Behavioural and personality characteristics of adolescents and adults with

Williams syndrome

K. Jariabková1, I. Ruisel2, V. Bzdúch3

1Department of Social and Biological Communication, 2Institute of Experimental Psychology, Slovak Academy of Sciences,

3First Department of Pediatrics, University Children’s Hospital,

Bratislava, Slovak Republic

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

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

Studies of adaptive behaviourof 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)

Subjects and procedure

14 Sb with WS (9 m, 5 f)Mean age 22 years 7 monthsAge 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.

Self-care skills

in %

Our sample (1997)

n=14, 15-35 yrs

Udwin (1990)

n=119, 16-38 yrs

Toileting 79 88

Bathing 43 61

Dressing 79 66

Independence skills

in %

Our samplen=14

15-35 yrs

Udwin (1990) n=119

16-38 yrsMoves around in near surroundings 79Goes around familiar places on his own 57Uses public transport -for familiar journeys -unfamiliar journeys

290

4210

Manages small purchases (does not manage money) 71

Telephone skills

in %

Our sample (1997)

n=14, 15-35 yrs

Davies et al.(1997)

n=70, 19-39 yrsTelephones -fully independently -to familiar numbers

1442

13

Answers the phone 29 63needs assistance

Dependent or does not use 14 24

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

29Cannot read

10 or more sight words

20

19

Short texts (magazines, encyclopaedias)

57 Simple stories 19

Books (rarely) 14 Books for children aged 9+

43

Current living arrangements20 - 43 years, n=19 (2005)

At home with relatives 18 (95%)

Institution for mentally retardedpeople

1 ( 5%)

Current daytime occupations20 - 43 years, n=19 (2005)

Sheltered employment 2 (11%)

Adult day centre 3 (16%)

Institution for mentallyretarded people

1 ( 5%)

No daytime occupation 12 (63%)

Unknown 1 ( 5%)

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.

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 childrencombined 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 etiologyWS > PWS, NS: often initiates interactions, never goesunnoticed, has many fears, feels terrible when others hurt (Dykens & Rosner, 1999)

Studies of personality in persons with WS

3-20-year-olds (n=28) vs. FXS, PWS, controlsWS > PWS, FXS - AgreeablenessWS < PWS, controls - ConscientiousnessWS < 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 yrsAdults > children - calm, inhibited, withdrawnAdults < children - lively, active, restless, decisive, tearful, quarrelsome, impertinent, over-friendly (Gosch & Pankau, 1997)

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

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

Subjects

Williams syndrome22 Sb (11 m, 11 f)

Agemean 23 years 7 monthsrange 14;7 - 37;2 yrs

Controls WS22 Sb (11 m, 11 f)

Agemean 23 years 7 monthsrange 14;7 - 38;0 yrs

Down syndrome22 Sb (10 m, 12 f)

Agemean 25 years 1 monthrange 14;7 - 38;9 yrs

Controls DS22 Sb (10 m, 12 f)

Agemean 25 yearsrange 14;9 - 38;7 yrs

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.

Personality profiles

1 2 3 4 5

N

A

E

C

O

Big

Fiv

e p

erso

nal

ity

dim

ensi

on

s

WSCWS

DSCDS

5-point rating scale

Openness

• WS < CWS, CDS

Conscientiousness

• WS < DS, CWS

Extroversion

• WS > DS, CWS, CDS

Agreeableness

• WS n.s. DS, CWS, CDS

Neuroticism

• WS > DS, CWS, CDS

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.

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).

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.

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