1. Behavioural and personality characteristics of adolescents and adults with Williams syndrome K. Jariabková 1, I. Ruisel 2, V. Bzdúch 3 1 Department of Social and Biological Communication, 2 Institute of Experimental Psychology, Slovak Academy of Sciences, 3 First Department of Pediatrics, University Children’s Hospital, Bratislava, Slovak Republic

2. Clinical observation and research findings Distinctive behavioural and personality patterns in WS Behavioural and emotional difficulties Less consistent data on: -level of independence, adaptive behaviour and the quality of life -changes during the life-span

3. Adaptive behaviour is stressed in definitions of mental retardation - subnormal functioning of both intelligence and adaptive behaviour ( DSM-IV; ICD-10 ) refers to the functioning of an individual in his or her environment, draws together a person’s cognitive and personality characteristics (Mervis & Klein-Tasman, 2000) assessment typically focus on domains of daily living skills, motor skills, communication, and socialization

4. Studies of adaptive behaviour of children and adolescents with WS 4-10-year-olds (n=19) WS less well-adjusted than nonspecific MR (Gosch & Pankau, 1994) 4-8-year-olds (n=41) Socialization > Communication > Daily living skills (Mervis et al., 2001) 4-18-year-olds (n=15) Socialization and Communication > Daily living skills (Greer et al.,1997) (based on the domains or composite scores of the Vineland Social Maturity Scales or Vineland Adaptive Behavior Scales)

5. Subjects and procedure 14 Sb with WS (9 m, 5 f) Mean age 22 years 7 months Age range 15;10 - 35;2 yrs Data collection (1996/97) in semistructured interviews with parents based on the Vineland Social Maturity Scales. Czech version (Kožený 1974): 36 items cover self-care skills, motor skills, occupation, communication, independence, socialization. Normative data as a composite score for ages 3;0 to 9;0 years in increment of 1 year.

6. Self-care skills in % Our sample (1997) n=14, 15-35 yrs Udwin (1990) n=119, 16-38 yrs Toileting7988 Bathing4361 Dressing7966

7. Independence skills in % Our sample n=14 15-35 yrs Udwin (1990) n=119 16-38 yrs Moves around in near surroundings 79 Goes around familiar places on his own 57 Uses public transport -for familiar journeys -unfamiliar journeys 29 0 42 10 Manages small purchases (does not manage money) 71

8. Telephone skills in % Our sample (1997) n=14, 15-35 yrs Davies et al. (1997) n=70, 19-39 yrs Telephones -fully independently -to familiar numbers 14 42 13 Answers the phone2963 needs assistance Dependent or does not use1424

9. Reading skills Our sample (1997) n=14, 15-35 yrs % Udwin (1990) n=119, 16-38 yrs % Cannot read, only letters or simple words, a short sentence 29 Cannot read 10 or more sight words 20 19 Short texts (magazines, encyclopaedias) 57Simple stories19 Books (rarely)14Books for children aged 9+ 43

10. Current living arrangements 20 - 43 years, n=19 (2005)

11. Current daytime occupations 20 - 43 years, n=19 (2005)

12. Summary Self-care and daily living skills remain limited in adolescents and adults with WS. Most of them require at least some support and supervision in everyday activities. Independent living is restricted by their limitations in adaptive functioning.

13. Studies of personality in persons with WS 8-10-year-olds (n=23) vs. mixed etiology sociability and empathy (CBQ) eagerness to interact with others, tension, sensitivity (MPQ) shyness (R) and empathy - 96% of WS children combined characteristics: gregarious, people-oriented, tense, sensitive, and visible - 96% of WS children (Klein-Tasman & Mervis, 2003) 14-50-year-olds (n=35) vs. PWS, nonspecific etiology WS > PWS, NS: often initiates interactions, never goes unnoticed, has many fears, feels terrible when others hurt (Dykens & Rosner, 1999)

14. Studies of personality in persons with WS 3-20-year-olds (n=28) vs. FXS, PWS, controls WS > PWS, FXS - Agreeableness WS < PWS, controls - Conscientiousness WS < controls - Openness, Emotional stability, Motor activity, Irritability (van Lieshout et al., 1997) 2-35-year-olds (n=105) Age groups: under 10 yrs, 10 to 20 yrs, over 20 yrs Adults > children - calm, inhibited, withdrawn Adults < children - lively, active, restless, decisive, tearful, quarrelsome, impertinent, over-friendly (Gosch & Pankau, 1997)

15. Five-factor model of personality description identified on the basis of lexical hypothesis: important individual differences are encoded as single terms in language ( Goldberg, 1990 ) personality traits usually grouped into five factors across different languages assessment - using lists of adjectives or questionnaire statements replicated in various populations and cultures using both self-reports and observer ratings

16. Big Five personality dimensions Openness to experience - preference for variety, for new ideas and experiences Conscientiousness - individual level of organization, achievement orientation Extroversion - quality and intensity of social orientation and activity Agreeableness- quality of interpersonal orientation Neuroticism - tendency to experience negative affects, anxiety, maladjustment in contrast to emotional stability

17. Subjects Williams syndrome 22 Sb (11 m, 11 f) Age mean 23 years 7 months range 14;7 - 37;2 yrs Controls WS 22 Sb (11 m, 11 f) Age mean 23 years 7 months range 14;7 - 38;0 yrs Down syndrome 22 Sb (10 m, 12 f) Age mean 25 years 1 month range 14;7 - 38;9 yrs Controls DS 22 Sb (10 m, 12 f) Age mean 25 years range 14;9 - 38;7 yrs

18. Procedure All subjects assessed by their parents and caregivers. FFI-MH inventory (I. Ruisel): Statements describing behaviour and personality characteristics. 5-point scale: from the least to the most characteristic of the person. Forty items assumed to correspond to the Big Five personality dimensions.

19. Personality profiles Openness WS < C WS, C DS Conscientiousness WS < DS, C WS Extroversion WS > DS, C WS, C DS Agreeableness WS n.s. DS, C WS, C DS Neuroticism WS > DS, C WS, C DS

20. Discriminant analysis Items important for the discrimination of the Williams syndrome and Down syndrome groups: The following combined characteristics: Keeps his/her things in order and cleanliness; Is talkative; Tries to be a friend to everyone; Likes order and regularity; Laughs readily Correctly classified 91% of the WS subjects and 96% of the DS subjects.

21. Comments Adolescents and adults (present sample): Higher scores on extroversion correspond to the findings on the interest in making interpersonal contacts. Children and adolescents: no differences between WS and comparison groups (van Lieshout et al., 1998) A decrease in extroversion in individuals with WS until adulthood (Gosch & Pankau, 1997). Adolescents and adults (present sample): Higher neurotism and lower conscientiousness than in DS and controls. A decrease in neuroticism with age until adulthood. (Gosch & Pankau, 1997).

22. Concluding remarks The personality profile of adolescents and adults with WS is characterized by higher extroversion and neuroticism. Their level of independence is low. Their over-friendly behaviour, talkativeness and interest in interpersonal contacts might give a false impression of their functioning in other areas. Possible changes in behavioural and personality traits during the life-span need further clarification.

23. Acknowledgements All participating parents and caregivers The Slovak Williams Syndrome Association Teachers and educators from special schools and daycare centres The Hungarian Williams Syndrome Association