Fifteen-year follow-up of 92 hospitalized adults with Down's syndrome: incidence of cognitive decline, its relationship to age and neuropathology
Fifteen-year follow-up of 92 hospitalized adults with Down's syndrome: incidence of cognitive decline, its relationship to age and neuropathology
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DOI：
10.1111/j.1365-2788.2006.00902.x
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发表时间：
2007-06-01
影响因子：
3.6
通讯作者：
Tyrer, S. P.
中科院分区：
文献类型：
--
作者：
Margallo-Lana, M. L.;Moore, P. B.;Tyrer, S. P.
唐氏综合征（DS）痴呆的临床和神经病理特征尚不明确。目的探讨成人退行性痴呆的临床病理相关性和认知能力下降的发生率。方法对1985年至2000年12月住院的92例退行性椎体滑移患者进行随访。一开始，87名参与者没有痴呆症，平均年龄为38岁。评估包括普拉德霍认知功能测试（PCFT）和适应行为量表（ABS），以衡量认知和行为的恶化。痴呆症是根据病例记录和护理人员的报告诊断出来的。18例（21%）患者在随访期间发生痴呆，中位发病年龄为55.5岁（45-74岁）。PCFT显示轻度智力残疾者（轻度和中度）认知能力下降，而重度智力残疾者（重度和重度）认知能力下降。临床痴呆与阿尔茨海默病的神经病理特征相关，并与新皮层神经原纤维缠结密度相关。在60岁及以上的患者中，略高于50%的患者仍然存在痴呆症的临床证据。结论：老年退行性痴呆患者常伴有可测量的认知和功能下降，在智力残疾程度较轻的人群中，可通过认知功能测试（如PCFT）和行为量表（如ABS）进行诊断。在智力残疾程度较深的人群中，可通过行为和神经学标准进行诊断。在本研究中，最大的前瞻性退行性痴呆系列研究包括对已故患者的神经病理学，与老年斑相比，神经原纤维缠结密度与退行性痴呆的关系更密切。在中老年退行性痴呆患者中，阿尔茨海默型痴呆的发展是频繁的，但不是必然的，一些退行性痴呆患者到老年时并没有痴呆的临床特征。
Background The clinical and neuropathological features associated with dementia in Down's syndrome (DS) are not well established. Aims To examine clinico-pathological correlations and the incidence of cognitive decline in a cohort of adults with DS. Method A total of 92 hospitalized persons with DS were followed up from 1985 to December 2000. At outset, 87 participants were dementia-free, with a median age of 38 years. Assessments included the Prudhoe Cognitive Function Test (PCFT) and the Adaptive Behavior Scale (ABS), to measure cognitive and behavioural deterioration. Dementia was diagnosed from case records and caregivers' reports. Results Eighteen (21%) patients developed dementia during follow-up, with a median age of onset 55.5 years (range 45-74). The PCFT demonstrated cognitive decline among those with a less severe intellectual disability (mild and moderate) but not among the profoundly disabled people (severe and profound). Clinical dementia was associated with neuropathological features of Alzheimer's disease, and correlated with neocortical neurofibrillary tangle densities. At the age of 60 years and above, a little more than 50% of patients still alive had clinical evidence of dementia. Conclusions Clinical dementia associated with measurable cognitive and functional decline is frequent in people with DS after middle age, and can be readily diagnosed among less severely intellectually disabled persons using measures of cognitive function such as the PCFT and behavioural scales such as the ABS. In the more profoundly disabled people, the diagnosis of dementia is facilitated by the use of behavioural and neurological criteria. In this study, the largest prospective DS series including neuropathology on deceased patients, the density of neurofibrillary tangles related more closely to the dementia of DS than senile plaques. In people with DS surviving to middle and old age, the development of dementia of Alzheimer type is frequent but not inevitable, and some people with DS reach old age without clinical features of dementia.