Memory abilities in Williams syndrome: Dissociation or developmental delay hypothesis?

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 has been also proposed to characterize WS memory ability, with sparing of auditory short-term memory and impairment of spatial and long-term memory abilities. In this study, we explored the possibility of a double memory dissociation in WS (short- versus long-term memory; verbal versus visual memory). Thus, verbal memory abilities were assessed using California Verbal Learning Test and Digit Span and Rey-Osterrieth Complex Figure and Corsi Blocks was used to assess visual-spatial memory abilities. Overall, WS subjects were found to present a generalized significant impairment in verbal and visuo-spatial components either in short- or long-term memory. In sum, data from this study brings support for a developmental delay hypothesis, rather than a double dissociation within memory systems in WS.     

People with Williams syndrome process faces holistically

This study compared the performance of 47 adolescents and adults with Williams syndrome to 39 age-matched controls on a face recognition task. Using the whole-part paradigm developed by Tanaka and his colleagues, we found that although performance overall was lower in the participants with Williams syndrome, both groups showed similar patterns of performance across the different conditions. Both groups performed significantly better in the whole-face than in the isolated-part test condition for upright faces, but not for inverted faces. The whole-face advantage only in the upright condition provides strong evidence that people with Williams syndrome encode and recognize faces holistically in the same way as normal controls, suggesting the use of similar underlying neurocognitive mechanisms. These findings contradict earlier reports in the literature that people with Williams syndrome process faces abnormally.     

威廉斯综合症及其研究现状

威廉斯综合症是一种上世纪 60年代才被人类认识的疾病。该病症有特殊的生理、行为和认知异常 ,脑发育伴随着结构和临床异常。本文对国外40年的研究成果进行了总结 ,初步介绍了威廉斯综合症的症状表现、认知特征、行为特征及诊断治疗方面的研究现状 ,最后对该领域的研究提出了展望。     

Perceptual grouping and distance estimates in typical and atypical development: comparing performance across perception, drawing and construction tasks

Perceptual grouping is a pre-attentive process which serves to group local elements into global wholes, based on shared properties. One effect of perceptual grouping is to distort the ability to estimate the distance between two elements. In this study, biases in distance estimates, caused by four types of perceptual grouping, were measured across three tasks, a perception, a drawing and a construction task in both typical development (TD: Experiment 1) and in individuals with Williams syndrome (WS: Experiment 2). In Experiment 1, perceptual grouping distorted distance estimates across all three tasks. Interestingly, the effect of grouping by luminance was in the opposite direction to the effects of the remaining grouping types. We relate this to differences in the ability to inhibit perceptual grouping effects on distance estimates. Additive distorting influences were also observed in the drawing and the construction task, which are explained in terms of the points of reference employed in each task. Experiment 2 demonstrated that the above distortion effects are also observed in WS. Given the known deficit in the ability to use perceptual grouping in WS, this suggests a dissociation between the pre-attentive influence of and the attentive deployment of perceptual grouping in WS. The typical distortion in relation to drawing and construction points towards the presence of some typical location coding strategies in WS. The performance of the WS group differed from the TD participants on two counts. First, the pattern of overall distance estimates (averaged across interior and exterior distances) across the four perceptual grouping types, differed between groups. Second, the distorting influence of perceptual grouping was strongest for grouping by shape similarity in WS, which contrasts to a strength in grouping by proximity observed in the TD participants.     

Object recognition with severe spatial deficits in Williams syndrome: sparing and breakdown

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 (MA) and chronological age-matched (CA) children, and normal adults viewed pictures of a large range of objects briefly presented under various conditions of degradation, including canonical and unusual orientations, and clear or blurred contours. Objects were shown as either full-color views (Experiment 1) or line drawings (Experiment 2). Across both experiments, WS and MA children performed similarly in all conditions while CA children performed better than both WS group and MA groups with unusual views. This advantage, however, was eliminated when images were also blurred. The error types and relative difficulty of different objects were similar across all participant groups. The results indicate selective sparing of basic mechanisms of object recognition in WS, together with developmental delay or arrest in recognition of objects from unusual viewpoints. These findings are consistent with the growing literature on brain abnormalities in WS which points to selective impairment in the parietal areas of the brain. As a whole, the results lend further support to the growing literature on the functional separability of object recognition mechanisms from other spatial functions, and raise intriguing questions about the link between genetic deficits and cognition.     

