Table 5.
Studies examining social cognition in Williams syndrome (WS).
| Reference | N | Mean age in years (range) | Full scale intelligence quotient (SD) [range] | Mental age (MA), verbal mental age (VMA), and non verbal mental age (NVMA) (SD) [range] | Comparison group | Social cognition domain (task) | Data analysis | Main results |
|---|---|---|---|---|---|---|---|---|
| (93) | 27 | 17.41 (5.33–43.66) | 47 (19) [18–84] | MA 5.75 (1.1) [3.78–8.66] | Typically developing matched on mental age (TDMA) + typically developing matched on chronological age (TDCA) | Cognitive theory of mind (ToM) | ANOVA | Individuals with WS attributed less negative intentions than TDMA and TDCA groups (p < 0.001) |
| (57) | 19 | 21.5 (7.16–38.83) | – | – | TDCA + TDVA | Identification of complex emotions (RMET) |
T-test ANOVA, Tukey post hoc tests |
Adults with WS perform at similar level than TDCA group when identifying whether the actors are “deciding” (p = 0.553), “not sure” (p = 0.279), or “worried” (p = 0.553) They were less accurate at identifying “ do not trust ” in comparison to TDCA group (p = 0.001) and TDVA group (p = 0.008), “disapproving” in comparison with TDCA group (p < 001), “relieved” in comparison to TDCA group (p = 0.013) |
| (94) | 24 | 32.36 (15.4–56.9) | 65 (7.10) [50–80] | – | Typically developing (TD) | Vocal emotion recognition in multisensory emotional information (paralanguage test) | T-test | Between-group differences in performance with the emotionally congruent multisensory (visual and vocal) did not reach the adjusted significance level (p = 0.07). But the TD group outperforming the WS group with the emotionally incongruent audiovisual stimuli (p < 0.001) and the unimodal auditory stimuli (p < 0.001) |
| (95) | 12 | 11.4y (9.6–13.9) | – | – | Autism spectrum disorder (ASD) + TD | Vocal emotion recognition in stimuli without semantic message (paralanguage test) | ANCOVA | No between-group differences were found (p > 0.065) |
| (96) | 8 | 27.8y (18–42) | – | – | DD + TD | Vocal emotion recognition in stimuli without semantic message (paralanguage test) | ANOVA T-test |
The TD group outperforming the WS group (p = 0.004). The interaction effect was due to the WS group exhibiting lower recognition accuracy for negative stimuli (p = 0.001), while no between-group differences were in evidence for the processing of the positive stimuli (p =0.73) |
| (97) | 21 | 24.0 (12–40) | – | – | DD + TD | Identification of static facial expressions (emotion is associated with congruent or non congruent evocativ music) Vocal emotion recognition in multisensory emotional information (paralanguage test) |
ANOVA | Emotionally evocative and congruent music facilitated the ability of participants with WS, DD, or TD to process emotional facial expressions (p = 0.03) |
| (98) | 57 | 9.24 (6.00–12.74) | 72.93 (15.17) [40–97] | – | – | Ability to infer first-order false beliefs | WS patients had difficulties to understand false beliefs | |
| (99) | 12 | 8.10 (6.1–15.3) | – | VMA 5.8 (1.11) [3.4–9.3] | AUT (autism group) + TDVMA | Identification of static facial expressions (labeling task) | ANOVA | Individuals with WS showed emotion recognition levels similar to controls with the same mental age or individuals with developmental disabilities |
| (100) | 25 | 9.5 (6–15) | 54.7 (8.95) | – | – | Identification of static facial expressions (labeling task) | Individuals with WS recognized facial affect at an appropriate developmental level | |
| (101) | 20 | 12.25 (5.6–23.58) | – | MA 5.58 (0.75) [4.25–6.83] | DS + TDCA + TDMA | Identification of static facial expressions (labeling task) | ANOVA (pair-wise comparisons with Bonferroni correction) | In emotion recognition task, no significant differences were found between participants with WS and TDMA group but participants with WS performed significantly lower than TDCA group (p < 0.001). the DS group performed significantly lower than the WS (p ≤ 0.001) |
| (102) | 11 | 30.6 | 66.4 (11.5) | – | TD | Processing of egocentric gaze | T-test | No difference in accuracy when WS and TD groups were compared (p < 0.057). However, WS participants were slower than controls (p < 0.0001) in determining the gaze direction |
| (103) | 47 | 19.49 (12.1–32.4) | 69.08 (12.2) [51–100] | – | Learning disability + TD | Identification of static facial expressions (labeling task) Vocal emotion recognition in stimuli with semantic message (paralanguage test) |
ANCOVA | In labeling task and paralanguage test, WS group was significantly less accurate in recognition of sadness, anger and fear (p < 0.0001) than TD group. No difference is revealed between WS and learning disability groups |
