The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome [version 1; referees: 2 approved]

Background: Down syndrome (DS), the most common genetic cause of intellectual disability, is associated with an ultra-high risk of developing Alzheimer’s disease. However, there is individual variability in the onset of clinical dementia and in baseline cognitive abilities prior to decline, particul...

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Main Authors: Carla M. Startin, Sarah Hamburg, Rosalyn Hithersay, Amy Davies, Erin Rodger, Nidhi Aggarwal, Tamara Al-Janabi, André Strydom
Format: Article
Language:English
Published: Wellcome 2016-11-01
Series:Wellcome Open Research
Subjects:
Online Access:https://wellcomeopenresearch.org/articles/1-11/v1
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author Carla M. Startin
Sarah Hamburg
Rosalyn Hithersay
Amy Davies
Erin Rodger
Nidhi Aggarwal
Tamara Al-Janabi
André Strydom
author_facet Carla M. Startin
Sarah Hamburg
Rosalyn Hithersay
Amy Davies
Erin Rodger
Nidhi Aggarwal
Tamara Al-Janabi
André Strydom
author_sort Carla M. Startin
collection DOAJ
description Background: Down syndrome (DS), the most common genetic cause of intellectual disability, is associated with an ultra-high risk of developing Alzheimer’s disease. However, there is individual variability in the onset of clinical dementia and in baseline cognitive abilities prior to decline, particularly in memory, executive functioning, and motor coordination. The LonDownS Consortium aims to determine risk and protective factors for the development of dementia and factors relating to cognitive abilities in people with DS. Here we describe our cognitive test battery and related informant measures along with reporting data from our baseline cognitive and informant assessments. Methods: We developed a cognitive test battery to assess general abilities, memory, executive function, and motor coordination abilities in adults with DS, with informant ratings of similar domains also collected, designed to allow for data on a broad range of participants. Participants (n=305) had a range of ages and abilities, and included adults with and without a clinical diagnosis of dementia. Results: Results suggest the battery is suitable for the majority of adults with DS, although approximately half the adults with dementia were unable to undertake any cognitive task. Many test outcomes showed a range of scores with low floor and ceiling effects. Non-verbal age-adjusted IQ scores had lower floor effects than verbal IQ scores. Before the onset of any cognitive decline, females aged 16-35 showed better verbal abilities compared to males. We also identified clusters of cognitive test scores within our battery related to visuospatial memory, motor coordination, language abilities, and processing speed / sustained attention. Conclusions: Our further studies will use baseline and longitudinal assessments to explore factors influencing cognitive abilities and cognitive decline related to ageing and onset of dementia in adults with DS.
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spelling doaj.art-9302829b8cd944928416901c5a7d333a2022-12-22T01:39:05ZengWellcomeWellcome Open Research2398-502X2016-11-01110.12688/wellcomeopenres.9961.110736The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome [version 1; referees: 2 approved]Carla M. Startin0Sarah Hamburg1Rosalyn Hithersay2Amy Davies3Erin Rodger4Nidhi Aggarwal5Tamara Al-Janabi6André Strydom7The LonDownS Consortium, University College London, London, UKThe LonDownS Consortium, University College London, London, UKThe LonDownS Consortium, University College London, London, UKFaculty of Health and Medical Sciences, University of Surrey, Guilford, UKUCL Division of Psychiatry, University College London, London, UKUCL Division of Psychiatry, University College London, London, UKThe LonDownS Consortium, University College London, London, UKThe LonDownS Consortium, University College London, London, UKBackground: Down syndrome (DS), the most common genetic cause of intellectual disability, is associated with an ultra-high risk of developing Alzheimer’s disease. However, there is individual variability in the onset of clinical dementia and in baseline cognitive abilities prior to decline, particularly in memory, executive functioning, and motor coordination. The LonDownS Consortium aims to determine risk and protective factors for the development of dementia and factors relating to cognitive abilities in people with DS. Here we describe our cognitive test battery and related informant measures along with reporting data from our baseline cognitive and informant assessments. Methods: We developed a cognitive test battery to assess general abilities, memory, executive function, and motor coordination abilities in adults with DS, with informant ratings of similar domains also collected, designed to allow for data on a broad range of participants. Participants (n=305) had a range of ages and abilities, and included adults with and without a clinical diagnosis of dementia. Results: Results suggest the battery is suitable for the majority of adults with DS, although approximately half the adults with dementia were unable to undertake any cognitive task. Many test outcomes showed a range of scores with low floor and ceiling effects. Non-verbal age-adjusted IQ scores had lower floor effects than verbal IQ scores. Before the onset of any cognitive decline, females aged 16-35 showed better verbal abilities compared to males. We also identified clusters of cognitive test scores within our battery related to visuospatial memory, motor coordination, language abilities, and processing speed / sustained attention. Conclusions: Our further studies will use baseline and longitudinal assessments to explore factors influencing cognitive abilities and cognitive decline related to ageing and onset of dementia in adults with DS.https://wellcomeopenresearch.org/articles/1-11/v1Cognitive Neurology & Dementia
spellingShingle Carla M. Startin
Sarah Hamburg
Rosalyn Hithersay
Amy Davies
Erin Rodger
Nidhi Aggarwal
Tamara Al-Janabi
André Strydom
The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome [version 1; referees: 2 approved]
Wellcome Open Research
Cognitive Neurology & Dementia
title The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome [version 1; referees: 2 approved]
title_full The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome [version 1; referees: 2 approved]
title_fullStr The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome [version 1; referees: 2 approved]
title_full_unstemmed The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome [version 1; referees: 2 approved]
title_short The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome [version 1; referees: 2 approved]
title_sort londowns adult cognitive assessment to study cognitive abilities and decline in down syndrome version 1 referees 2 approved
topic Cognitive Neurology & Dementia
url https://wellcomeopenresearch.org/articles/1-11/v1
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