Mathers S E, Chambers B R, Merory J R, Alexander I
Department of Neurology, Repatriation General Hospital, Heidelberg, Melbourne, Victoria.
Clin Exp Neurol. 1987;23:67-70.
Binswanger, in his 1894 dissertation on the differential diagnosis of general paresis of the insane, described a slowly progressive dementia associated with macroscopic loss of white matter. In recent years interest in Binswanger's disease was rekindled with CT demonstration of extensive white matter low densities in some patients. To define the clinical spectrum, we reviewed 22 consecutive cases in which the CT appearances suggested a diagnosis of Binswanger's disease. Two patients had focal neurological deficits at presentation, but recent anoxic or hypoglycaemic insults could not be excluded as the cause of the CT abnormalities. The 20 remaining patients were demented and showed variable combinations of corticobulbar dysfunction and gait dyspraxia. The duration of symptoms ranged from a few months to several years. Sixty per cent of this group gave a history of discrete stroke events and focal cortical and/or lacunar infarcts were a frequent CT finding. Binswanger's disease is probably due to chronic or acute-on-chronic white matter ischaemia. The association with lacunar and cortical infarctions suggests that a combination of large and small vessel disease produces diffuse ischaemia maximal in white matter watershed zones. Binswanger's disease is clinically differentiated from multi-infarct dementia by its time course.
宾斯万格在其1894年关于精神病性全身麻痹鉴别诊断的论文中,描述了一种与白质宏观损失相关的缓慢进行性痴呆。近年来,随着CT显示一些患者存在广泛的白质低密度影,人们对宾斯万格病的兴趣再度燃起。为明确其临床谱,我们回顾了连续22例CT表现提示宾斯万格病诊断的病例。2例患者就诊时存在局灶性神经功能缺损,但不能排除近期缺氧或低血糖损伤是CT异常的原因。其余20例患者患有痴呆,表现出皮质延髓功能障碍和步态失用的不同组合。症状持续时间从几个月到几年不等。该组60%的患者有明确的卒中事件史,局灶性皮质和/或腔隙性梗死是常见的CT表现。宾斯万格病可能是由于慢性或慢性基础上的急性白质缺血所致。与腔隙性和皮质梗死的关联表明,大血管和小血管疾病共同作用导致白质分水岭区出现最大程度的弥漫性缺血。宾斯万格病在临床上通过其病程与多发梗死性痴呆相鉴别。