Dubas F, Gray F, Roullet E, Escourolle R
Rev Neurol (Paris). 1985;141(2):93-108.
Seventeen clinico-pathological cases of leukoencephalopathy due to vascular diseases are reported. All of them had diffuse, often spotty demyelination sparing the U fibers, the corpus callosum, the internal capsule and the optic radiations. Microscopic examination of the white matter showed the association of the following lesions: oedema, swollen oligodendroglia, spongiosis, incomplete loss of myelin, astrocytic gliosis with Rosenthal's fibers, widening of the perivascular spaces with oedema fluid or hemosiderin laden macrophages, thickening and hyalinization of the blood vessels walls. Among those cases, 8 were consistent with Binswanger's subcortical encephalopathy (5 males, 3 females, mean age at death: 64 years, mean illness duration: 6, 7 years); all of them were hypertensive and had lacunae in the basal ganglia, hemispheric white matter and pons. Another case could be considered as a cortico-subcortical chronic hypertensive encephalopathy (77 y.o., female, illness duration: 4 years); she was hypertensive and had small cortical infarcts and perivascular bleedings, lobar and cerebellar hematomas, but no amyloid deposits in the blood vessels walls. Eight patients (5 males, 3 females mean age at death: 72 years, mean illness duration: 5, 4 years, 6 normotensive, 2 hypertensive) had diffuse meningocortical amyloid angiopathy with multiple small cortical infarcts, small cortical perivascular bleedings, slit haemorrhages and one or more lobar hematomas. Four of them had numerous senile plaques and neurofibrillary tangles. To our knowledge such a leukoencephalopathy in cerebral amyloid angiopathy has not been yet pointed out. It was present in 8 out of 12 cases of diffuse haemorrhagic form of cerebral amyloid angiopathy observed in the Charles Foix Laboratory of la Salpêtrière, during the last 10 years. A common mechanism with hypoperfusion of the distal white matter and alteration of the blood brain-barrier is suggested for this leukoencephalopathy.
报告了17例血管性疾病所致白质脑病的临床病理病例。所有病例均有弥漫性、常为斑点状脱髓鞘，U形纤维、胼胝体、内囊和视辐射未受累。白质显微镜检查显示有以下病变：水肿、少突胶质细胞肿胀、海绵状变性、髓鞘不完全丧失、伴有罗森塔尔纤维的星形细胞胶质增生、血管周围间隙因水肿液或含铁血黄素巨噬细胞而增宽、血管壁增厚和玻璃样变。其中8例符合宾斯旺格皮质下脑病（男5例，女3例，平均死亡年龄：64岁，平均病程：6.7年）；所有患者均患有高血压，基底节、半球白质和脑桥有腔隙。另一例可视为皮质 - 皮质下慢性高血压脑病（77岁，女性，病程：4年）；她患有高血压，有小的皮质梗死和血管周围出血、脑叶和小脑血肿，但血管壁无淀粉样沉积。8例患者（男5例，女3例，平均死亡年龄：72岁，平均病程：5.4年，6例血压正常，2例高血压）患有弥漫性脑膜皮质淀粉样血管病，伴有多发性小皮质梗死、小皮质血管周围出血、裂隙状出血和一个或多个脑叶血肿。其中4例有大量老年斑和神经原纤维缠结。据我们所知，脑淀粉样血管病中的这种白质脑病尚未被指出。在过去10年中，在拉萨尔佩特里埃的夏尔·福瓦实验室观察到的12例弥漫性出血型脑淀粉样血管病中，有8例存在这种情况。提示这种白质脑病的共同机制是远端白质灌注不足和血脑屏障改变。
