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Affective functioning and social cognition in Noonan syndrome

Published online by Cambridge University Press:  11 July 2011

E. Wingbermühle ,
J. I. M. Egger ,
W. M. A. Verhoeven ,
I. van der Burgt  and
R. P. C. Kessels
E. Wingbermühle*
Affiliation:
Vincent van Gogh Institute for Psychiatry, Venray, The Netherlands Donders Institute for Brain, Cognition and Behaviour, Centre for Cognition, Radboud University Nijmegen, The Netherlands
J. I. M. Egger
Affiliation:
Vincent van Gogh Institute for Psychiatry, Venray, The Netherlands Donders Institute for Brain, Cognition and Behaviour, Centre for Cognition, Radboud University Nijmegen, The Netherlands Behavioural Science Institute, Radboud University Nijmegen, The Netherlands
W. M. A. Verhoeven
Affiliation:
Vincent van Gogh Institute for Psychiatry, Venray, The Netherlands Department of Psychiatry, Erasmus University Medical Centre, Rotterdam, The Netherlands
I. van der Burgt
Affiliation:
Department of Human Genetics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
R. P. C. Kessels
Affiliation:
Vincent van Gogh Institute for Psychiatry, Venray, The Netherlands Donders Institute for Brain, Cognition and Behaviour, Centre for Cognition, Radboud University Nijmegen, The Netherlands Department of Medical Psychology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
*
*Address for correspondence: E. Wingbermühle, M.Sc., Centre of Excellence for Neuropsychiatry, Vincent van Gogh Institute for Psychiatry, Stationsweg 46, 5803 AC Venray, The Netherlands. (Email: [email protected])
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Abstract

Background

Noonan syndrome (NS) is a common genetic disorder, characterized by short stature, facial dysmorphia, congenital heart defects and a mildly lowered IQ. Impairments in psychosocial functioning have often been suggested, without, however, systematic investigation in a clinical group. In this study, different aspects of affective processing, social cognition and behaviour, in addition to personal well-being, were assessed in a large group of patients with NS.

Method

Forty adult patients with NS were compared with 40 healthy controls, matched with respect to age, sex, intelligence and education level. Facial emotion recognition was measured with the Emotion Recognition Task (ERT), alexithymia with both the 20-item Toronto Alexithymia Scale (TAS-20) and the Bermond–Vorst Alexithymia Questionnaire (BVAQ), and mentalizing with the Theory of Mind (ToM) test. The Symptom Checklist-90 Revised (SCL-90-R) and the Scale for Interpersonal Behaviour (SIB) were used to record aspects of psychological well-being and social interaction.

Results

Patients showed higher levels of cognitive alexithymia than controls. They also experienced more social distress, but the frequency of engaging in social situations did not differ. Facial emotion recognition was only slightly impaired.

Conclusions

Higher levels of alexithymia and social discomfort are part of the behavioural phenotype of NS. However, patients with NS have relatively intact perception of emotions in others and unimpaired mentalizing. These results provide insight into the underlying mechanisms of social daily life functioning in this patient group.

Keywords

Alexithymiabehavioural phenotypeemotion perceptiongeneticsneuropsychology
Type
Original Articles
Information
Psychological Medicine , Volume 42 , Issue 2 , February 2012 , pp. 419 - 426
Copyright
Copyright © Cambridge University Press 2011

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