Kellie Gergoudis1, Alan Weinberg2, Jonathan Templin3, Cristan Farmer4, Alison Durkin5, Jordana Weissman5, Paige Siper5, Jennifer Foss-Feig5, Maria Del Pilar Trelles5, Jonathan A Bernstein6, Joseph D Buxbaum5,7,8, Elizabeth Berry-Kravis9,10,11, Craig M Powell12,13, Mustafa Sahin14,15, Latha Soorya16, Audrey Thurm4, Alexander Kolevzon5,17. 1. Icahn School of Medicine at Mount Sinai, New York, New York, USA. 2. Department of Population Health Science and Policy, Icahn School of Medicine at Mount Sinai, New York, New York, USA. 3. Department of Psychology and Research in Education, University of Kansas, Lawrence, Kansas, USA. 4. Pediatrics and Developmental Neuroscience Branch, National Institute of Mental Health, National Institutes of Health, Bethesda, Maryland, USA. 5. Seaver Autism Center, Department of Psychiatry, Icahn School of Medicine at Mount Sinai, New York, New York, USA. 6. Department of Pediatrics, Stanford University School of Medicine, Stanford, California, USA. 7. Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York, USA. 8. Department of Neuroscience, Icahn School of Medicine at Mount Sinai, New York, New York, USA. 9. Department of Pediatrics, Rush University Medical Center, Chicago, Illinois, USA. 10. Department of Neurological Sciences, Rush University Medical Center, Chicago, Illinois, USA. 11. Department of Biochemistry, Rush University Medical Center, Chicago, Illinois, USA. 12. Department of Neurobiology, University of Alabama at Birmingham, Birmingham, Alabama, USA. 13. Civitan International Research Center, University of Alabama at Birmingham, Birmingham, Alabama, USA. 14. F.M. Kirby Neurobiology Center, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. 15. Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. 16. Department of Psychiatry, Rush University Medical Center, Chicago, Illinois, USA. 17. Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
Abstract
The Social Responsiveness Scale-2 (SRS-2) is a quantitative measure used to characterize symptoms of autism spectrum disorder (ASD). However, research suggests that SRS-2 scores are significantly influenced by language ability and intellectual disability (ID). Efforts to refine the SRS-2 by Sturm, Kuhfeld, Kasari, and Mccracken [Journal of Child Psychology and Psychiatry, 58(9), 1053-1061] yielded a shortened form, yet its psychometric properties in populations with severe ID remain unknown. This study aims to examine the psychometric properties of the SRS-2 in Phelan-McDermid syndrome (PMS), a genetic condition associated with ASD and ID, thereby guiding score interpretation in this population and future development of targeted scales. Analyses, including Item Response Theory (IRT), were conducted on a sample of individuals with PMS (n = 91) recruited at six sites nationally. Psychometric properties evaluated include measures of reliability (internal consistency, test-retest reliability) and validity (structural, construct, content). While both SRS-2 forms are reliable, the shortened SRS-2 shows superior validity to the full SRS-2 for measuring ASD symptoms in PMS. On IRT analysis, the shortened SRS-2 shows excellent discrimination and precisely evaluates respondents across a wide range of ASD symptomatology but interpretation is limited by uncertain content validity and small sample size. The shortened SRS-2 shows some promise for use in PMS, but future refinements and additions are needed to develop items that are tailored to identify ASD in children with severe ID and specifically PMS. LAY SUMMARY: This study determined that a shortened form of the Social Responsiveness Scale, Second Edition (SRS-2) shows both promise and limitations for the characterization of autism symptomatology in individuals with Phelan-McDermid syndrome (PMS), a population characterized by intellectual disability (ID). Caution should be used when interpreting SRS-2 scores in individuals with ID and future research should modify existing items and develop new items to improve the SRS-2's ability to accurately characterize autism symptomatology in PMS. Autism Res 2020, 13: 1383-1396.
The Social Responsiveness Scale-2 (SRS-2) is a quantitative measure used to characterize symptoms of autism spectrum disorder (ASD). However, research suggests that SRS-2 scores are significantly influenced by language ability and intellectual disability (ID). Efforts to refine the SRS-2 by Sturm, Kuhfeld, Kasari, and Mccracken [Journal of Child Psychology and Psychiatry, 58(9), 1053-1061] yielded a shortened form, yet its psychometric properties in populations with severe ID remain unknown. This study aims to examine the psychometric properties of the SRS-2 in Phelan-McDermid syndrome (PMS), a genetic condition associated with ASD and ID, thereby guiding score interpretation in this population and future development of targeted scales. Analyses, including Item Response Theory (IRT), were conducted on a sample of individuals with PMS (n = 91) recruited at six sites nationally. Psychometric properties evaluated include measures of reliability (internal consistency, test-retest reliability) and validity (structural, construct, content). While both SRS-2 forms are reliable, the shortened SRS-2 shows superior validity to the full SRS-2 for measuring ASD symptoms in PMS. On IRT analysis, the shortened SRS-2 shows excellent discrimination and precisely evaluates respondents across a wide range of ASD symptomatology but interpretation is limited by uncertain content validity and small sample size. The shortened SRS-2 shows some promise for use in PMS, but future refinements and additions are needed to develop items that are tailored to identify ASD in children with severe ID and specifically PMS. LAY SUMMARY: This study determined that a shortened form of the Social Responsiveness Scale, Second Edition (SRS-2) shows both promise and limitations for the characterization of autism symptomatology in individuals with Phelan-McDermid syndrome (PMS), a population characterized by intellectual disability (ID). Caution should be used when interpreting SRS-2 scores in individuals with ID and future research should modify existing items and develop new items to improve the SRS-2's ability to accurately characterize autism symptomatology in PMS. Autism Res 2020, 13: 1383-1396.
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