Raghavan R, Khin-Nu C, Brown A G, Day K A, Tyrer S P, Ince P G, Perry E K, Perry R H
Department of Neuropathology, Newcastle General Hospital, Newcastle upon Tyne, UK.
Neuroreport. 1994 Jun 27;5(11):1393-6.
Twenty-eight individuals with typical Down's syndrome (DS) phenotype (17 males and 11 females; age range: 10-74 years) were investigated for gender differences in the phenotypic expression of Alzheimer-type pathology (ATP). Quantitative neuropathology was performed in the 4 neocortical lobes of the right hemisphere, by counting senile plaques (SP), and neurofibrillary tangles (NFT). ATP was present in 25 middle-aged (> 40 years) individuals (16 males and 9 females). Females had significantly higher (p = 0.03) mean neocortical NFT densities (36.6 per mm2; s.e.m. +/- 6.6) than males (17.9 per mm2; s.e.m. +/- 4.7). None of the females had NFT densities below 10 per mm2, compared with 6 males in whom NFT were either absent or seen in very low densities (< 4 per mm2). Assessment of SP densities in the same cortical regions showed non-significant differences in females (42.4 per mm2; s.e.m. +/- 5.1) compared with males (33.6 per mm2; s.e.m. +/- 2.1). There was clinical evidence of dementia in all the female (8/8) individuals who were prospectively assessed, compared with only 54% (7/13) of males. The male individuals without clinical dementia had absent or low neocortical NFT densities regardless of high SP densities. Female DS cases (mean age: 48.8 years; s.e.m. +/- 1.9) had an earlier onset of dementia than males (mean age: 53.6 years; s.e.m. +/- 1.3; p = 0.05). Female middle-aged DS individuals have an earlier onset, and a more severe form of AD which correlates with higher neocortical NFT rather than SP density.
对28名具有典型唐氏综合征（DS）表型的个体（17名男性和11名女性；年龄范围：10 - 74岁）进行了阿尔茨海默病型病理（ATP）表型表达的性别差异研究。通过计数老年斑（SP）和神经原纤维缠结（NFT），在右半球的4个新皮质叶进行了定量神经病理学检查。25名中年（> 40岁）个体（16名男性和9名女性）存在ATP。女性的平均新皮质NFT密度（每平方毫米36.6个；标准误±6.6）显著高于男性（每平方毫米17.9个；标准误±4.7；p = 0.03）。没有女性的NFT密度低于每平方毫米10个，相比之下，有6名男性不存在NFT或其密度极低（<每平方毫米4个）。对相同皮质区域SP密度的评估显示，女性（每平方毫米42.4个；标准误±5.1）与男性（每平方毫米33.6个；标准误±2.1）相比无显著差异。在所有接受前瞻性评估的女性（8/8）个体中有痴呆的临床证据，相比之下男性只有54%（7/13）有该证据。没有临床痴呆的男性个体，无论SP密度多高，其新皮质NFT密度均不存在或较低。女性DS病例（平均年龄：48.8岁；标准误±1.9）的痴呆发病年龄早于男性（平均年龄：53.6岁；标准误±1.3；p = 0.05）。中年女性DS个体发病更早，且患更严重形式的AD，这与更高的新皮质NFT而非SP密度相关。
