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唐氏综合征中神经病理学改变及阿尔茨海默病性痴呆的发生情况。

Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's syndrome.

作者信息

Wisniewski K E, Wisniewski H M, Wen G Y

出版信息

Ann Neurol. 1985 Mar;17(3):278-82. doi: 10.1002/ana.410170310.

DOI:10.1002/ana.410170310
PMID:3158266
Abstract

One hundred brains of patients with Down's syndrome (DS) who died in institutions for chronic care were examined for clinicopathological correlation of Alzheimer's disease. Fifty-one were below and 49 were above age 30 years at death. Tissues from the right, prefrontal, and hippocampal cortices were processed for microscopy using H&E and Bodian-periodic acid-Schiff impregnation. Morphometric evaluations of plaques and tangles were carried out. Plaques or plaques and tangles were found in the brains of 56 patients with DS, 7 below age 30 and 49 above that age. A history of dementia was evident in the medical records of 15 of these patients; of these only 2 were below the age of 30. The brains of the patients with DS who also had clinical dementia had more than twenty plaques or plaques and tangles per 1.5 X 10(6) micron 2 of cortex. The numbers of plaques and tangles found in the brains of the patients with DS above the age of 30 greatly increased with age but varied from brain to brain. These observations suggest a correlation among dementia, the density of plaques and tangles, and age. All 100 brains studied showed early arrest of brain growth and brain atrophy, a condition that may have been due to prenatal arrest of neurogenesis mainly in the granular cell layers, prenatal and postnatal arrest of synaptogenesis, and early aging. Plaques and tangles developed twenty to thirty years earlier and dementia was clinically detected at least three times more frequently (20 to 30%) in DS than it is known to occur in the non-DS population.

摘要

对100例死于慢性病护理机构的唐氏综合征(DS)患者的大脑进行检查,以研究阿尔茨海默病的临床病理相关性。51例患者死亡时年龄低于30岁,49例患者死亡时年龄高于30岁。取右侧前额叶和海马皮质组织,用苏木精-伊红(H&E)染色和博迪安-过碘酸-希夫(Bodian-periodic acid-Schiff)浸染法进行显微镜检查。对斑块和缠结进行形态计量评估。在56例DS患者的大脑中发现了斑块或斑块与缠结,其中7例年龄低于30岁,49例年龄高于30岁。在这些患者的病历中,有15例有痴呆病史;其中只有2例年龄低于30岁。患有临床痴呆的DS患者大脑中,每1.5×10⁶平方微米皮质中有超过20个斑块或斑块与缠结。30岁以上DS患者大脑中发现的斑块和缠结数量随年龄大幅增加,但不同大脑之间存在差异。这些观察结果表明痴呆、斑块和缠结的密度与年龄之间存在相关性。所有研究的100例大脑均显示脑生长早期停滞和脑萎缩,这种情况可能是由于主要在颗粒细胞层的神经发生产前停滞、突触发生产前和产后停滞以及早衰所致。斑块和缠结的出现比正常情况早20至30年,DS患者临床检测到痴呆的频率至少是非DS人群的三倍(20%至30%)。

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Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's syndrome.唐氏综合征中神经病理学改变及阿尔茨海默病性痴呆的发生情况。
Ann Neurol. 1985 Mar;17(3):278-82. doi: 10.1002/ana.410170310.
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