Social responsiveness and competence in Prader–Willi syndrome: direct comparison to autism spectrum disorder
Abstract
Prader-Willi syndrome (PWS), a neurodevelopmental disorder primarily characterized by hyperphagia and food preoccupations, is caused by the absence of expression of the paternally active genes in the proximal arm of chromosome 15. Although maladaptive behavior and the cognitive profile in PWS have been well characterized, social functioning has only more recently been systematically examined. Findings to date indicate the social impairment exhibited may reflect specific difficulty interpreting and using social information effectively. In addition, evidence suggests that there is an increased risk of social deficits in people with the maternally-derived uniparental disomy (mUPD) subtype of PWS in comparison to those with 15q11-13 paternal deletion (DEL). Using the Social Responsiveness Scale (SRS) and the Social Competence Inventory, our goal was to compare social functioning in PWS to individuals with autism spectrum disorder (ASD). Participants with mUPD scored similarly to the ASD group across most SRS domains. All groups had difficulty with social competence, although the DEL group scored highest on prosocial behavior. Findings suggest further characterization of social behavior in PWS is necessary to aid in advancing the understanding of the contributions of genes in the 15q11-13 critical region to ASD susceptibility, particularly with respect to the overexpression of maternally expressed genes in this region, as well as aiding in awareness and development/implementation of interventions.
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As in ASD, individuals with Prader-Willi syndrome have difficulty with social competence, are aloof and avoid eye contact.
“For example, as in ASD, individuals with PWS have difficulty with social competence (Dimitropoulos et al., 2013), are aloof and avoid eye contact (Dimitropoulos et al., 2009).”
From Oxytocin and vasopressin systems in genetic syndromes and neurodevelopmental disorders (2014) · cites this paper as Dimitropoulos et al., 2013
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A cross-comparison of cognitive ability across 8 genomic disorders
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Study of the deficit in planning abilities of adults with Prader-Willi Syndrome
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Creative Play in Children with Neurodevelopmental Disorders
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Self-citationBehavioral Testing Design for Evaluation of Cognitive Disabilities
Riet, F., Mittelhaeuser, C., Lux, A., Bour, R., Selloum, M., Sorg, T., Hérault, Y., & Méziane, H. (2022) · Current Protocols · doi:10.1002/cpz1.382 · Cited by 3 · see citing works
Effects of Cognition and Age on Assessment of Autism and Pervasive Developmental Disabilities
Thurm, A. E., Bishop, S. L., & Giserman‐Kiss, I. (2022) · Autism and child psychopathology series · doi:10.1007/978-3-030-88538-0_12 · Cited by 3 · see citing works
Martínez, S. G., Cobo, J., Fernández-Lafitte, M., Coronas, R., Parra, I., Oliva, J. C., Pedrero, A. Á., Esteba‐Castillo, S., Giménez‐Palop, O., Corripio, R., et al. (2023) · Journal of Clinical Medicine · doi:10.3390/jcm12031155 · Cited by 3 · see citing works
Genetic Disorders and Dual Diagnosis: Building Clinical Management on Etiology and Neurocognition
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Rehabilitación neuropsicológica en personas con síndrome de Prader-Willi
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- Authors
- Dimitropoulos, A., Ho, A., & Feldman, B.
- Affiliations
- Case Western Reserve University (US)
- Corresponding author
- Dimitropoulos, Anastasia
- Venue
- Journal of Autism and Developmental Disorders
- Published
- 2012-05-11
- Volume
- 43
- Issue
- 1
- Pages
- 103-113
- ISSN
- 0162-3257 (ISSN-L)
- 1573-3432
- OpenAlex
- W2052941943
- Cited by
- 77
- References
- 63
- Citations by year
- 2026: 2 · 2025: 2 · 2024: 7 · 2023: 8 · 2022: 10 · 2021: 9 · 2020: 4 · 2019: 6 · 2018: 6 · 2017: 5 · 2016: 4 · 2015: 6 · 2014: 5 · 2013: 2 · 2012: 1
- FWCI
- 2.68
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Journal of Autism and Developmental Disorders (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic Syndromes and Imprinting
- Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
- Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
- Keywords
- Prader-Willi syndrome, autism spectrum disorder, social responsiveness, social competence, 15q11-13, uniparental disomy, paternal deletion, Social Responsiveness Scale, prosocial behavior, social dysfunction, maternally expressed genes
- MeSH terms
- Adolescent, Adult, Child, Child Development Disorders, Pervasive, Female, Humans, Interpersonal Relations, Male, Prader-Willi Syndrome, Psychological Tests, Severity of Illness Index, Social Adjustment, Social Behavior, Young Adult
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