Cross-sectional and longitudinal assessment of cognitive development in Williams syndrome.

Williams syndrome (WS) is a rare genetic syndrome. As with all rare syndromes, obtaining adequately powered sample sizes is a challenge. Here we present legacy data from seven UK labs, enabling the characterisation of cross-sectional and longitudinal developmental trajectories of verbal and non-verbal development in the largest sample of individuals with WS to-date.

The role of context in verbal humor processing in autism.

Difficulties in processing humor have been associated with individuals with autism. The current study investigated whether humor comprehension and appreciation could be augmented in children with autism by providing contextual support suggesting that humor was to be expected. A verbally presented riddle task was used in which participants were assessed for their subjective ratings and comprehension of the materials.

The development of children's comprehension and appreciation of riddles.

Humor appreciation and understanding is important for children's social relationships. The current study examined the associations among riddle comprehension, riddle appreciation, and smiling/laughter in children from a wide age range (4-11 years) as well as how cognitive processing style relates to riddle comprehension. Style was distinguished between local and global language processing at the sentence level.

Déjà Vécu and Déjà Visité: Similarities and Differences: Further Results From an Online Questionnaire.

Over an 8-year period, an online questionnaire, devised as a pilot project, collected exploratory data in order to compare the particularities of déjà vécu (i.e., event related) experiences with those of déjà visité (i.

Route knowledge and configural knowledge in typical and atypical development: a comparison of sparse and rich environments.

Background: Individuals with Down syndrome (DS) and individuals with Williams syndrome (WS) have poor navigation skills, which impact their potential to become independent. Two aspects of navigation were investigated in these groups, using virtual environments (VE): route knowledge (the ability to learn the way from A to B by following a fixed sequence of turns) and configural knowledge (knowledge of the spatial relationships between places within an environment).

Methods: Typically developing (TD) children aged 5 to 11 years (N = 93), individuals with DS (N = 29) and individuals with WS (N = 20) were presented with a sparse and a rich VE grid maze.

Improving working memory abilities in individuals with Down syndrome: a treatment case study.

Working memory (WM) skills of individuals with Down's syndrome (DS) tend to be very poor compared to typically developing children of similar mental age. In particular, research has found that in individuals with DS visuo-spatial WM is better preserved than verbal WM. This study investigated whether it is possible to train short-term memory (STM) and WM abilities in individuals with DS.

The development of route learning in Down syndrome, Williams syndrome and typical development: investigations with virtual environments.

The ability to navigate new environments has a significant impact on the daily life and independence of people with learning difficulties. The aims of this study were to investigate the development of route learning in Down syndrome (N = 50), Williams syndrome (N = 19), and typically developing children between 5 and 11 years old (N = 108); to investigate use of landmarks; and to relate cognitive functions to route-learning ability in these groups. Overall, measures of attention and long-term memory were strongly associated with route learning, even once non-verbal ability was controlled for.

Poor phonemic discrimination does not underlie poor verbal short-term memory in Down syndrome.

Individuals with Down syndrome tend to have a marked impairment of verbal short-term memory. The chief aim of this study was to investigate whether phonemic discrimination contributes to this deficit. The secondary aim was to investigate whether phonological representations are degraded in verbal short-term memory in people with Down syndrome relative to control participants.

Short-term memory, executive control, and children's route learning.

The aim of this study was to investigate route-learning ability in 67 children aged 5 to 11years and to relate route-learning performance to the components of Baddeley's model of working memory. Children carried out tasks that included measures of verbal and visuospatial short-term memory and executive control and also measures of verbal and visuospatial long-term memory; the route-learning task was conducted using a maze in a virtual environment. In contrast to previous research, correlations were found between both visuospatial and verbal memory tasks-the Corsi task, short-term pattern span, digit span, and visuospatial long-term memory-and route-learning performance.

Are imaging and lesioning convergent methods for assessing functional specialisation?: investigations using an artificial neural network.

This article presents an investigation of the relationship between lesioning and neuroimaging methods of assessing functional specialisation, using synthetic brain imaging (SBI) and lesioning of a connectionist network of past-tense formation. The model comprised two processing 'routes': one was a direct route between layers of input and output units, while the other, indirect, route featured an intermediate layer of processing units. Emergent specialisation within the network was assessed (1) by lesioning either the direct or indirect route and measuring past-tense performance for regular and irregular verbs, and (2) by measuring functional activation in each route when processing each verb type (SBI).

Definitions versus categorization: assessing the development of lexico-semantic knowledge in Williams syndrome.

Background: Williams syndrome (WS) is associated with relatively strong language abilities despite mild to moderate intellectual disability, particularly when language is indexed by vocabulary.

Aims: The aim of the study was twofold: (1) to investigate whether reported lexical anomalies in WS can be explained with reference to anomalous semantic development; and (2) to assess whether receptive vocabulary skills in WS, a relative strength, are underpinned by commensurate semantic knowledge.

Methods & Procedures: The development of lexical-semantic knowledge was investigated in 45 typically developing individuals (chronological age range = 5-10 years, mental age range = 5-13 years) and 15 individuals with WS (chronological age range = 12-50 years, mental age range = 4-17 years) by means of (1) a categorization task and (2) a definitions task, which was expected to make additional metacognitive demands.

The development of metaphorical language comprehension in typical development and in Williams syndrome.

The domain of figurative language comprehension was used to probe the developmental relation between language and cognition in typically developing individuals and individuals with Williams syndrome. Extending the work of Vosniadou and Ortony, the emergence of nonliteral similarity and category knowledge was investigated in 117 typically developing children between 4 and 12 years of age, 19 typically developing adults, 15 children with Williams syndrome between 5 and 12 years of age, and 8 adults with Williams syndrome. Participants were required to complete similarity and categorization statements by selecting one of two words (e.

Impaired verbal short-term memory in Down syndrome reflects a capacity limitation rather than atypically rapid forgetting.

Individuals with Down syndrome suffer from relatively poor verbal short-term memory. Recent work has indicated that this deficit is not caused by problems of audition, speech, or articulatory rehearsal within the phonological loop component of Baddeley and Hitch's working memory model. Given this, two experiments were conducted to investigate whether abnormally rapid decay underlies the deficit.