Sequence of cognitive decline in dementia in adults with Down's syndrome
Sequence of cognitive decline in dementia in adults with Down's syndrome
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DOI：
10.1046/j.1365-2788.2000.00305.x
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发表时间：
2000-12-01
影响因子：
3.6
通讯作者：
Silverman, WP
中科院分区：
文献类型：
--
作者：
Devenny, DA;Krinsky-McHale, SJ;Silverman, WP
众所周知，患有唐氏综合症（DS）的成年人有患阿尔茨海默氏型痴呆（DAT）的风险，但由于他们终生的智力缺陷，很难确定与年龄相关的衰退和痴呆的最早迹象和特征。在一项纵向研究中，所有参与者在进入研究时都是健康的，本文作者比较了WISC-R子测试的下降量，以确定与不同阶段痴呆相关的认知能力下降的顺序。22名不同程度认知能力下降的个体与44名保持健康的成人退行性痴呆患者进行了比较。在最初的测试中，所有的参与者都表现出轻度或中度的智力残疾。在WISC-R的每个子测试中，将健康参与者在研究期间经历的变化量与每个下降组的变化量进行比较。在表现出衰退的个体中，10名患有退行性痴呆的成年人被归类为“有问题的”衰退，这是基于记忆障碍的存在、唐氏综合症痴呆量表的得分和医生的诊断，5名和7名患有退行性痴呆的成年人分别被归类为DAT的“早期”和“中期”。研究发现，那些被认定为“有问题”的参与者，除了决定他们分类的记忆丧失之外，在分组设计和编码子测试中也表现出明显的下降。五名早期痴呆的成年人在这些测试中表现出下降，此外，在物体组装、图片完成、算术和理解测试中也表现出下降。七名处于痴呆症中期的成年人在这些测试中表现出下降，在信息、词汇和数字广度测试中也表现出下降。图片排列和相似性子测试在区分组之间没有用处，因为对相当大比例的参与者存在基线底效应。目前的纵向研究显示了与DAT相关的认知能力下降的顺序，从可能的“临床前”阶段开始，经过早期和中期阶段。这种方法开始定义认知能力下降的顺序，这种顺序有助于观察到阿尔茨海默病引起的功能退化，并且可能反映出随着疾病的进展皮质区域的参与。
Adults with Down's syndrome (DS) are known to be at risk of dementia of the Alzheimer type (DAT), but because of their lifelong intellectual deficits, it is difficult to determine the earliest signs and characteristics of age-associated decline and dementia. In a longitudinal study in which all participants were healthy at the time of their entry into the study, the present authors compared the amount of decline on the subtests of the WISC-R to determine the sequence of cognitive decline associated with varying stages of dementia. Twenty-two individuals with varying degrees of cognitive decline were compared to 44 adults with DS who have remained healthy. All participants functioned in the mild or moderate range of intellectual disability at initial testing. On each subtest of the WISC-R, the amount of change experienced by the healthy participants over the study period was compared to the amount of change found for each of the groups with decline. Out of the individuals who showed declines, 10 adults with DS were classified as having 'questionable' decline based on the presence of memory impairment, and five and seven adults with DS were classified as in the 'early stage' and 'middle stage' of DAT, respectively, based on the presence of memory impairment, score on the Dementia Scale for Down Syndrome and a physician's diagnosis. It was found that participants who were identified as 'questionable', in addition to the memory loss that determined their classification, also showed significant declines on the Block Design and Coding subtests. The five adults in the early stage of dementia showed declines on these subtests, and in addition, on the Object Assembly, Picture Completion, Arithmetic and Comprehension subtests. The seven adults in the middle stage of dementia showed declines on these subtests, plus declines on Information, Vocabulary and Digit Span subtests. The Picture Arrangement and Similarities subtests were not useful in distinguishing between the groups because of baseline floor effects for a substantial proportion of participants. The present longitudinal study showed a sequence of cognitive decline associated with DAT, beginning with a possible 'pre-clinical' stage, and progressing through the early and middle stages. This approach begins to define the sequence of declining cognitive capacities that contributes to the observed functional deterioration caused by Alzheimer's disease and that is likely to reflect the involvement of cortical areas as the disease progresses.