Cerebrovascular pathology in Down syndrome and Alzheimer disease.
Cerebrovascular pathology in Down syndrome and Alzheimer disease.
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DOI：
10.1186/s40478-017-0499-4
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发表时间：
2017-12-01
影响因子：
7.1
通讯作者：
Lott IT
中科院分区：
文献类型：
--
作者：
Head E;Phelan MJ;Doran E;Kim RC;Poon WW;Schmitt FA;Lott IT
随着年龄的增长，唐氏综合症（DS）患者患阿尔茨海默病（AD）的风险很高。通常，到40岁时，大多数退行性椎体滑移患者的神经病理学足以诊断为AD。有趣的是，随着年龄的增长，动脉粥样硬化和高血压在DS中不典型，这表明缺乏这些血管危险因素可能与脑血管病理减少有关。然而，由于APP的额外拷贝导致DS中β -淀粉样肽（Aβ）积累增加，我们假设相对于散发性AD，随着年龄的增长，DS中会有更广泛和广泛的脑淀粉样血管病（CAA）。为了验证这一假设，我们将CAA、动脉粥样硬化和小动脉硬化作为脑血管病理学的指标，并在DS （n = 32）、散发性AD （n = 80）和对照组（n = 37）的死后组织中进行了比较。与散发性AD和对照组相比，在DS患者的大脑中观察到CAA的频率明显更高。在退行性椎体滑移的病例中，动脉粥样硬化和小动脉硬化是罕见的。CAA可能是未来干预性临床试验的目标。
People with Down syndrome (DS) are at high risk for developing Alzheimer disease (AD) with age. Typically, by age 40 years, most people with DS have sufficient neuropathology for an AD diagnosis. Interestingly, atherosclerosis and hypertension are atypical in DS with age, suggesting the lack of these vascular risk factors may be associated with reduced cerebrovascular pathology. However, because the extra copy of APP leads to increased beta-amyloid peptide (Aβ) accumulation in DS, we hypothesized that there would be more extensive and widespread cerebral amyloid angiopathy (CAA) with age in DS relative to sporadic AD. To test this hypothesis CAA, atherosclerosis and arteriolosclerosis were used as measures of cerebrovascular pathology and compared in post mortem tissue from individuals with DS (n = 32), sporadic AD (n = 80) and controls (n = 37). CAA was observed with significantly higher frequencies in brains of individuals with DS compared to sporadic AD and controls. Atherosclerosis and arteriolosclerosis were rare in the cases with DS. CAA in DS may be a target for future interventional clinical trials.
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