Brugge K L, Nichols S L, Salmon D P, Hill L R, Delis D C, Aaron L, Trauner D A
Department of Neurosciences, University of California, San Diego, School of Medicine, La Jolla.
Neurology. 1994 Feb;44(2):232-8. doi: 10.1212/wnl.44.2.232.
Postmortem studies of brains from adults with Down's syndrome (DS) reveal a dramatic age-dependent increase in the incidence of neuropathology associated with Alzheimer's disease (AD). By the age of 40 years, virtually all DS individuals have AD neuropathology. Documentation of cognitive correlates of this phenomenon has been difficult, partly because of the preexisting mental retardation in DS. In the current study, we compared a group of adults with DS, 22 to 51 years old, with a matched control group on various behavioral measures such as savings scores, which are known to be sensitive in detecting early dementia in AD patients. By using the short delayed savings score from the California Verbal Learning Test (a test of verbal memory), a subgroup of DS adults was identified as memory-impaired. This group demonstrated a decline in performance on various other cognitive tests with advancing age, whereas another group identified as having non-memory-impaired DS, and the non-DS controls, showed no evidence of decline with age. These results provide evidence for the presence of early dementia among adults with DS within an age range in which neuropathologic manifestations of AD are predicted to be developing.
对患有唐氏综合征（DS）的成年人进行的大脑尸检研究表明，与阿尔茨海默病（AD）相关的神经病理学发病率随年龄急剧增加。到40岁时，几乎所有唐氏综合征患者都有AD神经病理学表现。记录这一现象的认知相关性一直很困难，部分原因是唐氏综合征患者本身就存在智力发育迟缓。在当前的研究中，我们将一组年龄在22至51岁之间的唐氏综合征成年人与一个匹配的对照组在各种行为指标上进行了比较，比如节省分数，众所周知，节省分数对检测AD患者的早期痴呆很敏感。通过使用加利福尼亚言语学习测试（一项言语记忆测试）中的短延迟节省分数，确定了一组记忆受损的唐氏综合征成年人。随着年龄的增长，这组人在其他各种认知测试中的表现出现下降，而另一组被确定为非记忆受损的唐氏综合征患者以及非唐氏综合征对照组，均未显示出随年龄下降的迹象。这些结果为年龄范围内的唐氏综合征成年人中存在早期痴呆提供了证据，在这个年龄范围内预计AD的神经病理学表现正在发展。
