The Down syndrome brain in the presence and absence of fibrillar β-amyloidosis.
The Down syndrome brain in the presence and absence of fibrillar β-amyloidosis.
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DOI：
10.1016/j.neurobiolaging.2017.01.009
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发表时间：
2017-05
影响因子：
4.2
通讯作者：
Nestor PJ
中科院分区：
文献类型：
--
作者：
Annus T;Wilson LR;Acosta-Cabronero J;Cardenas-Blanco A;Hong YT;Fryer TD;Coles JP;Menon DK;Zaman SH;Holland AJ;Nestor PJ
唐氏综合征（DS）患者的大脑神经发育不同，并且在50岁时总是出现淀粉样蛋白神经病理学。这项横断面研究旨在提供DS脑形态学的详细描述和淀粉样蛋白神经病理学的变化。46名患有DS的成年人接受了结构和淀粉样蛋白成像-后者使用匹兹堡化合物B（PI B）将队列分为PIB阳性（n = 19）和PIB阴性（n = 27）。对照组（n = 30）进行结构成像。研究了深部灰质体积和皮质厚度的组间差异。PIB阴性的DS患者具有神经发育不典型的大脑，其特征是与对照组相比，额叶和枕顶皮层不成比例地增厚，运动皮层和颞极变薄，壳核变大，脑嵴变小。在淀粉样蛋白神经病理学的存在下，DS大脑表现出惊人的相似模式的后显性皮质变薄和皮质下萎缩的海马，丘脑和纹状体，在非DS阿尔茨海默病中观察到的。必须小心，以避免低估淀粉样蛋白相关的形态学变化，在DS由于不成比例的大小，一些皮质下结构和厚度的皮质。
People with Down syndrome (DS) have a neurodevelopmentally distinct brain and invariably developed amyloid neuropathology by age 50. This cross-sectional study aimed to provide a detailed account of DS brain morphology and the changes occuring with amyloid neuropathology. Forty-six adults with DS underwent structural and amyloid imaging—the latter using Pittsburgh compound B (PIB) to stratify the cohort into PIB-positive (n = 19) and PIB-negative (n = 27). Age-matched controls (n = 30) underwent structural imaging. Group differences in deep gray matter volumetry and cortical thickness were studied. PIB-negative people with DS have neurodevelopmentally atypical brain, characterized by disproportionately thicker frontal and occipitoparietal cortex and thinner motor cortex and temporal pole with larger putamina and smaller hippocampi than controls. In the presence of amyloid neuropathology, the DS brains demonstrated a strikingly similar pattern of posterior dominant cortical thinning and subcortical atrophy in the hippocampus, thalamus, and striatum, to that observed in non-DS Alzheimer's disease. Care must be taken to avoid underestimating amyloid-associated morphologic changes in DS due to disproportionate size of some subcortical structures and thickness of the cortex.