Romero López J, Rivas Infante E, Maciñeiras Montero J L, Montero Carretero M J
Sección de Neurología, Hospital do Meixoeiro, Vigo.
Neurologia. 2006 Dec;21(10):729-32.
Middle-age Down's syndrome patients develop dementia with antomicopathological changes that are characteristic for Alzheimer's disease. Cerebral amyolid angiopathy (CAA) most commonly manifests itself as a lobar intracerebral haemorrhage that tends to recur. Multiple haemorrhages may occur simultaneously. CAA is frequently associated with Alzheimer's disease, however the relationship between CCA and Down's syndrome is poorly know. Although there are established clinical criteria for the diagnosis of CAA related to lobar intracerebral haemorrhage, definitive CAA needs anatomicopathologial confirmation. This paper presents the case of a male with Down's syndrome and recurrent lobar intracerebral haemorrhage. An autopsy showed lesions in leptomeningeal and cortical arteries which are characteristic of CAA in addition to anatomicopathological changes of Alzheimer s disease that were mostly seen in entorhinal cortex and hippocampus. The paper also discusses the pathological association between beta-amyloid protein, apolipoprotein alleles, CAA, Alzheimer's disease and Down's syndrome.
中年唐氏综合征患者会出现痴呆,并伴有阿尔茨海默病特有的解剖病理学变化。脑淀粉样血管病(CAA)最常见的表现是脑叶内脑出血,且容易复发。可能会同时发生多处出血。CAA常与阿尔茨海默病相关,但CAA与唐氏综合征之间的关系却鲜为人知。尽管已有针对与脑叶内脑出血相关的CAA的诊断临床标准,但确诊CAA需要解剖病理学证实。本文介绍了一名患有唐氏综合征且反复发生脑叶内脑出血的男性病例。尸检显示,除了在内嗅皮质和海马体中常见的阿尔茨海默病的解剖病理学变化外,软脑膜和皮质动脉还存在CAA特有的病变。本文还讨论了β-淀粉样蛋白、载脂蛋白等位基因、CAA、阿尔茨海默病和唐氏综合征之间的病理关联。