This study investigated the influence of maternal and child variables on the maternal responsivity of 55 mothers with young children with fragile X syndrome. Data included video observations of mother-child interactions in four different contexts, standardized assessments with the children, and standardized questionnaires for the mothers. The…

Children with 22q11.2 deletion syndrome exhibit high rates of social-behavioral problems, particularly in the internalizing domain, indicating an area in need of intervention. The current investigation was designed to obtain information regarding parent and teacher ratings of the social-emotional behavior of children with 22q11DS. Using the Child…

This methodological review draws attention to the challenges faced by intellectual and developmental disabilities researchers in the appropriate design and analysis of group comparison studies. We provide a brief overview of matching methodologies in the field, emphasizing group-matching designs used in behavioral research on cognition and…

This study examined how children and adolescents with Williams syndrome (WS; ages 8 years, 0 months [8;0]-14;5) used referential devices (determiners and pronouns), tense, and connectives to create cohesion in oral narratives based on a storybook compared to typically developing mentally and chronologically age-matched children. WS children used…

This study compared the receptive and expressive language profiles of verbally expressive children and adolescents with Down Syndrome (DS) and those with Fragile X syndrome (FXS) and examined the extent to which these profiles reliably differentiate the diagnostic groups. A total of twenty-four verbal participants with DS (mean age: 12 years),…

This study investigated the development of novel metaphor and metonymy comprehension in both typically developing (TD) children and individuals with Williams syndrome (WS). Thirty-one TD children between the ages of 3;09 and 17;01 and thirty-four individuals with WS between the ages of 7;01 and 44 years old were administered a newly developed task…

Background: Individuals with Prader-Willi syndrome (PWS) have been shown to demonstrate a particular cognitive deficit in attention switching and high levels of preference for routine and temper outbursts. This study assesses whether a specific pathway between a cognitive deficit and behaviour via environmental interaction can exist in individuals…

Background: Individuals with Angelman syndrome appear strongly motivated by social contact, but there have been few studies that have examined the relationship between sociability and familiarity. In this study we compared social behaviour in Angelman syndrome when in contact with mothers and strangers. Methods: We systematically manipulated adult…

Background: The current study focuses on mothers and fathers of children with three rare genetic syndromes that are relatively unexplored in terms of family experience: Angelman syndrome, Cornelia de Lange syndrome and Cri du Chat syndrome. Method: Parents of children with Angelman syndrome (n = 15), Cornelia de Lange syndrome (n = 16) and Cri du…

RSK2 is a Ser/Thr kinase acting in the Ras/MAPK pathway. "Rsk2" gene deficiency leads to the Coffin-Lowry Syndrome, notably characterized by cognitive deficits. We found that "mrsk2" knockout mice are unable to associate an aversive stimulus with context in a lithium-induced conditioned place aversion task requiring both high-order cognition and…

To assess whether there are qualitatively deviant characteristics in the early vocalizations of children with Rett syndrome, we had 400 native Austrian-German speakers listen to audio recordings of vocalizations from typically developing girls and girls with Rett syndrome. The audio recordings were rated as (a) inconspicuous, (b) conspicuous or…

Experience engenders learning, but not all learning involves representational change. In this paper, we provide a dramatic case study of the distinction between learning and representational change. Specifically, we examined long- and short-term changes in representations of numeric magnitudes by asking individuals with Williams syndrome (WS) and…

The prevalence of sleep problems in individuals with intellectual disability (ID) seems to vary between genetic syndromes associated with ID. Different types of sleep disturbances may indicate underlying causes of sleep problems and these types of sleep disturbances may vary between different genetic syndromes. We examined and compared five types…

Science teachers are aware of many social issues that intersect with science. These socio-scientific issues (SSIs) are "open-ended problems without clear-cut solutions [that] can be informed by scientific principles, theories, and data, but…cannot be fully determined by [them]" (Sadler 2011, p. 4). This article describes the SSI lessons…

Fragile X syndrome (FXS) is a well-described inherited cause of intellectual disability and the most common known genetic cause of autism. Social deficits in girls with FXS are not well understood. To better understand barriers to social functioning that may contribute to mental health outcomes, we administered a theoretically based social…