Continuous Cognitive Dynamics of the Evaluation of Trustworthiness in Williams Syndrome

The decision to approach or avoid an unfamiliar person is based in part on one’s evaluation of facial expressions. Individuals with Williams syndrome (WS) are characterized in part by an excessive desire to approach people, but they display deficits in identifying facial emotional expressions. Likert-scale ratings are generally used to examine approachability ratings in WS, but these measures only capture an individual’s final approach/avoid decision. The present study expands on previous research by utilizing mouse-tracking methodology to visually display the nature of approachability decisions via the motor movement of a computer mouse. We recorded mouse movement trajectories while participants chose to approach or avoid computer-generated faces that varied in terms of trustworthiness. We recruited 30 individuals with WS and 30 chronological age-matched controls (mean age = 20 years). Each participant performed 80 trials (20 trials each of four face types: mildly and extremely trustworthy; mildly and extremely untrustworthy). We found that individuals with WS were significantly more likely than controls to choose to approach untrustworthy faces. In addition, WS participants considered approaching untrustworthy faces significantly more than controls, as evidenced by their larger maximum deviation, before eventually choosing to avoid the face. Both the WS and control participants were able to discriminate between mild and extreme degrees of trustworthiness and were more likely to make correct approachability decisions as they grew older. These findings increase our understanding of the cognitive processing that underlies approachability decisions in individuals with WS

Williams Syndrome and Music: A Systematic Integrative Review

Background: Researchers and clinicians have often cited a strong relationship between individuals with Williams syndrome (WS) and music. This review systematically identified, analyzed, and synthesized research findings related to WS and music.Methods: Thirty-one articles were identified that examined this relationship and were divided into seven areas. This process covered a diverse array of methodologies, with aims to: (1) report current findings; (2) assess methodological quality; and (3) discuss the potential implications and considerations for the clinical use of music with this population.Results: Results indicate that individuals with WS demonstrate a high degree of variability in skill and engagement in music, presenting with musical skills that are more in line with their cognitive abilities than chronological age (CA). Musical strengths for this population appear to be based more in musicality and expressivity than formal musical skills, which are expressed through a heightened interest in music, a greater propensity toward musical activities, and a heightened emotional responsiveness to music. Individuals with WS seem to conserve the overall structure of musical phrases better than they can discriminate or reproduce them exactly. The affinity for music often found in this population may be rooted in atypical auditory processing, autonomic irregularities, and differential neurobiology.Conclusions: More studies are needed to explore how this affinity for music can be harnessed in clinical and educational interventions

Williams Syndrome: links between brain, cognition, and behaviour

© 2005 Marilee A. Martens.The interrelationships between brain, cognition, and behaviour are complex but can be more clearly characterised by studying disorders with an underlying genetic basis. This thesis examined these interrelationships in the context of Williams syndrome (WS), a neurodevelopmental genetic disorder that affects aspects of cognition, behaviour, and brain structure. The principal aims of this thesis were to evaluate the cognitive, behavioural, and neuroanatomical profile of WS individuals and to explore the relationships between aspects of the cognitive and behavioural profile and the neuroanatomical changes that are evident in WS. Three general hypotheses, and 10 specific hypotheses, were postulated as a means of exploring these aims. The first general hypothesis predicted that WS individuals would demonstrate distinct features within their cognitive and behavioural profile. Specifically, it was predicted that WS individuals would show relative strengths on verbal tasks and significant deficits on visuospatial and mathematical tasks, in contrast to control participants who were predicted to show a more even profile. It was also predicted that WS individuals would show evidence of heightened affect in response to music and demonstrate hypersociability as compared to control participant

Adaptive Behavior and Development of Infants and Toddlers with Williams Syndrome

Williams syndrome (WS) is a neurodevelopmental disorder that causes deficits in adaptive behavior, difficulties eating and sleeping, cognitive delays, and delayed development. Although researchers have conducted characterizations of children and adults with WS, less is known about young children with this disorder. This study characterizes the developmental and adaptive behavior features of 16 infants and toddlers with WS aged 3 months - 5 years. Data for this project was obtained from 2007-2014, and includes parent report data and standardized developmental testing. Thirty-one percent (31.3%) of parents reported that their infant/toddler with WS had sleeping problems and 58.3% reported feeding difficulties. Levels of adaptive behavior were in the Mildly Delayed range as measured by the Adaptive Behavior Assessment System, Second Edition. Self care skills such as feeding or dressing oneself were significantly weaker than skills needed to function in the community, such as recognizing his/her home or throwing away trash. The difficulty with self-care skills is hypothesized to be related to the reported difficulties with eating and sleeping. Motor skills were significantly lower than both cognitive and language skills on the Bayley Scales of Infant and Toddler Development, Third Edition. The current study highlights the need for early intervention in these young children across all areas of development, particularly in self-care skills

Approachability and the amygdala: Insights from Williams syndrome

Williams syndrome (WS) is a genetic neurodevelopmental disorder in which hypersociability is a characteristic feature. Given that the amygdala has been identified as an integral component of the neural system underlying sociability, researchers have suggested that the abnormal amygdala volumes found in individuals with WS may play a role in their hypersociability. The aim of this study was to examine the relationship between amygdala volume and hypersociability, as measured by approachability ratings, in 22 individuals with WS and 22 normal controls matched on chronological age, sex, and handedness. We confirmed previous findings of increased amygdala volumes and higher approachability ratings of both ‘positive’ and ‘negative’ faces in individuals with WS. A positive relationship between right amygdala volume and approachability ratings was found in individuals with WS, particularly ratings of ‘negative’ faces. The results unexpectedly revealed that individuals with WS report using features other than the eyes and mouth to determine approachability, particularly when they are younger. These findings support the theory that amygdala dysfunction in WS is related to their hypersociability. Furthermore, we propose that individuals with WS use atypical cognitive strategies compared to controls to determine approachability

Handedness and corpus callosal morphology in Williams syndrome

Williams syndrome is a neurodevelopmental genetic disorder caused by a hemizygous deletion on chromosome 7q11.23, resulting in atypical brain structure and function, including abnormal morphology of the corpus callosum. An influence of handedness on the size of the corpus callosum has been observed in studies of typical individuals, but handedness has not been taken into account in studies of callosal morphology in Williams syndrome. We hypothesized that callosal area is smaller and the size of the splenium and isthmus is reduced in individuals with Williams syndrome compared to healthy controls, and examined age, sex, and handedness effects on corpus callosal area. Structural magnetic resonance imaging scans were obtained on 25 individuals with Williams syndrome (18 right-handed, 7 left-handed) and 25 matched controls. We found that callosal thickness was significantly reduced in the splenium of Williams syndrome individuals compared to controls. We also found novel evidence that the callosal area was smaller in left-handed participants with Williams syndrome than their right-handed counterparts, with opposite findings observed in the control group. This novel finding may be associated with LIM-kinase hemizygosity, a characteristic of Williams syndrome. The findings may have significant clinical implications in future explorations of the Williams syndrome cognitive phenotype