Caplan L R, Schoene W C
Neurology. 1978 Dec;28(12):1206-15. doi: 10.1212/wnl.28.12.1206.
Subcortical arteriosclerotic encephalopathy, a chronic vascular dementia with hydrocephalus, was characterized pathologically in five patients by severe thickening of small vessels and by diffuse regions of white matter loss with gliosis. Lacunar infarcts were also present. The clinical picture in 11 patients was characterized by: (1) persistent hypertension and systemic vascular disease; (2) acute strokes; (3) subacute accumulation of focal neurologic symptoms and signs over weeks to months; (4) long plateau periods; (5) lengthy clinical course; (6) dementia; (7) prominent motor signs and pseudobulbar palsy and; (8) hydrocephalus. The pathogenesis of subcortical arteriosclerotic encephalopathy is unknown; possible mechanisms include diffuse ischemia and fluid transudation with subsequent gliosis related to subacute hypertensive encephalopathy.
皮质下动脉硬化性脑病是一种伴有脑积水的慢性血管性痴呆,5例患者的病理特征为小血管严重增厚以及伴有胶质增生的弥漫性白质丢失区域。还存在腔隙性梗死。11例患者的临床表现特征为:(1)持续性高血压和全身性血管疾病;(2)急性中风;(3)数周数月内亚急性局灶性神经症状和体征的累积;(4)长期平台期;(5)病程漫长;(6)痴呆;(7)明显的运动体征和假性延髓麻痹;(8)脑积水。皮质下动脉硬化性脑病的发病机制尚不清楚;可能的机制包括弥漫性缺血和液体渗出,随后出现与亚急性高血压脑病相关的胶质增生。