An experimental study of executive function and social impairment in Cornelia de Lange syndrome

Research output: Contribution to journalArticlepeer-review

Standard

An experimental study of executive function and social impairment in Cornelia de Lange syndrome. / Nelson, Lisa; Crawford, Hayley; Reid, Donna; Moss, Joanna; Oliver, Christopher.

In: Journal of Neurodevelopmental Disorders, Vol. 9, No. 33, 11.09.2017.

Research output: Contribution to journalArticlepeer-review

Harvard

APA

Vancouver

Author

Bibtex

@article{7884b624b01a45649b06c34a2262612d,
title = "An experimental study of executive function and social impairment in Cornelia de Lange syndrome",
abstract = "Background: Extreme shyness and social anxiety is reported to be characteristic of adolescents and adults with Cornelia de Lange syndrome (CdLS); however, the nature of these characteristics is not well documented. In this study, we develop and apply an experimental assessment of social anxiety in a group of adolescents and adults with CdLS to determine the nature of the social difficulties and whether they are related to impairments in executive functioning.Methods: A familiar and unfamiliar examiner separately engaged in socially demanding tasks comprising three experimental conditions with a group of individuals with CdLS (n = 25; % male = 44; mean age = 22.16; SD = 8.81) and a comparable group of individuals with Down syndrome (DS; n = 20; % male = 35; mean age = 24.35; SD = 5.97). Behaviours indicative of social anxiety were coded. The Behavior Rating Inventory of Executive Function-Preschool version, an informant measure of executive function, was completed by participants{\textquoteright} caregivers.Results: Significantly less verbalisation was observed in the CdLS group than the DS group in conditions requiring the initiation of speech. In the CdLS group, impairments in verbalisation were not associated with a greater degree of intellectual disability but were significantly correlated with impairments in both planning and working memory. This association was not evident in the DS group.Conclusions: Adolescents and adults with CdLS have a specific difficulty with the initiation of speech when social demands are placed upon them. This impairment in verbalisation may be underpinned by specific cognitive deficits, although further research is needed to investigate this fully.",
keywords = "executive function , social anxiety, Cornelia de Lange syndrome , Down syndrome",
author = "Lisa Nelson and Hayley Crawford and Donna Reid and Joanna Moss and Christopher Oliver",
year = "2017",
month = sep,
day = "11",
doi = "10.1186/s11689-017-9213-x",
language = "English",
volume = "9",
journal = "Journal of Neurodevelopmental Disorders",
issn = "1866-1955",
publisher = "Springer",
number = "33",

}

RIS

TY - JOUR

T1 - An experimental study of executive function and social impairment in Cornelia de Lange syndrome

AU - Nelson, Lisa

AU - Crawford, Hayley

AU - Reid, Donna

AU - Moss, Joanna

AU - Oliver, Christopher

PY - 2017/9/11

Y1 - 2017/9/11

N2 - Background: Extreme shyness and social anxiety is reported to be characteristic of adolescents and adults with Cornelia de Lange syndrome (CdLS); however, the nature of these characteristics is not well documented. In this study, we develop and apply an experimental assessment of social anxiety in a group of adolescents and adults with CdLS to determine the nature of the social difficulties and whether they are related to impairments in executive functioning.Methods: A familiar and unfamiliar examiner separately engaged in socially demanding tasks comprising three experimental conditions with a group of individuals with CdLS (n = 25; % male = 44; mean age = 22.16; SD = 8.81) and a comparable group of individuals with Down syndrome (DS; n = 20; % male = 35; mean age = 24.35; SD = 5.97). Behaviours indicative of social anxiety were coded. The Behavior Rating Inventory of Executive Function-Preschool version, an informant measure of executive function, was completed by participants’ caregivers.Results: Significantly less verbalisation was observed in the CdLS group than the DS group in conditions requiring the initiation of speech. In the CdLS group, impairments in verbalisation were not associated with a greater degree of intellectual disability but were significantly correlated with impairments in both planning and working memory. This association was not evident in the DS group.Conclusions: Adolescents and adults with CdLS have a specific difficulty with the initiation of speech when social demands are placed upon them. This impairment in verbalisation may be underpinned by specific cognitive deficits, although further research is needed to investigate this fully.

AB - Background: Extreme shyness and social anxiety is reported to be characteristic of adolescents and adults with Cornelia de Lange syndrome (CdLS); however, the nature of these characteristics is not well documented. In this study, we develop and apply an experimental assessment of social anxiety in a group of adolescents and adults with CdLS to determine the nature of the social difficulties and whether they are related to impairments in executive functioning.Methods: A familiar and unfamiliar examiner separately engaged in socially demanding tasks comprising three experimental conditions with a group of individuals with CdLS (n = 25; % male = 44; mean age = 22.16; SD = 8.81) and a comparable group of individuals with Down syndrome (DS; n = 20; % male = 35; mean age = 24.35; SD = 5.97). Behaviours indicative of social anxiety were coded. The Behavior Rating Inventory of Executive Function-Preschool version, an informant measure of executive function, was completed by participants’ caregivers.Results: Significantly less verbalisation was observed in the CdLS group than the DS group in conditions requiring the initiation of speech. In the CdLS group, impairments in verbalisation were not associated with a greater degree of intellectual disability but were significantly correlated with impairments in both planning and working memory. This association was not evident in the DS group.Conclusions: Adolescents and adults with CdLS have a specific difficulty with the initiation of speech when social demands are placed upon them. This impairment in verbalisation may be underpinned by specific cognitive deficits, although further research is needed to investigate this fully.

KW - executive function

KW - social anxiety

KW - Cornelia de Lange syndrome

KW - Down syndrome

U2 - 10.1186/s11689-017-9213-x

DO - 10.1186/s11689-017-9213-x

M3 - Article

VL - 9

JO - Journal of Neurodevelopmental Disorders

JF - Journal of Neurodevelopmental Disorders

SN - 1866-1955

IS - 33

ER -