ArticleJournal of neurodevelopmental disorders2017
Executive functioning in Cornelia de Lange syndrome: domain asynchrony and age-related performance.
Article in Journal of neurodevelopmental disorders, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 24 citations in OpenAlex.
- Evidence of spontaneous mentalizing in children with Cornelia de Lange and fragile X syndromes, but not autistic children.Oxford open neuroscience · 2025Article
- Clinical Characteristics, Genetic Analysis, and Literature Review of Cornelia de Lange Syndrome Type 4 Associated With a RAD21 Variant.Molecular genetics & genomic medicine · 2024Review
- Divergent presentation of anxiety in high-risk groups within the intellectual disability population.Journal of neurodevelopmental disorders · 2022Article
- Development, behaviour and sensory processing in Marshall-Smith syndrome and Malan syndrome: phenotype comparison in two related syndromes.Journal of intellectual disability research : JIDR · 2020Article
- An Observational Study of Social Interaction Skills and Behaviors in Cornelia de Lange, Fragile X and Rubinstein-Taybi Syndromes.Journal of autism and developmental disorders · 2020Observational
- A Behavioural Assessment of Social Anxiety and Social Motivation in Fragile X, Cornelia de Lange and Rubinstein-Taybi Syndromes.Journal of autism and developmental disorders · 2020Article
- Lifespan trajectory of affect in Cornelia de Lange syndrome: towards a neurobiological hypothesis.Journal of neurodevelopmental disorders · 2019Article
- Age-related Behavioural Change in Cornelia de Lange and Cri du Chat Syndromes: A Seven Year Follow-up Study.Journal of autism and developmental disorders · 2019Article
- Cornelia De Lange Syndrome In A 4-Year-Old Child From India: Phenotype Description And Role Of Genetic Counseling.Medical archives (Sarajevo, Bosnia and Herzegovina) · 2018Article
- Diagnosis and management of Cornelia de Lange syndrome: first international consensus statement.Nature reviews. Genetics · 2018Review
- Memory profiles in Down syndrome across development: a review of memory abilities through the lifespan.Journal of neurodevelopmental disorders · 2018Review
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe 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.
methodsParticipants 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.
resultsIndividuals 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.
conclusionsThe 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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