Williams Syndrome Flashcards
What is WS?
Neurodevelopmental disorder that is genetically caused by deletion of genes on chromosome 7.
It affects 1 in 20,000 births and individuals with WS have distinct physical, cognitive and behavioural presentation.
Diagnosis is tested biologically and therefore much more accurate than autism.
Physical signs - characteristic facial features, hypercalcemia, low birth wight and slow weight gain, slightly shorter than average and have cardiovascular and musculoskeletal problems.
What are the deficits of WS?
Low IQ (av 55) - stringer verbal than non
Highly specific and universal visuospatial deficits - block design and mental rotation
EF - planning, inhibition, shifting and WM
Hypersociability - exaggerated interest in social interaction and are often described as ‘over- friendly, more readily approach strangers, subject to social victimisation, lack of social judgement, usually isolated and vulnerable to sexual abuse
Looking patterns
Riby and Hancock (2008) compared WS, ASD and TD children on a free viewing of a social seen using eye tracking. Results show that ASD individuals’ looking patterns seem fairly random and complete avert from looking at the face and eyes but the complete opposite result is seen in WS individuals who intensely look at the face and particularly the eyes, scanning much less of the rest of the photo.
Just because WS show excessive looking to the eyes and face it doesn’t necessarily mean that they are social experts.
Emotion recognition
Javinen-Paisley et al (2010) compared emotion recognition in WS compared to TD individuals and WS performed significantly worse in recognising fear which may explain why they freely approach strangers. They also stuggle to identify neutral face and are more likely to attach an emotion to a neutral face and misread their emotional state. TD individuals achieved the max score for each emotion demonstrating a ceiling effect which suggests it wasn’t challenging enough.
An anger superiority effect is seen in most TD individuals when shown one angry face amongst varying distractor happy faces as there is an evolutionary advantage. This effect is not seen in WS individuals.
ToM is also impaired in WS individuals as Van Herwegen et al (2013) found 79% failed Sally-Anne task. This failure to understand other peoples mental states and read emotional cued may account for their hypersociability.
Gaze following
ASD show significantly less spontaneous looking to the face compared to TD. They increase looking when cued but are unable to follow gaze. WS show increased looking to the face compared to TD. They show the same looking pattern as TD i.e increased looking to the target when cued but to a less significant degree. They find it difficult to disengage from the face. The same behavioural response occurs but different underlying pathology.
Riby et al (2013)
Showed participants a picture of a person in a room looking at something.
Participants are either asked to detect what they are looking at or just to look at the picture freely.
Found that ASD individuals have strange looking patterns and accuracy was very low, but WS were better at identifying the target in cued condition but still spent too long focussing on the face which prevents them identifying target in free looking condition.
