Behavioural and personality characteristics of adolescents and adults with Williams syndrome

1
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
  Childrens 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 persons
  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 behaviourof children and
adolescents with WS

• 4-10-year-olds (n19)
• WS less well-adjusted than nonspecific MR
• (Gosch Pankau, 1994)
• 4-8-year-olds (n41)
• Socialization gt Communication gt Daily living
  skills
• 
  (Mervis et al., 2001)
• 4-18-year-olds (n15)
• Socialization and Communication gt 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 1510 - 352 yrs
• Data collection (1996/97) in semistructured
  interviews
• with parents based on the Vineland Social
  Maturity Scales.
• Czech version (Koený 1974) 36 items cover
  self-care
• skills, motor skills, occupation, communication,
• independence, socialization.
• Normative data as a composite score for ages 30
  to 90
• years in increment of 1 year.

6
Self-care skills
7
Independence skills
8
Telephone skills
9
Reading skills
10
Current living arrangements20 - 43 years, n19
(2005)
11
Current daytime occupations20 - 43 years, n19
(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 (n23) 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 (n35) vs. PWS, nonspecific
  etiology
• WS gt 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 (n28) vs. FXS, PWS, controls
• WS gt PWS, FXS - Agreeableness
• WS lt PWS, controls - Conscientiousness
• WS lt controls - Openness, Emotional stability,
  Motor
• activity, Irritability
  
• 
  (van Lieshout et al., 1997)
• 2-35-year-olds (n105)
• Age groups under 10 yrs, 10 to 20 yrs, over 20
  yrs
• Adults gt children - calm, inhibited, withdrawn
• Adults lt 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 147 - 372 yrs
• Controls WS
• 22 Sb (11 m, 11 f)
• Age
• mean 23 years 7 months
• range 147 - 380 yrs

• Down syndrome
• 22 Sb (10 m, 12 f)
• Age
• mean 25 years 1 month
• range 147 - 389 yrs
• Controls DS
• 22 Sb (10 m, 12 f)
• Age
• mean 25 years
• range 149 - 387 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 lt CWS, CDS
• Conscientiousness
• WS lt DS, CWS
• Extroversion
• WS gt DS, CWS, CDS
• Agreeableness
• WS n.s. DS, CWS, CDS
• Neuroticism
• WS gt DS, CWS, CDS

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