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. 2016 Nov 15:1:11.
doi: 10.12688/wellcomeopenres.9961.1.

The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome

Carla M Startin  1   2 , Sarah Hamburg  1   2 , Rosalyn Hithersay  1   2 , Amy Davies  1   3 , Erin Rodger  1 , Nidhi Aggarwal  1 , Tamara Al-Janabi  1   2 , André Strydom  1   2
Affiliations

The LonDownS adult cognitive assessment to study cognitive abilities and decline in Down syndrome

Carla M Startin et al. Wellcome Open Res. .

Abstract

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.

Keywords: Alzheimer’s disease; Down syndrome; cognition; dementia; executive function; intellectual disability; memory; motor coordination.

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Conflict of interest statement

AS has acted as an investigator for clinical trials sponsored by Roche Pharmaceuticals and consulted for Ono pharmaceuticals. He has been an adviser to the UK Down Syndrome Association and is an advisory board member of the LuMind Foundation (USA). No other authors have any competing interests to declare.

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