Department of Neuroscience, University of Florida College of Medicine, Gainesville, Florida.
Clinical Neuroanatomy (Department of Neurology), Center for Biomedical Research, University of Ulm, Ulm, Germany.
Glia. 2018 Dec;66(12):2550-2562. doi: 10.1002/glia.23510. Epub 2018 Nov 11.
Sporadic Alzheimer's disease (AD) is marked by a lengthy preclinical phase during which patients are nonsymptomatic but show pathology in variable manifestations. Whether or not neuroinflammation occurs in such nondemented individuals is unknown. We evaluated the medial temporal lobe of 66 nondemented subjects, aged 42-93, in terms of tau pathology, Aβ deposition, and microglial activation. We show that 100% of subjects had neurofibrillary degeneration (NFD), 35% had Aβ deposits, and 8% revealed microglial activation in individuals where early amyloid formation was apparent by Congo Red staining. Amyloid-induced neuroinflammatory clusters of Iba1, CD68, and ferritin-positive microglia were evident in the immediate vicinity of aggregated Aβ. Microglia in the adjacent neuropil were nonactivated. Thus, neuroinflammation in AD represents a highly localized phagocyte reaction, essentially a foreign body response, geared toward removal of insoluble Aβ. Because clustered microglia in some amyloid plaques were dystrophic and ferritin-positive, we hypothesize that these cells were exhausted by their attempts to remove the aggregated, insoluble Aβ. Our findings show that the sequence of pathologic events in AD begins with tau pathology, followed by Aβ deposition, and then by microglial activation. Because only 8% of our subjects revealed all three hallmark pathologic features, we propose that these nondemented individuals were near the threshold of transitioning from nonsymptomatic to symptomatic disease. The onset of neuroinflammation in AD may thus represent a tipping point in AD pathogenesis. Our study suggests that the role of microglia in AD pathogenesis entails primarily the attempted removal of potentially toxic, extracellular material.
散发性阿尔茨海默病(AD)的特征是存在一个漫长的临床前期,在此期间患者没有症状,但表现出不同表现的病理学变化。在没有痴呆的个体中是否发生神经炎症尚不清楚。我们评估了 66 名年龄在 42-93 岁之间的无痴呆受试者的内侧颞叶,评估指标包括 Tau 病理学、Aβ 沉积和小胶质细胞激活。我们发现,100%的受试者存在神经纤维缠结(NFD),35%的受试者存在 Aβ 沉积,8%的受试者在刚果红染色显示早期淀粉样形成的情况下,存在小胶质细胞激活。在聚集的 Aβ 附近,可见到淀粉样蛋白诱导的小胶质细胞 Iba1、CD68 和铁蛋白阳性的炎症簇。邻近神经胶质的小胶质细胞没有被激活。因此,AD 中的神经炎症代表了一种高度局部的吞噬细胞反应,本质上是一种异物反应,旨在清除不溶性 Aβ。由于一些淀粉样斑块中的聚集小胶质细胞出现营养不良和铁蛋白阳性,我们假设这些细胞在试图清除聚集的不溶性 Aβ 时已经衰竭。我们的发现表明,AD 中病理事件的顺序首先是 Tau 病理学,然后是 Aβ 沉积,最后是小胶质细胞激活。由于我们的研究对象中只有 8%显示出所有三个标志性的病理特征,我们提出这些无痴呆的个体处于从无症状向有症状疾病转变的临界点。AD 中神经炎症的发生可能代表 AD 发病机制中的一个转折点。我们的研究表明,小胶质细胞在 AD 发病机制中的作用主要是试图清除潜在的毒性、细胞外物质。