The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes

Abstract Background Cornelia de Lange (CdLS), Fragile X (FXS) and Rubinstein–Taybi syndromes (RTS) evidence unique profiles of autistic characteristics. To delineate these profiles further, the development of early social cognitive abilities in children with CdLS, FXS and RTS was compared to that ob...

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Autores principales: Katherine Ellis, Jo Moss, Chrysi Stefanidou, Chris Oliver, Ian Apperly
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Publicado: BMC 2021
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spelling oai:doaj.org-article:7fcc59ffb57f47c88bf32a9f28b07faf2021-11-28T12:22:45ZThe development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes10.1186/s13023-021-02117-41750-1172https://doaj.org/article/7fcc59ffb57f47c88bf32a9f28b07faf2021-11-01T00:00:00Zhttps://doi.org/10.1186/s13023-021-02117-4https://doaj.org/toc/1750-1172Abstract Background Cornelia de Lange (CdLS), Fragile X (FXS) and Rubinstein–Taybi syndromes (RTS) evidence unique profiles of autistic characteristics. To delineate these profiles further, the development of early social cognitive abilities in children with CdLS, FXS and RTS was compared to that observed in typically developing (TD) and autistic (AUT) children. Methods Children with CdLS (N = 22), FXS (N = 19) and RTS (N = 18), completed the Early Social Cognition Scale (ESCogS). Extant data from AUT (N = 19) and TD (N = 86) children were used for comparison. Results Similar to AUT children, children with CdLS, FXS and RTS showed an overall delay in passing ESCogS tasks. Children with CdLS showed a similar degree of delay to AUT children and greater delay than children with FXS and RTS. The CdLS, FXS and RTS groups did not pass tasks in the same sequence observed in TD and AUT children. Children with CdLS (p = 0.04), FXS (p = 0.02) and RTS (p = 0.04) performed better on tasks requiring understanding simple intentions in others significantly more than tasks requiring joint attention skills. Conclusions An underlying mechanism other than general cognitive delay may be disrupting early social cognitive development in children with CdLS, FXS and RTS. Factors that may disrupt early social cognitive development within these syndromes are discussed.Katherine EllisJo MossChrysi StefanidouChris OliverIan ApperlyBMCarticleDevelopmentIntellectual disabilityRare systemic diseasesAutismCornelia de Lange syndromeFragile X syndromeMedicineRENOrphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-12 (2021)
institution DOAJ
collection DOAJ
language EN
topic Development
Intellectual disability
Rare systemic diseases
Autism
Cornelia de Lange syndrome
Fragile X syndrome
Medicine
R
spellingShingle Development
Intellectual disability
Rare systemic diseases
Autism
Cornelia de Lange syndrome
Fragile X syndrome
Medicine
R
Katherine Ellis
Jo Moss
Chrysi Stefanidou
Chris Oliver
Ian Apperly
The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes
description Abstract Background Cornelia de Lange (CdLS), Fragile X (FXS) and Rubinstein–Taybi syndromes (RTS) evidence unique profiles of autistic characteristics. To delineate these profiles further, the development of early social cognitive abilities in children with CdLS, FXS and RTS was compared to that observed in typically developing (TD) and autistic (AUT) children. Methods Children with CdLS (N = 22), FXS (N = 19) and RTS (N = 18), completed the Early Social Cognition Scale (ESCogS). Extant data from AUT (N = 19) and TD (N = 86) children were used for comparison. Results Similar to AUT children, children with CdLS, FXS and RTS showed an overall delay in passing ESCogS tasks. Children with CdLS showed a similar degree of delay to AUT children and greater delay than children with FXS and RTS. The CdLS, FXS and RTS groups did not pass tasks in the same sequence observed in TD and AUT children. Children with CdLS (p = 0.04), FXS (p = 0.02) and RTS (p = 0.04) performed better on tasks requiring understanding simple intentions in others significantly more than tasks requiring joint attention skills. Conclusions An underlying mechanism other than general cognitive delay may be disrupting early social cognitive development in children with CdLS, FXS and RTS. Factors that may disrupt early social cognitive development within these syndromes are discussed.
format article
author Katherine Ellis
Jo Moss
Chrysi Stefanidou
Chris Oliver
Ian Apperly
author_facet Katherine Ellis
Jo Moss
Chrysi Stefanidou
Chris Oliver
Ian Apperly
author_sort Katherine Ellis
title The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes
title_short The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes
title_full The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes
title_fullStr The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes
title_full_unstemmed The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes
title_sort development of early social cognitive skills in neurogenetic syndromes associated with autism: cornelia de lange, fragile x and rubinstein–taybi syndromes
publisher BMC
publishDate 2021
url https://doaj.org/article/7fcc59ffb57f47c88bf32a9f28b07faf
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