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Lookup NU author(s): Dr Maria Margallo-Lana,
Dr Brian Moore,
Emeritus Professor Robert Perry,
Dr Thomas Berney,
Dr Stephen Tyrer
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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. © 2007 The Authors. Journal Compilation © 2007 Blackwell Publishing Ltd.
Author(s): Margallo-Lana ML, Moore PB, Kay DWK, Perry RH, Reid BE, Berney TP, Tyrer SP
Publication type: Article
Publication status: Published
Journal: Journal of Intellectual Disability Research
ISSN (print): 0964-2633
ISSN (electronic): 1365-2788
Publisher: Wiley-Blackwell Publishing Ltd.
PubMed id: 17493029
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