| (104) | 20 | 16.13 (5.33–43.67) | – | – | DS + TDCA + TDMA | Identification of static facial expressions (labeling task) | ANOVA | Individuals with WS showed emotion recognition levels similar to TDMA group (p > 0.1). WS groups outperformed the DS group (p < 0.05). And TDCA group outperformed WS group (p < 0.01) |
| (105) | 31 | 17.02 (5.33–43.67) | – | MA 5.65 (1.31) [3.58–9.33] | TDCA + TDMA | Ability to infer first-order false beliefs (non-verbal picture-sequencing task) | ANOVA | The WS group performed significantly below the TDMA group on the false belief stories (p < 0.01). These groups performed similarly on all other story types (understanding of pretense and intent). TDCA group performed significantly better than the WS group and TDMA group on all story type (p < 0.01) |
| (106) | 16 | 25.14 (11.42–50.58) | 61 (15) [38–84] | – | TDMA | Identification of static facial expressions (labeling task) Exploring face |
ANOVA | Individuals with WS showed emotion recognition levels similar to TDMA group: no significant main effect for group was revealed (p > 0.1) The WS group spent significantly more time looking at the eyes than TD controls (p < 0.05) but the groups spent a similar amount of time looking at the nose (p > 0.1) and the mouth (p = 0.1) |
| (107) | 15 | 10.41 (6.0–15.83) | – | VMA7:2 (20) | ASD + TDVMA match with WS + TDNV match with WS + VMA match with ASD + TDNV match with ASD | Perception of facial expression (picture-to-picture matching task) | T-test | Participants with WS were more accurate when perceiving happiness, sadness, anger, and surprise relative to TDVMA (p < 0.05) and TDNV (p < 0.01) group, who did not differ |
| (108) | 14 | 15.16 (8.75–28.0) | – | – | ASD + TDNV matched individually with WS + TDNV matched individually with ASD | Exploring face | T-test | Individuals with WS fixated faces for longer than participants who were developing typically (p < 0.001) |
| (109) | 15 | 13.50 (8.66–28.0) | – | – | ASD + TDNV matched individually with WS + TDNV matched individually with ASD | Processing of allocentric gaze | T-test | In comparison with TDNV, participants with WS had difficulties to interpret eye-gaze direction (p < 0.05) |
| (110) | 29 | 14.7 (7–27) | 57.8 (12.3) [40–93] | MA 8.3 (2.9) [4–17] | TDMA +TDCA | Identification of static facial expressions (labeling task) | ANOVA | No such group differences were found for photographs of real faces (WS vs. TDMA, p > 0.05; WS vs. TDCA, p > 0.05) |
| (111) | 19 | 14.4 (7–26) | 57.5 (11.0) | MA 8.3 (3.5) VMA 9.3 (3.5) PMA 7.9 (2.7) |
TD | Cognitive ToM (strange stories) | ANOVA (turkey test) | WS group were significantly less accurate on the visual than on the verbal modality (p < 0.001) |
| (112) | 19 | 13.7 (5.1–30.0) | 63.3 (12.33) | MA 6.5 (1.9) [4.1–11.2] | TDMA + TDCA | Processing of motor action | ANOVA | The WS and TDMA groups differed from performance of the TDCA group (p = 0.015 and p = 0.025, respectively). Most interestingly both the WS and the TDMA groups were significantly aided by the presence of contextual cues (p < 0.001), while the presence of contextual cueing brought no variation in performance within the TDCA group (p = 0.063) |
| (113) | 16 | 12.58 (5.08–22.66) | 64.9 (13.50) [44–87] | MA 6.5 (1.33) [4.1–11.2] | ASD + TDMA + TDCA | Processing of motor action | ANOVA | In the presence of context cues, children with WS were as accurate as children with the same chronological age and children with the same mental age in determining why others perform specific motor actions. Amount of errors made by all groups did not differ |
| (114) | 29 | 19.1 (13.1–32.1) | 68.1 (12.8) [45–94] | Learning/intellectual disability + TDCA | Identification of dynamic facial expression (labeling task) | ANOVA | Participants with WS were less accurate than controls with TDCA (p < 0.001). But they were as accurate as participants with intellectual disabilities (p = 0.87) | |
| (115) | 21 | 7.16 (4.5–8.58) | 68 (12) [43–93] | VMA 4.91 (1.33) [3.1–8.2] | Prader–Willi syndrome (PWS) + NMR | Perception of facial expression (picture-to-picture matching task) Ability to infer first-order false beliefs (similar to the Sally–Ann task) Affective ToM (explanation of action task) |
Chi-square test | On the false belief question, more of the MRU and PWS children passed than did the WMS children (p < 0.06). The three groups performed at a similar level in picture-to-picture matching test and the explanation of action task |
| (116) | 30 | 9.91 (5.00–17.08) | – | – | TD | Ability to infer first-order false beliefs (task similar to the Sally–Ann task but presented in video and without narrative) | Fisher’s exact test | More participants in the WS group failed the false belief question in contrast to the TD group (p = 0.001) |