Akiguchi I, Tomimoto H, Suenaga T, Wakita H, Budka H
Department of Neurology, Faculty of Medicine, Kyoto University, Japan.
Acta Neuropathol. 1998 Jan;95(1):78-84. doi: 10.1007/s004010050768.
Binswanger's disease is pathologically characterized by a combination of diffuse cerebrovascular white matter lesions and lacunar infarcts in the basal ganglia and white matter. Although a blood-brain barrier (BBB) dysfunction has been implicated in the pathogenesis of these white matter (WM) lesions, few authors have addressed this problem. In the present study, we describe BBB dysfunction and its regional differences in the brains of Binswanger's disease patients. Twelve brains from Binswanger's disease patients (group III) were examined and compared with those from five patients with non-neurological disease (group I) and five cortical infarct patients without significant WM lesions (group II). Immunohistochemistry was performed for glial fibrillary acidic protein and vimentin as astroglial cell markers, and for immunoglobulins, complements and fibrinogen as extravasated serum protein markers. The grading scores for IgG extravasation were significantly higher in group III as compared to group I, in both the periventricular WM and the subcortical WM (P < 0.01). In group III, the scores in the periventricular WM and subcortical WM were significantly higher than in the subcortical U fibers and cerebral cortex (P < 0.01 for the periventricular WM; P < 0.001 for the subcortical WM), respectively. Clasmatodendritic astroglia, which had swollen cell bodies and large cytoplasmic vacuoles with disintegrated processes, incorporated the serum components IgG, IgM, C3d, Clq and fibrinogen, both in the periventricular WM and subcortical WM in 5 out of 12 (42%) Binswanger's disease brains. These results indicate that WM lesions in Binswanger's disease are accompanied by BBB dysfunction, although it remains uncertain whether BBB dysfunction is secondary to either chronic cerebral ischemia or arterial hypertension.
宾斯旺格病的病理特征是弥漫性脑血管白质病变与基底节和白质的腔隙性梗死并存。尽管血脑屏障（BBB）功能障碍被认为与这些白质（WM）病变的发病机制有关，但很少有作者探讨过这个问题。在本研究中，我们描述了宾斯旺格病患者大脑中BBB功能障碍及其区域差异。对12例宾斯旺格病患者（III组）的大脑进行了检查，并与5例非神经系统疾病患者（I组）和5例无明显WM病变的皮质梗死患者（II组）的大脑进行了比较。对胶质纤维酸性蛋白和波形蛋白进行免疫组织化学染色，作为星形胶质细胞标志物；对免疫球蛋白、补体和纤维蛋白原进行免疫组织化学染色，作为血管外渗血清蛋白标志物。与I组相比，III组在脑室周围WM和皮质下WM中的IgG外渗分级评分显著更高（P < 0.01）。在III组中，脑室周围WM和皮质下WM的评分分别显著高于皮质下U纤维和大脑皮质（脑室周围WM，P < 0.01；皮质下WM，P < 0.001）。在12例（42%）宾斯旺格病患者的大脑中，有5例在脑室周围WM和皮质下WM中，具有肿胀细胞体和带有崩解突起的大细胞质空泡的破碎性星形胶质细胞摄取了血清成分IgG、IgM、C3d、Clq和纤维蛋白原。这些结果表明，宾斯旺格病中的WM病变伴有BBB功能障碍，尽管BBB功能障碍是继发于慢性脑缺血还是动脉高血压仍不确定。
