Study N Ages Study type Methodology Main findings m;f mean (sd) FSIQ mean (sd) Del;UPD Range Range

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1 Table 5 Social Cognition Study N Ages Study type Methodology Main findings m;f mean (sd) FSIQ mean (sd) Del;UPD Range Range Lo et al.(2013) 66 median 11 ToM 14 stories, 33 questions, 3 pretence Median ToM score SDS was ; median 61 exercises covering 3 stages of ToM It correlated significantly with FSIQ 25;39 Maximum score 36 and VIQ but not PIQ. Dutch ToM test-r (Steerneman) Median scores for 3 stages were 10, 8, 5 Sullivan & 14 PWS 12.1 (2.8) ToM Two second-order belief stories: First-order belief: PWS 1.07 (0.92 Tager-Flusberg 10; (10) children asked questions about WS 1.09 (0.92); NMR 0.62 (0.77) (1999) characters beliefs and asked to Second-order ignorance: PWS WS 11.6 (2.7) 65 (11) justify their answers. (0.63); WS 1.59 (0.73); NMR ; (0.78) 13 NMR 11.4 (3.3) 72 (11) Second-order false belief: PWS 6; (0.84); WS ); NMR 0.62 (0.77) 57% of PWS correctly answered second-order belief question but none could provide an explicit justification; 21% gave implicit justifications. Tager-Flusberg 15 PWS 6.9 (1.6) ToM Two false belief tasks were used: Failure on a control (factual) question & Sullivan 8; (17) a standard location change story eliminated 20% PWS, 15% WS and (2000) and an unexpected contents story. 20% NMR. With a maximum score of 2

2 21 WS 7.2 (1.3) 68 (12) Only the child s best score was mean scores for the groups were: 5; recorded. PWS 1.00 (0.95); WS 0.58 (0.87) 15 NMR 7.6 (1.9) 75 (18) NMR 1.33 (0.88) 6; Correct answers to the false belief question were given by 42% PWS; 24% WS: 58% NMR. For PWS false belief correlated 0.63 with age. Causal Nine stories to elicit children s 3 PWS, 1 WS and 3 NMR dropped out. Attribution explanation of a person s actions Total scores (max 12) were: using desire, emotion and cognition PWS 5.50 (2.87); WS 6.40 (2.66); terms (3 each) plus 3 control stories NMR 6.50 (2.50). Mean scores by explanation type (max 3) were: PWS WS NMR Control Desire Emotion Cognition Emotion 4 target photos of basic emotional 1 extra WS and 4 fewer NMR children Matching expressions happy, sad, angry, took part. Overall scores (max 4) were: scared - were displayed. Sets of scared,3.38 happy, 2.38 angry and photos depicting each emotion were 2.33 sad. By group scores were: to be placed under the matching target PWS WS NMR emotion. There were 4 such sets. scared happy angry sad Whittington Emotion 20 faces depicting the 6 basic emotions Mean scores: total 10.7; males 11.7,

3 al. (2011) 24; recognition (4 happy, 4 sad, 3 angry, 3 scared, females 9.9 deletion 10.6, disomy ;15 3 surprised 3 disgusted) were displayed Emotions correctly identified were: in random order on a screen, which Total 55%, 90% happy, 55% sad, 55% also showed the names of the 6 surprised, 43% disgusted, 40% angry emotions. and 37% scared. Correlations with FSIQ, VIQ, PIQ and VS were significant overall but only VIQ and VS were significant for UPD. Halit et al face processing Passive viewing of coloured photos N170 was similar in time course and (2008) ERP study with straight or indirect gaze, upright scalp distribution to that of typical 8;8 72 (19) or inverted, while ERP scalp recordings adults. In deletions the amplitude was were made. The face sensitive similar for upright and inverted faces component N170 was monitored and also for direct and indirect gaze. for peak amplitude and peak latency. In UPDs the amplitude was greater for inverted faces and for averted gaze. For all PWs latency was longer for inverted faces but unaffected by gaze direction. Benton Face A target face is to be identified 3 of 8 deletion and 2 of 6 UPD scores Recognition among 6 simultaneously presented fell outside the normal range (41-54). (22 items) faces with no time constraint 2 UPD failed to complete the task. Eye direction Participants asked which of 3 coloured Mean scores were deletions 5.1 (1.8) detection test pegs a face in a photo is looking at UPDs 4.2 (1.1) (Leekam 1997) Key et al (6.6) Face and object Upright and inverted faces and objects Inverted faces showed lower response (2013) 10;14 processing with positive and negative emotional accuracy than objects. There was a trend 13;11 ERP study valence presented in oddball paradigm to longer reaction times to negative

4 with smiling faces as targets. The stimuli in UPD relative to deletions. N170 ERP component was monitored. In deletions the occipito-temporal N170 There were 144 trials (24x3x2) amplitude was larger for upright faces containing 48 target smiling faces. than for objects, with inverted faces in between. Koenig et al. 18 PWS 19.9 (9.0) Social attribution Participants shown a video in which Responses coded in terms of: salience (2004) 13;5 task. there was a static rectangle with a (%of correctly identified story elements. 13; (14.0) moveable flap on one side which max 20), propositions (number of 21 PDD 15.7 (7.3) 68.9 (8.4) opened or closed and 3 protagonists statements about video content), affect 20;1 a large triangle, a small triangle and (ratio of number of feelings mentioned 17 control 20.8 (6.8) 72.2 (14.3) a small circle, all of which moved to propositions), problem solving (% of 11;6 around. Participants asked to describe questions correctly answered), persons what happened in the video, first with (rating of description of characters in no interpretation of the symbols and video), animation (rating of description then with symbols regarded as people. of actions in video), pertinence (ratio of non-pertinent statements to propositions), and ToM (ratio of statements indicating thinking, planning or intentionality to propositions). Overall PWS performance was similar to PDD and significantly worse than IQmatched controls. The PWS group was particularly impaired on salience, affect and problem solving and was the only group that showed no correlation between salience and affect. Dimitropoulos 39 PWS 16.7 (6.3) Social behaviour Social Responsiveness Scale (SRS) Deletion PWS had significantly worse

5 et al. (2013) 14;25 questionnaires Social competence Inventory (SCI) scores than UPD and ASD groups on SRS 20; (13.5) SRS has subscales: social awareness and its subscales except social 19 ASD 14.2 (4.2) 74.5 (23.3) social cognition, social communication awareness. Deletion PWS scored 16;3 social motivation, social mannerisms significantly higher than ASD on SCI SCI has subscales: SCI prosocial, SCI prosocial. 20% deletion PWS, 5% UPD social initiative. and 5% ASD scored in the severe impairment range on the SRS. Social norms Individual items on the SRS 84% UPD and 60% deletions said to Invade others personal space; 79% UPD and 55% deletions said to talk too loud; 68% UPD and 55% deletions did not respond appropriately to others mood changes; 63% UPD and 35% deletions walked between people when talking. FSIQ = full-scale intelligence quotient. VIQ = verbal IQ. PIQ = performance IQ ToM = theory of mind. WS = Williams syndrome. NMR = non-specific mental retardation. VS = Vineland socialisation scale. ERP=electrophysiological.