The eyes or the mouth? Feature salience and unfamiliar face processing in Williams syndrome and autism

Using traditional face perception paradigms the current study explores unfamiliar face processing in two neurodevelopmental disorders. Previous research indicates that autism and Williams syndrome (WS) are both associated with atypical face processing strategies. The current research involves these groups in an exploration of feature salience for processing the eye and mouth regions of unfamiliar faces. The tasks specifically probe unfamiliar face matching by using (a) upper or lower face features, (b) the Thatcher illusion, and (c) featural and configural face modifications to the eye and mouth regions. Across tasks, individuals with WS mirror the typical pattern of performance, with greater accuracy for matching faces using the upper than using the lower features, susceptibility to the Thatcher illusion, and greater detection of eye than mouth modifications. Participants with autism show a generalized performance decrement alongside atypicalities, deficits for utilizing the eye region, and configural face cues to match unfamiliar faces. The results are discussed in terms of feature salience, structural encoding, and the phenotypes typically associated with these neurodevelopmental disorders.     

Taxonomy of moderators that govern explicit memory in individuals with intellectual disability: Integrative research review

This integrative research review aims to discover moderators that influence explicit memory performance of individuals with intellectual disability (ID). We reviewed 47 explicit memory studies (since 1990) that were conducted in populations with ID. We suggest a taxonomy of moderators related to the participants, encoding and retrieval stages, where only an interrelation between the three dictates memory performance. We found that individuals with nonspecific ID can achieve the same level of recognition as individuals with typical development (TD) with the same chronological age when all encoding moderators are favorable. Recognition tests facilitate recollection more than free recall in all etiologies. The performance of individuals with ID was poorer than that of individuals with TD in all auditory memory tasks. Spatial location memory varied with task demands, practice, intention, age and intelligence. We conclude with suggestions for further research and educational implications.     

Global and Local processing in Williams Syndrome: Drawing versus Perceiving

It has been hypothesized that a local processing bias underlies overall visuospatial impairments in Williams syndrome (WS). However, recent studies have challenged this hypothesis by providing evidence against a local processing bias at the perceptual level. The aim of the present study was to further examine drawing and perceptual skills in children with WS using closely matched-hierarchical stimuli. In the drawing task children with WS exhibited a local processing bias. However, no significant preferential bias was found in the perceptual task. This indicates that children with WS do not systematically present a preferential bias for local information. Taken together the findings of the present study suggest that perceptual processing deficits per se are unlikely to explain local processing biases in visuoconstructive tasks often described in people with WS.     

Attentional disengagement in adults with Williams syndrome

Williams syndrome (WS) is a neurodevelopmental disorder characterized by a distinctive behavioral and cognitive profile, including widespread problems with attention. However, the specific nature of their attentional difficulties, such as inappropriate attentional allocation and/or poor attentional disengagement abilities, has yet to be elucidated. Furthermore, it is unknown if there is an underlying difficulty with the temporal dynamics of attention in WS or if their attentional difficulties are task-dependent, because previous studies have examined attention in established areas of deficit and atypicality (specifically, visuospatial and face processing). In this study, we examined attentional processing in 14 adults with WS (20–59 years) and 17 typically developing controls (19–39 years) using an attentional blink (AB) paradigm. The AB is the decreased ability to detect a second target when it is presented in close proximity to an initial target. Overall, adults with WS had an AB that was prolonged in duration, but no different in magnitude, compared with typically developing control participants. AB performance was not explained by IQ, working memory, or processing speed in either group. Thus, results suggest that the attention problems in WS are primarily due to general attentional disengagement difficulties rather than inappropriate attentional allocation.     

