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Will, Elizabeth A.; Roberts, Jane E. – American Journal on Intellectual and Developmental Disabilities, 2021
Motor skills, an important foundation for language and communication, are considerably delayed in children with Down syndrome (DS) and fragile X syndrome (FXS). However, the impact of these impairments on expressive and receptive communication and the phenotypic specificity of these associations remains unknown. Participants included 37 with DS…
Descriptors: Psychomotor Skills, Down Syndrome, Genetic Disorders, Children
Block, Elisa; Farran, Emily K.; Van Herwegen, Jo – American Journal on Intellectual and Developmental Disabilities, 2022
The block design task (BDT) is a visuospatial measure that individuals with Williams syndrome (WS) perform poorly on. However, it is unclear what underlies their impaired performance. This study investigated whether poorer performance is a result of visuospatial difficulties, executive function (EF) difficulties, atypical looking strategies, or a…
Descriptors: Task Analysis, Visual Perception, Spatial Ability, Executive Function
Robinson, Marissa; Klusek, Jessica; Poe, Michele D.; Hatton, Deborah D.; Roberts, Jane E. – American Journal on Intellectual and Developmental Disabilities, 2018
Effortful control, or the ability to suppress a dominant response to perform a subdominant response, is an early-emerging temperament trait that is linked with positive social-emotional development. Fragile X syndrome (FXS) is a single-gene disorder characterized by hallmark regulatory impairments, suggesting diminished effortful control. This…
Descriptors: Genetic Disorders, Congenital Impairments, Intellectual Disability, Males
Mason, Gina Marie; Spanó, Goffredina; Edgin, Jamie – American Journal on Intellectual and Developmental Disabilities, 2015
This study examined individual differences in ADHD symptoms and executive function (EF) in children with Down syndrome (DS) in relation to the dopamine receptor D4 (DRD4) gene, a gene often linked to ADHD in people without DS. Participants included 68 individuals with DS (7-21 years), assessed through laboratory tasks, caregiver reports, and…
Descriptors: Symptoms (Individual Disorders), Attention Deficit Hyperactivity Disorder, Individual Differences, Genetics
Kover, Sara T.; McCary, Lindsay M.; Ingram, Alexandra M.; Hatton, Deborah D.; Roberts, Jane E. – American Journal on Intellectual and Developmental Disabilities, 2015
Fragile X syndrome (FXS) is associated with significant language and communication delays, as well as problems with attention. This study investigated early language abilities in infants and toddlers with FXS (n = 13) and considered visual attention as a predictor of those skills. We found that language abilities increased over the study period of…
Descriptors: Genetic Disorders, Infants, Toddlers, Language Acquisition
Chevalère, Johann; Postal, Virginie; Jauregui, Joseba; Copet, Pierre; Laurier, Virginie; Thuilleaux, Denise – American Journal on Intellectual and Developmental Disabilities, 2015
The aim of this study was to support the growing evidence suggesting that Prader-Willi Syndrome (PWS) might present with an impairment of executive functions (EFs) and to investigate whether this impairment is specific to patients with PWS or due to their intellectual disability (ID). Six tasks were administered to assess EFs (inhibition,…
Descriptors: Genetic Disorders, Disabilities, Executive Function, Neurological Impairments
Giersch, Anne; Glaser, Bronwyn; Pasca, Catherine; Chabloz, Mélanie; Debbané, Martin; Eliez, Stephan – American Journal on Intellectual and Developmental Disabilities, 2014
Individuals with 22q11.2 deletion syndrome (22q11.2DS) are impaired at exploring visual information in space; however, not much is known about visual form discrimination in the syndrome. Thirty-five individuals with 22q11.2DS and 41 controls completed a form discrimination task with global forms made up of local elements. Affected individuals…
Descriptors: Genetic Disorders, Cognitive Ability, Visual Perception, Visual Discrimination
Lanfranchi, Silvia; De Mori, Letizia; Mammarella, Irene C.; Carretti, Barbara; Vianello, Renzo – American Journal on Intellectual and Developmental Disabilities, 2015
