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伴有复发性中风及CT扫描显示白质低密度影的缓慢进行性家族性痴呆

Slowly progressive familial dementia with recurrent strokes and white matter hypodensities on CT scan.

作者信息

Salvi F, Michelucci R, Plasmati R, Parmeggiani L, Zonari P, Mascalchi M, Tassinari C A

机构信息

Clinica Neurologica Università di Bologna, Ospedale Bellaria.

出版信息

Ital J Neurol Sci. 1992 Mar;13(2):135-40. doi: 10.1007/BF02226962.

Abstract

We describe 2 normotensive sisters presenting slowly progressive dementia associated with acute or subacute focal neurological symptoms, unilateral or bilateral motor signs, and dysarthria. Their father, who died in the seventh decade, had a similar clinical picture. Computerized axial tomography (CT) scan of the head showed symmetrical hypodensities in the periventricular white matter and mild to moderate hydrocephalus. In these patients a diagnosis of Binswanger's disease was based on the clinical features supported by white matter changes on CT scan. Our study suggests that genetic factors may play a role in the etiology of Binswanger's disease.

摘要

我们描述了2名血压正常的姐妹,她们表现出与急性或亚急性局灶性神经症状、单侧或双侧运动体征及构音障碍相关的缓慢进展性痴呆。她们死于七旬的父亲有类似的临床表现。头部计算机断层扫描(CT)显示脑室周围白质对称低密度影及轻至中度脑积水。在这些患者中,基于CT扫描显示的白质改变所支持的临床特征,诊断为宾斯旺格病。我们的研究提示,遗传因素可能在宾斯旺格病的病因中起作用。

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