Social context with developmental disorders

Williams syndrome: cause

Deletion of genes on chromosome 7

Williams syndrome: affects how many

1 in 20,000 births

Williams syndrome: 5 key physical signs

1. Low birth weight/ slow weight gain

2. Hypercalcemia

3. Short

4. Musculoskeletal issues

5. Cardiovascular issue

Williams syndrome: Cognitive profile

• Low IQ

• Highly specific visuospatial deficits e.g. mental rotation

• Stronger verbal than non-verbal skills

• Hypersociability

What did Jaiven Palsey (200) find about developmental trajectories in Williams syndrome?

• Language trajectory is steeper than spatial

• There is a relative strength of face recognition

executive function deficits in Williams syndrome

Menghini 2010

• Planning (tower of London)

• Inhibition (Go/No-go)

• Working memory (digit)

• Shifting (trail making test)

Williams syndrome: What is hyper sociability and what does it lead to?

Exaggerated interest in social interaction

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75% of WS individuals report social isolation, 20% sexual abuse (vulnerability), and social victimisation

Santos 2010

Detecting angry & happy faces: WS individuals do not display an anger superiority effect

Comparing WS and other disorders: Sociability questionnaire

Jones 200: WS scored highest on global sociability, social approach and social emotion compared to DS and autism

Riby and Hancock 2008

Free viewing of social scene with eye tracking

Results: WS showed excessive looking to eyes and face

Comparing WS and other disorders: Gaze following

Riby 2013: Free and cued looking

Results: WS look to face whether free or cued looking, better looking to target than autism

ToM in Williams syndrome

Vanterwerten 2013

Compared younger typically developing children with older children with WS

Typically developing group 3x more likely to pass sally-Anne test

Why are individuals with Williams syndrome hypersocial?

• Increased facial orientation/ struggle to disengage from the face

• Potter 2003- poor inhibition

• Poor ability to read negative social cues

• Difficulty understanding others mental states

Turner syndrome: cause and who affected

Caused by part/all of one X chromosome missing

Affects 1 in 20,000 female births

Turner syndrome: Key physical signs (5)

1. Short

2. Heart, kidney and thyroid problems

3. webbed neck

4. low set ears/hairline

5. Underdeveloped ovaries

What other issues stem from underdeveloped ovaries?

1. Amenorrhea

2. Infertility

3. Pubertal delay

Cognitive profile for Turner syndrome

Average full scale IQ but uneven profile:

• Relatively strong verbal IQ

• Relatively weak non-verbal IQ

Weak non-verbal IQ is driven by…

Poor visuo-spatial skills (e.g. embedded figured) and poor EF (planning, working memory etc)

Psychosocial profile in Turners syndrome

• good education/employment outcomes

• Few friends/social activity, withdrawal

• Delay reaching adult sexual and social milestones

• Vulnerable to: depression, anxiety, poor self-image

Turner syndrome and social responsiveness

Hong et al 2013

TS individuals were significantly impaired on all subscales except social motivation

Social cognition in turners syndrome: Emotional recognition

Lawrence 2003- Impairments in emotional recognition, especially regarding fear

Mazzola 2006

Eye-gaze to emotional faces, TS individuals showed more fixations to mouth than eyes

ToM and Turners syndrom

Lawrence 2007- Frith-Happe animations

Women with Turner’s syndrome are significantly impaired in their ability to use mental state language

Which disorder would be categorised by…

• low social motivation

• Face and eye gaze reduced

• Poor emotional Recognition

• Poor ToM

Autism

Which disorder would be categorised by…

• Excessive social motivation

• Excessive looking at face and eyes

• Poor emotional recognition

• Poor ToM

Williams syndrome

Which disorder would be categorised by…

• Typical social motivation

• Reduced eye gaze

• Emotional recognition impaired especially regarding fear

• Poor ToM

Turner syndrome