The pattern of amyloid accumulation in the brains of adults with Down syndrome.
The pattern of amyloid accumulation in the brains of adults with Down syndrome.
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DOI：
10.1016/j.jalz.2015.07.490
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发表时间：
2016-05
期刊：
影响因子：
--
通讯作者：
Holland AJ
中科院分区：
文献类型：
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作者：
Annus T;Wilson LR;Hong YT;Acosta-Cabronero J;Fryer TD;Cardenas-Blanco A;Smith R;Boros I;Coles JP;Aigbirhio FI;Menon DK;Zaman SH;Nestor PJ;Holland AJ
患有唐氏综合征（DS）的成年人不可避免地发展为阿尔茨海默病（AD）神经病理学。了解淀粉样蛋白沉积在DS可以产生关于疾病发病机制的重要信息。49名年龄在25-65岁的DS成人接受了匹兹堡化合物-B（PIB）的正电子发射断层扫描。根据年龄、临床和认知状态评估区域PIB结合。异常PIB结合变得明显，从39岁，首先在纹状体，然后是喙前额叶扣带回顶叶区域，然后是尾侧额叶，喙颞，初级感觉运动和枕叶，最后是海马旁皮质，丘脑和杏仁核。PIB结合与年龄、诊断状态和认知功能有关。DS中的PIB结合，首先出现在纹状体，开始于40岁左右，与痴呆和认知能力下降密切相关。淀粉样蛋白积累和认知能力下降之间没有明显的时间间隔，这与散发性/家族性AD形成对比，表明该人群适合进行淀粉样蛋白一级预防试验。
Adults with Down syndrome (DS) invariably develop Alzheimer's disease (AD) neuropathology. Understanding amyloid deposition in DS can yield crucial information about disease pathogenesis. Forty-nine adults with DS aged 25–65 underwent positron emission tomography with Pittsburgh compound–B (PIB). Regional PIB binding was assessed with respect to age, clinical, and cognitive status. Abnormal PIB binding became evident from 39 years, first in striatum followed by rostral prefrontal-cingulo-parietal regions, then caudal frontal, rostral temporal, primary sensorimotor and occipital, and finally parahippocampal cortex, thalamus, and amygdala. PIB binding was related to age, diagnostic status, and cognitive function. PIB binding in DS, first appearing in striatum, began around age 40 and was strongly associated with dementia and cognitive decline. The absence of a substantial time lag between amyloid accumulation and cognitive decline contrasts to sporadic/familial AD and suggests this population's suitability for an amyloid primary prevention trial.