Assessment & Research

Processing of stimulus content but not of emotional valence is altered in persons with Williams syndrome.

Key et al. (2016) · Journal of intellectual disability research : JIDR 2016
★ The Verdict

Williams syndrome keeps emotional meaning intact but turns down the brain's first step of face perception.

✓ Read this if BCBAs serving adults or teens with Williams syndrome in day programs or residential homes.
✗ Skip if Clinicians looking for emotion-regulation interventions in ASD or CP only.

01Research in Context

01

What this study did

The team tested the adults with Williams syndrome and 20 typical adults. They showed happy, angry, and neutral faces while recording brain waves. Each picture stayed on screen for one second. The task was simple: press a button when a face appeared.

Researchers looked at two early brain peaks. The P100 shows basic visual attention. The N200 shows deeper face processing. They asked: Do WS brains react differently to emotion?

02

What they found

Both groups rated the emotions the same way. Happy faces felt happy. Angry faces felt angry. There was no sign that WS adults over-react to feelings.

Yet their brain waves told another story. WS adults had smaller P100 and N200 peaks when looking at any face. The timing was normal, but the size was lower. This hints at quieter, not hotter, face circuits.

03

How this fits with other research

Fink et al. (2014) also found no emotion-recognition deficit in autistic children once verbal skill was held steady. Both papers warn us: slow or odd social responses may stem from language or perception, not from feeling the emotion wrong.

Belmonte et al. (2019) used the same ERP method in kids with cerebral palsy. They saw reduced brain waves to emotional pictures. Together the studies show smaller early brain responses across three diagnoses, yet everyday feelings stay intact.

Payne et al. (2020) did find that autistic teens mis-read negative faces even when basic face perception was accounted for. This contrast underlines a key point: WS spares emotion labeling, while ASD can leave a specific negative-emotion gap.

04

Why it matters

If you work with WS adults, do not assume they are overwhelmed by emotion. Their social spark may come from poor face filtering, not from extra feelings. Teach them to scan faces longer or use verbal labels to compensate for quieter early vision. Skip programs that try to dial down emotional reactivity; instead, boost attention and memory for facial details.

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Add a five-second look time prompt before asking your client to name a feeling.

02At a glance

Intervention
not applicable
Design
quasi experimental
Sample size
29
Population
other
Finding
null

03Original abstract

BACKGROUND: Individuals with Williams syndrome (WS) exhibit hypersociability and may respond atypically to emotional information in social and nonsocial stimuli. It is not yet clear whether these difficulties are specific to emotional content or stimulus type. This study examined the neural processes supporting social and emotional information processing in WS. METHOD: Visual event-related potentials were recorded in 19 adults with WS and 10 typical peers during a picture-viewing task requiring detection of smiling faces among other social and nonsocial images with positive and negative emotional content. RESULTS: The participant groups were not significantly different in affective processing of positive and negative stimuli and perceived faces as different from nonsocial images. Participants with WS showed subtle differences in face-specific perceptual processes (e.g. face inversion, N170 lateralisation), suggesting a more feature-based processing. They also demonstrated reduced attention and arousal modulation (P3, late positive potential) in response to faces vs. nonsocial images. These differences were independent of intelligence quotient. CONCLUSIONS: There was no evidence of greater than typical perceptual, attentional or affective processing of social information in WS. The results support the idea that altered face perception processes and not the increased salience of social stimuli or difficulties with emotion discrimination may contribute to the hypersocial phenotype in WS.

Journal of intellectual disability research : JIDR, 2016 · doi:10.1037/a0020242