L9 - Social Understanding in the context of developmental disorder Flashcards
1
Q
What is Williams Syndrome?
A
- Neurodevelopmental disorder
- Genetic cause - deletion of genes on chromosome 7
- Affects 1 in 20000 births
- Distinct physical, cognitive and behavioural presentation
2
Q
What are key physical signs of WS?
A
- Characteristic facial features
- Hypercalcemia: bone issues
- Low Birth weight & slow weight gain
- Height shorter than average
- Cardiovascular problems
- Musculoskeletal problems
3
Q
What is a cognitive profile for someone with WS?
A
- 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
4
Q
What deficits do people with WS show in executive function?
A
- Planning
- Inhibition
- Shifting
- Working memory
5
Q
How does WS show Hypersociability?
A
- 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
6
Q
What was the cross-syndrome comparison?
A
- Sociability questionnaire made up of three components
- WS show more sociability in all three domains
7
Q
How did WS patients do in the social scene perception?
A
- Free viewing of a social scene by typically developing children
- Shows excessive looking to the eyes and face but they are not social experts
8
Q
How to measure emotion recognition in people with WS?
A
- 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
9
Q
How to measure gaze following in people with WS?
A
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
10
Q
Results with autism:
A
- 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
11
Q
ToM in Williams Syndrome
A
- ToM is impaired in WS
- Compared WS (5-17) to younger TD (2-9) and matched on verbal ability
12
Q
Why do WS have a hypersocial profile?
A
- 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
13
Q
What is turner syndrome?
A
- 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
14
Q
How to treat turner’s Syndrome?
A
- Growth hormone treatment in childhood
- Oestrogen replacement therapy in adolescence
15
Q
Key physical signs of TS:
A
- Shorter than average
- Underdeveloped ovaries: pubertal delay, amenorrhea, infertility
- Webbed neck, low set ears and hairline
- Associated with heart, kidney and thyroid problems
