L9 - Social Understanding in the context of developmental disorder Flashcards
What is Williams Syndrome?
- Neurodevelopmental disorder
- Genetic cause - deletion of genes on chromosome 7
- Affects 1 in 20000 births
- Distinct physical, cognitive and behavioural presentation
What are key physical signs of WS?
- Characteristic facial features
- Hypercalcemia: bone issues
- Low Birth weight & slow weight gain
- Height shorter than average
- Cardiovascular problems
- Musculoskeletal problems
What is a cognitive profile for someone with WS?
- Low IQ: possible learning disabilities
- Stronger verbal than non-verbal abilities
- Highly specific and universal visuo-spatial deficits e.g block design and mental rotation
- Distinct developmental trajectories: they are good at recognising faces
- More patchy/inconsistent profile than other disorders
What deficits do people with WS show in executive function?
- Planning
- Inhibition
- Shifting
- Working memory
How does WS show Hypersociability?
- Exaggerated interest in social interaction
- Over friendly
- Social approaches to strangers
- Difficulties with peer relationships
- Social victimisation
- Lack social judgement
- Social isolation
- Vulnerable to sexual abuse
What was the cross-syndrome comparison?
- Sociability questionnaire made up of three components
- WS show more sociability in all three domains
How did WS patients do in the social scene perception?
- Free viewing of a social scene by typically developing children
- Shows excessive looking to the eyes and face but they are not social experts
How to measure emotion recognition in people with WS?
- Ppts indicated if they could see an angry/happy face among distractors = either 2,5, 8
- Number of distractors = harder to find happy face in typically development but no no. of distractors affects finding the angry face
- WS show absence of anger superiority as distractors increase= performance drops
- Reduce ability to detect social threat signals = inc, social approaches
How to measure gaze following in people with WS?
Either free looking: look at each picture for as long as it stays on the screen, OR cued looking: Detect and name what actor is looking at
Results with autism:
- Autistic group show less spontaneous looking to the face compared to TD
- Increase looking when cued but are unable to follow gaze
- WS show increased looking to the face compared to TD = found it hard to disengage from the face
- Same behavioural responses & poor detection of correct target but different underlying pathology
- Good for eye tracking e.g look the same behaviourally but their looking patterns are very different due to eye gaze
ToM in Williams Syndrome
- ToM is impaired in WS
- Compared WS (5-17) to younger TD (2-9) and matched on verbal ability
Why do WS have a hypersocial profile?
- Increased orientation to the face and difficulty in disengaging attention
- Poor inhibition
- Poor ability to read emotional cues, particularly negative cues
- Difficulty in understanding other people’s mental states
What is turner syndrome?
- Neurodevelopmental disorder
- Genetic cause - part or all of one of the X chromosomes is missing
- Affects 1/2000 female birth
- Key physical signs emerge at puberty so diagnosis often only occurs in adolescence
How to treat turner’s Syndrome?
- Growth hormone treatment in childhood
- Oestrogen replacement therapy in adolescence
Key physical signs of TS:
- Shorter than average
- Underdeveloped ovaries: pubertal delay, amenorrhea, infertility
- Webbed neck, low set ears and hairline
- Associated with heart, kidney and thyroid problems
What is the cognitive profile for TS?
- Average full scale IQ but uneven profile: strong verbal IQ, weak non-verbal IQ
- Non-verbal weakness driven by poor visuo-spatial skills
What is the psychosocial profile for TS?
- Fewer friends, engage in fewer social activities, more social withdrawal
- Delay in reaching adult sexual and social developmental milestones
- Vulnerability for depression, anxiety and poor self image
- Good educational and employment outcomes
How do TS compare to autistic traits?
- Social responsiveness scale
- Identifies social impairments associated with autism
- Found that significantly impaired social responsiveness on all subscale scores bar social motivation
- However, autistic groups would score a lot higher than TS, but they still score higher than control, just not as much as autistic people
What is social cognition like in TS?
Physical differences that women with TS face are likely to contribute to difficulties with peer relationships, social withdrawal and late onset of sexual relationships
Emotion recognition in TS:
- Specific fear recognition deficit but other difficulties are more subtle, but fear is most obvious one
- Compared to controls, women with TS made more fixations to the mouth and less fixations to the eyes
ToM in TS:
Significantly impaired in use of mental state language
