生长因子受体Tie-1/2、PDGFR-α/β、内皮糖蛋白和Flk-1的表达均无异常。源自不同个体发育阶段的各种类型病理性血管的存在表明，致畸因素在胎儿血管发育的所有阶段都有间歇性或持续性的影响。
