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Executive functioning in Cornelia de Lange syndrome: domain asynchrony and age-related performance

Overview of attention for article published in Journal of Neurodevelopmental Disorders, August 2017
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Title
Executive functioning in Cornelia de Lange syndrome: domain asynchrony and age-related performance
Published in
Journal of Neurodevelopmental Disorders, August 2017
DOI 10.1186/s11689-017-9208-7
Pubmed ID
Authors

Donna Reid, Jo Moss, Lisa Nelson, Laura Groves, Chris Oliver

Abstract

The aim of this study was to examine executive functioning in adolescents and adults with Cornelia de Lange syndrome (CdLS) to identify a syndrome and age-related profile of cognitive impairment. Participants were 24 individuals with CdLS aged 13-42 years (M = 22; SD = 8.98), and a comparable contrast group of 21 individuals with Down syndrome (DS) aged 15-33 years (M = 24; SD = 5.82). Measures were selected to test verbal and visual fluency, inhibition, perseverance/flexibility, and working memory and comprised both questionnaire and performance tests. Individuals with CdLS showed significantly greater impairment on tasks requiring flexibility and inhibition (rule switch) and on forwards span capacity. These impairments were also reported in the parent/carer-rated questionnaire measures. Backwards Digit Span was significantly negatively correlated with chronological age in CdLS, indicating increased deficits with age. This was not identified in individuals with DS. The relative deficits in executive functioning task performance are important in understanding the behavioural phenotype of CdLS. Prospective longitudinal follow-up is required to examine further the changes in executive functioning with age and if these map onto observed changes in behaviour in CdLS. Links with recent research indicating heightened responses to oxidative stress in CdLS may also be important.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 55 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 55 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 10 18%
Student > Master 9 16%
Student > Bachelor 9 16%
Researcher 5 9%
Student > Doctoral Student 5 9%
Other 5 9%
Unknown 12 22%
Readers by discipline Count As %
Psychology 14 25%
Nursing and Health Professions 10 18%
Medicine and Dentistry 6 11%
Social Sciences 3 5%
Biochemistry, Genetics and Molecular Biology 2 4%
Other 4 7%
Unknown 16 29%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 20 August 2017.
All research outputs
#20,444,703
of 22,999,744 outputs
Outputs from Journal of Neurodevelopmental Disorders
#453
of 479 outputs
Outputs of similar age
#276,394
of 316,580 outputs
Outputs of similar age from Journal of Neurodevelopmental Disorders
#12
of 12 outputs
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We're also able to compare this research output to 12 others from the same source and published within six weeks on either side of this one. This one is in the 1st percentile – i.e., 1% of its contemporaries scored the same or lower than it.