Department of Molecular & Cellular Biochemistry, University of Kentucky, Lexington, KY, USA.
Sanders Brown Center on Aging, University of Kentucky, Lexington, KY, USA.
J Alzheimers Dis. 2019;67(1):103-112. doi: 10.3233/JAD-180589.
Cerebrovascular pathology is a significant mediator in Alzheimer's disease (AD) in the general population. In people with Down syndrome (DS), the contribution of vascular pathology to dementia may play a similar role in age of onset and/or the rate of progression of AD. In the current study, we explored the extent of microbleeds (MBs) and the link between cerebral amyloid angiopathy (CAA) and MBs in the frontal cortex (FCTX) and occipital cortex (OCTX) in an autopsy series from individuals with DS (<40 years), DS with AD pathology (DSAD), sporadic AD, and control cases (2-83 years). Sections were immunostained against Aβ1 - 40 and an adjacent section stained using Prussian blue for MBs. MBs were both counted and averaged in each case and CAA was scored based on previously published methods. MBs were more frequent in DS cases relative to controls but present to a similar extent as sporadic AD. This aligned with CAA scores, with more extensive CAA in DS relative to controls in both brain regions. CAA was also more frequent in DSAD cases relative to sporadic AD. We found CAA to be associated with MBs and that MBs increased with age in DS after 30 years of age in the OCTX and after 40 years of age in the FCTX. MB and CAA appear to be a significant contributors to the development of dementia in people with DS and are important targets for future clinical trials.
脑血管病理学是普通人群中阿尔茨海默病(AD)的重要介导因素。在唐氏综合征(DS)患者中,血管病理学对痴呆的贡献可能在发病年龄和/或 AD 的进展速度方面发挥类似作用。在本研究中,我们在一系列尸检样本中探讨了微出血(MBs)的程度,以及额皮质(FCTX)和枕皮质(OCTX)中的脑淀粉样血管病(CAA)与 MBs 之间的关系,这些样本来自 DS(<40 岁)、DS 合并 AD 病理(DSAD)、散发性 AD 和对照病例(2-83 岁)。组织切片用 Aβ1-40 进行免疫染色,并用普鲁士蓝对 MBs 进行相邻染色。在每个病例中,我们都对 MBs 进行计数和平均,并根据先前发表的方法对 CAA 进行评分。DS 病例中的 MBs 比对照组更频繁,但与散发性 AD 相似。这与 CAA 评分一致,DS 在两个脑区的 CAA 均比对照组更为广泛。DSAD 病例中的 CAA 也比散发性 AD 更频繁。我们发现 CAA 与 MBs 相关,并且在 OCTX 中,DS 在 30 岁后和在 FCTX 中在 40 岁后,MBs 随年龄增长而增加。MB 和 CAA 似乎是 DS 患者发生痴呆的重要原因,也是未来临床试验的重要目标。