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50岁以下唐氏综合征患者大脑中斑块和缠结的形成模式。

The pattern of acquisition of plaques and tangles in the brains of patients under 50 years of age with Down's syndrome.

作者信息

Mann D M, Esiri M M

机构信息

Department of Pathology, University of Manchester, U.K.

出版信息

J Neurol Sci. 1989 Feb;89(2-3):169-79. doi: 10.1016/0022-510x(89)90019-1.

DOI:10.1016/0022-510x(89)90019-1
PMID:2522541
Abstract

The form and distribution of senile plaques (SP) and neurofibrillary tangles (NFT) has been examined in the brains of 13 patients with Down's syndrome (DS), aged less than or equal to 50 years, using immunocytochemical and silver staining procedures. SP become present within the brain before NFT and these appear firstly as fine, even, diffuse areas of anti-amyloid (A4) protein immunoreactivity in the absence of any discernable neuritic change. Later the amount of anti-A4 protein in SP increases and SP show a marked surrounding neuritic response which is detectable using either silver or anti-paired helical filament (PHF) staining methods. At this stage NFT also become detectable within the perikaryon of nerve cells in both the cortex and the subcortex, with the large stellate neurones of layer II of the entorhinal cortex showing an early involvement. By the age of 50 years, most patients are well on the way towards achieving (and some have already achieved) that pattern of SP and NFT morphology and distribution that is typically seen in patients over 50 years of age with DS and in other patients in the general population with Alzheimer's disease.

摘要

采用免疫细胞化学和银染法,对13例年龄小于或等于50岁的唐氏综合征(DS)患者的大脑中老年斑(SP)和神经原纤维缠结(NFT)的形态和分布进行了检查。SP在NFT之前出现在大脑中,最初表现为抗淀粉样蛋白(A4)蛋白免疫反应性的细微、均匀、弥漫区域,此时没有任何可辨别的神经炎性改变。后来,SP中抗A4蛋白的量增加,SP显示出明显的周围神经炎性反应,使用银染或抗双螺旋丝(PHF)染色方法均可检测到。在这个阶段,NFT在皮质和皮质下神经细胞的核周体中也可检测到,内嗅皮质第II层的大星形神经元早期受累。到50岁时,大多数患者已朝着(有些患者已经达到)50岁以上DS患者以及普通人群中其他阿尔茨海默病患者典型的SP和NFT形态及分布模式发展。

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