The aim of the present study was to compare visuospatial working memory performance in 18 individuals with Williams syndrome (WS) and 18 typically developing (TD) children matched for nonverbal mental age. Two aspects were considered: task presentation format (i.e., spatial-sequential or spatial-simultaneous), and level of attentional control…
Descriptors: Genetic Disorders, Disabilities, Visual Perception, Spatial Ability
Hahn, Laura J.; Fidler, Deborah J.; Hepburn, Susan L. – American Journal on Intellectual and Developmental Disabilities, 2014
The present study compares the adaptive behavior profile of 18 young children with Williams syndrome (WS) and a developmentally matched group of 19 children with developmental disabilities and examines the relationship between adaptive behavior and problem behaviors in WS. Parents completed the Vineland Adaptive Behavioral Scales--Interview…
Descriptors: Genetic Disorders, Behavior Problems, Young Children, Comparative Analysis
Turkstra, Lyn S.; Abbeduto, Leonard; Meulenbroek, Peter – American Journal on Intellectual and Developmental Disabilities, 2014
This study aimed to characterize social cognition, executive functions (EFs), and everyday social functioning in adolescent girls with fragile X syndrome, and identify relationships among these variables. Participants were 20 girls with FXS and 20 age-matched typically developing peers. Results showed significant between-groups differences in…
Descriptors: Executive Function, Social Cognition, Interpersonal Competence, Females
Nelson, Lisa; Moss, Jo; Oliver, Chris – American Journal on Intellectual and Developmental Disabilities, 2014
Studies of individuals with Cornelia de Lange syndrome (CdLS) have described changes in mood and behavior with age, although no empirical or longitudinal studies have been conducted. Caregivers of individuals with CdLS (N = 67), cri du chat syndrome (CdCS; N = 42), and Fragile X syndrome (FXS; N = 142) completed the Mood, Interest and Pleasure…
Descriptors: Longitudinal Studies, Followup Studies, Children, Adults
Tonnsen, Bridgette L.; Shinkareva, Svetlana V.; Deal, Sara C.; Hatton, Deborah D.; Roberts, Jane E. – American Journal on Intellectual and Developmental Disabilities, 2013
Anxiety is among the most impairing conditions associated with Fragile X syndrome (FXS) and is putatively linked to atypical physiological arousal. However, few studies have examined this association in young children with FXS. The authors examined whether patterns of arousal and behavior during an experimental stranger approach paradigm differ…
Descriptors: Anxiety, Genetic Disorders, Physiology, Arousal Patterns
Lense, Miriam Diane; Tomarken, Andrew J.; Dykens, Elisabeth M. – American Journal on Intellectual and Developmental Disabilities, 2013
Williams syndrome (WS) is a neurodevelopmental genetic disorder associated with high rates of anxiety and social issues. We examined diurnal cortisol, a biomarker of the stress response, in adults with WS in novel and familiar settings, and compared these profiles to typically developing (TD) adults. WS and TD participants had similar profiles in…
Descriptors: Novelty (Stimulus Dimension), Metabolism, Familiarity, Genetic Disorders
Hogan-Brown, Abigail L.; Losh, Molly; Martin, Gary E.; Mueffelmann, Deborah J. – American Journal on Intellectual and Developmental Disabilities, 2013
Whereas pragmatic language difficulties are characteristic of both autism and Fragile X syndrome, it is unclear whether such deficits are qualitatively similar or whether certain skills are differentially affected. This study compared narrative competence in boys with autism, Fragile X syndrome, Down syndrome, and typical development. Results…
Descriptors: Males, Story Telling, Autism, Pervasive Developmental Disorders
Klusek, Jessica; Martin, Gary E.; Losh, Molly – American Journal on Intellectual and Developmental Disabilities, 2013
This study tested the hypothesis that pragmatic (i.e., social) language impairment is linked to arousal dysregulation in autism spectrum disorder (ASD) and fragile X syndrome (FXS). Forty boys with ASD, 39 with FXS, and 27 with typical development (TD), aged 4-15 years, participated. Boys with FXS were hyperaroused compared to boys with TD but did…
Descriptors: Physiology, Genetic Disorders, Pervasive Developmental Disorders, Autism
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