What makes counting count? Verbal and visuo-spatial contributions to typical and atypical number development

Williams Syndrome (WS) is marked by a relative strength in verbal cognition coupled with a serious impairment in non-verbal cognition. A strong deficit in numerical cognition has been anecdotally reported in this disorder; however, its nature has not been systematically investigated. Here, we tested 14 children with WS (mean age=7 years 2 months), 14 typically developing controls individually matched on visuo-spatial ability (mean age=3 years 5 months) as well as a larger group of typically developing controls (mean age=3 years 4 months) on two tasks to assess their understanding that counting determines the exact quantity of sets (cardinality principle). The understanding of the cardinality principle in children with WS is extremely delayed and only at the level predicted by their visuo-spatial MA. In this clinical group, only language accounted for a significant amount of the variance in cardinality understanding, whereas in the normal comparison group only visuo-spatial competence predicted the variance. The present findings suggest that visuo-spatial ability plays a greater role than language ability in the actual development of cardinality understanding in typically developing children, whereas the opposite obtains for the clinical group.     

Configural and local processing of faces in children with Williams syndrome

Three experiments investigated face processing in children with Williams syndrome (WS). In Experiment 1, the ability to discriminate different aspects of faces was compared between WS subjects and a group of children individually matched for chronological age (CA-matches) and another group matched for mental age (MA-matches). In Experiments 2 and 3, the ability to process the local and configural aspects of geometrical patterns and faces was assessed within the same groups of subjects. The results indicated that the WSs' overall performance on face recognition was below that of the CA-matches, but similar to that of the MA-matches. This study revealed in addition that the CA- and MA-matches showed a bias toward a configural mode of face and geometrical shape processing, whereas children with WS did not show any bias. These findings suggest that face processing undergoes an abnormal developmental course in WS.     

Affect, Social Behavior, and the Brain in Williams Syndrome

ABSTRACT— Williams syndrome (WS) is a rare genetic disorder characterized by intellectual impairment and a distinctive physical and neuropsychological profile. Relative to their level of intellectual functioning, individuals with WS exhibit strengths in language and face recognition, with deficits in visual-spatial cognition. A heightened appetitive drive toward social interaction is a strong behavioral feature. Relative to other neurodevelopmental disorders, WS has a clearly defined genetic basis, together with a consistent neurocognitive profile of strengths and deficits. Thus, this disorder offers unique opportunities for elucidating gene–brain–behavior relationships. We focus on manifestations of the unusual social profile in WS, by examining data within and across levels of cognition, brain, and molecular genetics.     

Delineation of a spatial working memory profile using a non-verbal eye-tracking paradigm in young children with autism and Williams syndrome

Working memory deficits profoundly inhibit children’s ability to learn. While deficits have been identified in disorders such as autism spectrum disorder (ASD) and Williams syndrome (WS), findings are equivocal, and very little is known about the nature of these deficits early in development. A major barrier to advances in this area is the availability of tasks suitable for young children with neurodevelopmental disorders who experience difficulties with following verbal instructions or who are distressed by formal testing demands. To address these issues, a novel eye-tracking paradigm was designed based on an adaptation of the classic A not B paradigm in order to examine the early foundations of spatial working memory capabilities in 26 developmentally delayed preschool children with ASD, 18 age- and IQ-matched children with WS, and 19 age-matched typically-developing (TD) children. The results revealed evidence that foundational spatial working memory performance in ASD and WS was comparable with that of TD children. Performance was associated with intellectual ability in the ASD and TD groups, but not in the WS group. Performance was not associated with adaptive behavior in any group. These findings are discussed in the context of previous research that has been largely limited to older and substantially less developmentally delayed children with these neurodevelopmental disorders.     

Morphological patterns in Hungarian children with Williams syndrome and the rule debates

Williams syndrome (WMS), a rare neurogenetic disorder, has been in the forefront of research in cognitive psychology for the last 10 years. Studies of grammatical development in 14 Hungarian WMS children are presented: they were examined on tasks testing regular and irregular morphology; measures of digit span were also obtained. Results on the production of accusative and plural forms confirmed for Hungarian that regardless of the frequency of the item, inflected forms of irregulars are harder to produce, and often regularized in WMS, revealing a dissociation between the rules of grammar vs. the mental lexicon. Overall performance on the morphology task is associated with the capacity of phonological short-term memory: subjects with higher span perform better on both tasks. The specification of the surprisingly close relation of phonological short-term memory with the linguistic measures awaits further study.