Longitudinal investigation of cognition, social competence, and anxiety in children and adolescents with Turner syndrome
Open accessAbstract
Turner syndrome (TS), a common neurogenetic disorder caused by complete or partial absence of an X chromosome in females, is characterized by distinct physical, cognitive, and social-emotional features. Girls with TS typically display average overall intellectual functioning with relative strength in verbal abilities and weaknesses in visuospatial processing, executive function (EF), and social cognition. This study was designed to better understand longitudinal trajectories of cognitive and social-emotional domains commonly affected in TS. Participants included 57 girls with monosomic 45,X TS and 55 age- and verbal-IQ matched girls who completed behavioral, child-report, and parent-report measures across four timepoints. Group differences in visuospatial processing, EF, social cognition, and anxiety were assessed longitudinally. Potential effects of estrogen replacement therapy (ERT) were assessed cross-sectionally on an exploratory basis. The TS group showed poorer performance on measures of visuospatial processing, EF, and social cognition, but not anxiety, compared to controls throughout childhood and adolescence. There were no significant group differences in the trajectory of skill development over time. Exploratory analyses within the TS group revealed that girls who were receiving ERT showed better performance on measures of overall IQ, expressive vocabulary, and visuospatial processing compared to those not receiving ERT. Consistent with existing literature, weaknesses in visuospatial processing, EF, and social competence among girls with TS persisted throughout childhood and adolescence. Exploratory analyses suggest that ERT may help improve some aspects of cognitive function in TS, although other pre-existing, nonhormonal differences between the two TS subgroups may alternatively explain these findings, given our study design. Future studies are needed to examine potential impacts of ERT on cognitive and social-emotional development in TS.
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Aspects of care in individuals with TS that pose barriers to transition unique from other chronic conditions include lower visuospatial processing, lower self-esteem, and difficulties with executive functioning.
“There are aspects of care in individuals with TS that pose barriers to transition that are unique from other chronic conditions, such as lower visuospatial processing and self-esteem, and difficulties with executive functioning.367”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [367]
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Research works printed but not linked: 1
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Clinical practice guidelines for the care of girls and women with Turner syndrome.
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Self-citation
- Authors
- Jordan, T. L., Klabunde, M., Green, T., Hong, D. S., Ross, J. L., Jo, B., & Reiss, A. L.
- Affiliations
- Stanford Medicine (US)
- University of Essex (GB)
- Alfred I. duPont Hospital for Children (US)
- Corresponding author
- Reiss, Allan L.
- ORCID
- Venue
- Hormones and Behavior
- Publisher
- Elsevier BV
- Published
- 2023-03
- Volume
- 149
- Pages
- 105300
- ISSN
- 1556-5068 (ISSN-L)
- 0018-506X
- PMC
- PMC9974892
- OpenAlex
- W4316340218
- Cited by
- 11
- References
- 49
- Citations by year
- 2026: 5 · 2025: 1 · 2024: 5
- Type
- posted content
- Language
- en
- Open access
- green
- doi.org
- Licence
- www.elsevier.com/tdm/userlicense/1.0/ (tdm)
- www.elsevier.com/legal/tdmrep-license (tdm)
- www.elsevier.com/open-access/userlicense/1.0/ (am, 317 d)
- doi.org/10.15223/policy-017 (stm-asf)
- doi.org/10.15223/policy-037 (stm-asf)
- doi.org/10.15223/policy-012 (stm-asf)
- doi.org/10.15223/policy-029 (stm-asf)
- doi.org/10.15223/policy-004 (stm-asf)
- Locations
- SSRN Electronic Journal (publishedVersion)
- Hormones and Behavior (acceptedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Open Access at Essex (University of Essex) (acceptedVersion)
- Funders
- National Institutes of Health
- Alliance for Lupus Research
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
- Keywords
- Turner syndrome, visuospatial processing, executive function, social cognition, social competence, anxiety, estrogen replacement therapy, cognitive development, social-emotional development, childhood and adolescence
- MeSH terms
- Social Cognition, Adolescent, Child, Cognition, Female, Humans, Turner Syndrome, Executive Function, Social Skills
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