Attention and working memory are fundamental for selecting and maintaining behaviorally relevant information. Not only do both processes closely intertwine at the cognitive level, but they implicate similar functional brain circuitries, namely the frontoparietal and the frontostriatal networks, which are innervated by cholinergic and dopaminergic…

Fragile X syndrome is the most common cause of inherited intellectual impairment and the most common single-gene cause of autism. Individuals with fragile X syndrome present with a neurobehavioural phenotype that includes selective deficits in spatiotemporal visual perception associated with neural processing in frontal-parietal networks of the…

The collection of articles in this special issue and related studies over the past decade provides a fine example of the substantial progress that has been made in our understanding and remediation of mathematical learning disabilities and difficulties since 1993 (Geary, 1993). The originally proposed procedural and retrieval deficits have been…

Glucose transporter type 1 deficiency syndrome (GLUT1-DS) is caused by a defect in glucose transport across the blood-brain barrier. The main symptoms are epilepsy, developmental delay, movement disorders, and deceleration of head circumference. A ketogenic diet has been shown to be effective in controlling epilepsy in GLUT1-DS. We report a female…

Williams syndrome (WS) is a neurodevelopmental genetic disorder often described as being characterized by a dissociative cognitive architecture, in which profound impairments of visuo-spatial cognition contrast with relative preservation of linguistic, face recognition and auditory short-memory abilities. This asymmetric and dissociative cognition…

Background: In addition to their more clinically evident abnormalities of social cognition, people with autism spectrum conditions (ASC) manifest perturbations of attention and sensory perception which may offer insights into the underlying neural abnormalities. Similar autistic traits in ASC relatives without a diagnosis suggest a continuity…

Some individuals with Prader-Willi syndrome exhibit strengths in solving jigsaw puzzles. We compared visuospatial ability and jigsaw puzzle performance and strategies of 26 persons with Prader-Willi syndrome and 26 MA-matched typically developing controls. Individuals with Prader-Willi syndrome relied on piece shape. Those in the control group…

Background: The 22q11.2 deletion syndrome (22qDS) confers high risk for intellectual disability and neuropsychological/academic impairment, although a minority of patients show average intelligence. Intellectual heterogeneity and the high prevalence of psychiatric diagnoses in earlier studies may have obscured the prototypical neuropsychological…

Empirical data suggest that there is at most a very small sex difference in general mental ability, but men clearly perform better on visuospatial tasks while women clearly perform better on tests of verbal usage and perceptual speed. In this study, we integrated these overall findings with predictions based on the Verbal-Perceptual-Rotation (VPR)…

One of the defining cognitive characteristics of the chromosome 22q deletion syndrome (DS22q11.2) is visuospatial processing impairments. The purpose of this study was to investigate and extend the specific attentional profile of children with this disorder using both an object-based attention task and an inhibition of return task. A group of…

Williams syndrome (WS) is a rare genetic disorder that results in severe visual-spatial cognitive deficits coupled with relative sparing in language, face recognition, and certain aspects of motion processing. Here, we look for evidence for sparing or impairment in another cognitive system--object recognition. Children with WS, normal mental-age…

The test scores of spatial ability for women lag behind those of men in many spatial tests. On the Mental Rotations Test (MRT), a significant gender gap has existed for over 20 years and continues to exist. High spatial ability has been linked to efficiencies in typical computing tasks including Web and database searching, text editing, and…

Explores (1) problems of the validity of tests of spatial ability, and (2) problems of the recessive gene influence theory of the origin of sex differences in spatial ability. Studies of cognitive strategies in spatial problem solving are suggested as a way to further investigate recessive gene influence. (Author/RH)

The academic achievement of boys with fragile X syndrome and the relation between several predictive factors and academic performance are reported. Boys with fragile X syndrome displayed significant deficits in all academic skill areas. Relative strengths were observed in general knowledge, reflecting the ability to integrate experiential…

Studying the biological mechanisms underlying mental retardation and developmental disabilities (MR/DD) is a very complex task. This is due to the wide heterogeneity of etiologies and pathways that lead to MR/DD. Breakthroughs in genetics and molecular biology and the development of sophisticated brain imaging techniques during the last decades…