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Adults with Williams syndrome: Preliminary study of social, emotional and behavioural difficulties

  • Mark Davies (a1), Patricia Howlin (a1) and Orlee Udwin (a2)

Abstract

Background

In recent years there has been an upsurge of interest in the study of distinctive patterns of behavioural and psychological characteristics associated with specific, biologically determined, intellectually disabling conditions. This study investigates whether such a profile can be identified in adults with Williams syndrome.

Method

Parents and other care-givers were interviewed about the social, emotional and behavioural characteristics of 70 adults with Williams syndrome, aged 19 years to 39 years 9 months.

Results

The adults were reported to have high rates of behavioural and emotional difficulties, particularly in terms of poor social relationships, over-friendliness and social disinhibition, preoccupations and obsessions, and high levels of anxiety and distractibility.

Conclusions

The findings provide preliminary support for the existence of a specific pattern of behavioural and personality characteristics and associated difficulties in adults with Williams syndrome, which persist from childhood and often require intervention from mental health professionals. Implications for clinical practice are considered.

Copyright

Corresponding author

Mark Davies, School of Psychology. Queen's University of Belfast, David Keir Building, Melone Road, Belfast BT7 INN; Tel: (44) 1232 245 133

References

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Davies, M. T., Howlin, P. A. & Udwin, O. (1997) Independence and adaptive behavior in adults with Williams syndrome. American Journal of Medical Genetics. 70, 188195.
Dilts, C. V., Morris, C. A. & Leonard, C. O. (1990) Hypothesis for development of a behavioural phenotype in Williams syndrome. American Journal of Medical Genetics, suppl. 6, 126131.
Ewart, A. K., Morris, C. A., Atkinson, D., et al (1993) Hemizygosity at the elastin locus in a developmental disorder. Williams syndrome. Nature Genetics, 5, 1116.
Howlin, P. A., Davies, M. T. & Udwin, O. (1998) Cognitive functioning in adults with Williams syndrome. Journal of Child Psychology and Psychiatry, 39, in press.
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Morris, C. A., Leonard, C. O., Dilts, C, et al (1990) Adults with Williams syndrome . American Journal of Medical Genetics, suppl. 6#. 102107.
Sparrow, S. S., Balla, D. & Cichetti, D (1984) Vineland Adaptive Behavior Scales. Circle Pines, MN: American Guidance Service.
Udwin, O. (1990) A survey of adults with Williams syndrome and idiopathic infantile hypercalcaemia. Developmental Medicine and Child Neurology, 32, 129141.
Udwin, O., Yule, W. & Martin, N. (1987) Cognitive abilities and behavioural characteristics of children with idiopathic infantile hypercalcaemia. Journal of Child Psychology and Psychiatry, 28, 297309.
Wechsler, D. (1981) Wechsler Adults Intelligence Scale – (UK edn). London: Psychological Corporation.

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Adults with Williams syndrome: Preliminary study of social, emotional and behavioural difficulties

  • Mark Davies (a1), Patricia Howlin (a1) and Orlee Udwin (a2)
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