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Direct linkEllis, Katherine; Oliver, Chris; Stefanidou, Chrysi; Apperly, Ian; Moss, Jo – Journal of Autism and Developmental Disorders, 2020
We directly assessed the broader aspects of sociability (social enjoyment, social motivation, social interaction skills and social discomfort) in individuals with Cornelia de Lange (CdLS), fragile X (FXS) and Rubinstein-Taybi syndromes (RTS), and their association with autism characteristics and chronological age in these groups. Individuals with…
Descriptors: Interpersonal Competence, Motivation, Neurological Impairments, Genetic Disorders
Raspa, Melissa; Bann, Carla M.; Gwaltney, Angela; Benke, Timothy A.; Fu, Cary; Glaze, Daniel G.; Haas, Richard; Heydemann, Peter; Jones, Mary; Kaufmann, Walter E.; Lieberman, David; Marsh, Eric; Peters, Sarika; Ryther, Robin; Standridge, Shannon; Skinner, Steven A.; Percy, Alan K.; Neul, Jeffrey L. – American Journal on Intellectual and Developmental Disabilities, 2020
Rett syndrome (RTT) is a neurodevelopmental disorder that primarily affects females. Recent work indicates the potential for disease modifying therapies. However, there remains a need to develop outcome measures for use in clinical trials. Using data from a natural history study (n = 1,075), we examined the factor structure, internal consistency,…
Descriptors: Genetic Disorders, Psychometrics, Psychomotor Skills, Physical Disabilities
Brawn, Gabrielle; Porter, Melanie – International Journal of Disability, Development and Education, 2018
Literature on the level of adaptive functioning and relative strengths and weaknesses in functioning of individuals with Williams syndrome (WS) was reviewed. The electronic databases PsycINFO, PubMed, Expanded Academic, Web of Science, Scopus and ProQuest were searched electronically for relevant articles and dissertations using the search terms…
Descriptors: Literature Reviews, Adjustment (to Environment), Congenital Impairments, Genetic Disorders
Klein-Tasman, Bonita P.; Lira, Ernesto N.; Li-Barber, Kirsten T.; Gallo, Frank J.; Brei, Natalie G. – American Journal on Intellectual and Developmental Disabilities, 2015
Problem behavior of 52 children with Williams syndrome ages 6 to 17 years old was examined based on both parent and teacher report. Generally good inter-rater agreement was found. Common areas of problem behavior based both on parent and teacher report included attention problems, anxiety difficulties, repetitive behaviors (e.g., obsessions,…
Descriptors: Behavior Problems, Genetic Disorders, Children, Adolescents
Hartley, Sigan L.; Wheeler, Anne C.; Mailick, Marsha R.; Raspa, Melissa; Mihaila, Iulia; Bishop, Ellen; Bailey, Donald B. – Journal of Autism and Developmental Disorders, 2015
A cross-sectional analysis was used to examine age-related differences in ASD symptoms and corresponding differences in disruptive behavior and social skills in 281 adult men with fragile X syndrome. Four age groups were created: 18-21, 22-29, 30-39, and 40-49 years. The 18-21 year-old group was reported to have more impairments in verbal…
Descriptors: Autism, Pervasive Developmental Disorders, Adults, Males
Adams, Dawn; Horsler, Kate; Mount, Rebecca; Oliver, Chris – Journal of Autism and Developmental Disorders, 2015
Elevated laughing and smiling is a key characteristic of the Angelman syndrome behavioral phenotype, with cross-sectional studies reporting changes with environment and age. This study compares levels of laughing and smiling in 12 participants across three experimental conditions [full social interaction (with eye contact), social interaction with…
Descriptors: Genetic Disorders, Mental Retardation, Affective Behavior, Nonverbal Communication
Download full textBruce, Susan M.; Zatta, Mary C.; Gavin, Mary; Stelzer, Sharon – Journal of Visual Impairment & Blindness, 2016
Introduction: Deafblindness limits access to social cues and social feedback, thus restricting the development of social skills. Many children with CHARGE syndrome, a leading cause of deafblindness, experience challenges with emotional self-regulation and anxiety that may interfere with socialization. Learning about self-determination skills such…
Descriptors: Socialization, Self Determination, Deaf Blind, Interpersonal Competence
Kaufmann, W. E.; Tierney, E.; Rohde, C. A.; Suarez-Pedraza, M. C.; Clarke, M. A.; Salorio, C. F.; Bibat, G.; Bukelis, I.; Naram, D.; Lanham, D. C.; Naidu, S. – Journal of Intellectual Disability Research, 2012
Background: While behavioural abnormalities are fundamental features of Rett syndrome (RTT), few studies have examined the RTT behavioural phenotype. Most of these reports have focused on autistic features, linked to the early regressive phase of the disorder, and few studies have applied standardised behavioural measures. We used a battery of…
Descriptors: Interaction, Factor Analysis, Severity (of Disability), Interpersonal Competence
Elison, Sarah; Stinton, Chris; Howlin, Patricia – Research in Developmental Disabilities: A Multidisciplinary Journal, 2010
Previous studies have investigated trajectories of cognitive, language and adaptive functioning in Williams syndrome (WS) but little is known about how other aspects of the Williams syndrome behavioural phenotype change across the life-span. Therefore, the present study examined age associated changes in a number of different domains of…
Descriptors: Physical Health, Interviews, Genetic Disorders, Aging (Individuals)
McDuffie, Andrea; Abbeduto, Leonard; Lewis, Pamela; Kover, Sara; Kim, Jee-Seon; Weber, Ann; Brown, W. Ted – American Journal on Intellectual and Developmental Disabilities, 2010
The Autism Diagnostic Interview-Revised (ADI-R) was used to examine diagnostic profiles and age-related changes in autism symptoms for a group of verbal children and adolescents who had fragile X syndrome, with and without autism. After controlling for nonverbal IQ, we found statistically significant between-group differences for lifetime and…
Descriptors: Autism, Interests, Intelligence Quotient, Interaction
Dimitropoulos, Anastasia; Ho, Alan Y.; Klaiman, Cheryl; Koenig, Kathy; Schultz, Robert T. – Journal of Mental Health Research in Intellectual Disabilities, 2009
In order to investigate unique and shared characteristics and to determine factors predictive of group classification, quantitative comparisons of behavioral and emotional problems were assessed using the Developmental Behavior Checklist (DBC-P) and the Vineland Adaptive Behavior Scales in autistic disorder, Williams syndrome (WS), and…
Descriptors: Check Lists, Emotional Problems, Behavior Problems, Mental Retardation
Rosner, Beth A.; Hodapp, Robert M.; Fidler, Deborah J.; Sagun, Jaclyn N.; Dykens, Elisabeth M. – Journal of Applied Research in Intellectual Disabilities, 2004
Background: This study explores everyday social competence in the lives of persons with three genetic intellectual disability syndromes. Methods: Using parent reports on the Social and Activity Competence domains of Achenbach's Child Behavior Checklist (CBCL), socially competent behaviours were examined in 58 persons with Williams syndrome, 54…
Descriptors: Check Lists, Mental Retardation, Child Behavior, Interpersonal Competence
