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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| Title: | 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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| Authors: | Margallo-Lana ML (AUTHOR), Moore PB (AUTHOR), Kay DWK (AUTHOR), Perry RH (AUTHOR), Reid BE (AUTHOR), Berney TP (AUTHOR), Tyrer SP (AUTHOR) |
| Source: | Journal of Intellectual Disability Research. Jun2007, Vol. 51 Issue 6, p463-477. 15p. |
| Abstract: | 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. [ABSTRACT FROM AUTHOR] |
| Copyright of Journal of Intellectual Disability Research is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) | |
| Database: | Psychology and Behavioral Sciences Collection |
| FullText | Links: – Type: pdflink Text: Availability: 0 |
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| Header | DbId: pbh DbLabel: Psychology and Behavioral Sciences Collection An: 105874549 AccessLevel: 6 PubType: Academic Journal PubTypeId: academicJournal PreciseRelevancyScore: 0 |
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| Items | – Name: Title Label: Title Group: Ti Data: Fifteen-year follow-up of 92 hospitalized adults with Down's syndrome: incidence of cognitive decline, its relationship to age and neuropathology. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Margallo-Lana+ML%22">Margallo-Lana ML</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Moore+PB%22">Moore PB</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Kay+DWK%22">Kay DWK</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Perry+RH%22">Perry RH</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Reid+BE%22">Reid BE</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Berney+TP%22">Berney TP</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Tyrer+SP%22">Tyrer SP</searchLink> (AUTHOR) – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%22Journal+of+Intellectual+Disability+Research%22">Journal of Intellectual Disability Research</searchLink>. Jun2007, Vol. 51 Issue 6, p463-477. 15p. – Name: Abstract Label: Abstract Group: Ab Data: 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. [ABSTRACT FROM AUTHOR] – Name: AbstractSuppliedCopyright Label: Group: Ab Data: <i>Copyright of Journal of Intellectual Disability Research is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.) |
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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.1111/j.1365-2788.2006.00902.x Languages: – Code: eng Text: English PhysicalDescription: Pagination: PageCount: 15 StartPage: 463 Titles: – TitleFull: Fifteen-year follow-up of 92 hospitalized adults with Down's syndrome: incidence of cognitive decline, its relationship to age and neuropathology. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Margallo-Lana ML – PersonEntity: Name: NameFull: Moore PB – PersonEntity: Name: NameFull: Kay DWK – PersonEntity: Name: NameFull: Perry RH – PersonEntity: Name: NameFull: Reid BE – PersonEntity: Name: NameFull: Berney TP – PersonEntity: Name: NameFull: Tyrer SP IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 06 Text: Jun2007 Type: published Y: 2007 Identifiers: – Type: issn-print Value: 09642633 Numbering: – Type: volume Value: 51 – Type: issue Value: 6 Titles: – TitleFull: Journal of Intellectual Disability Research Type: main |
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