Lifespan trajectory of affect in Cornelia de Lange syndrome: towards a neurobiological hypothesis

Research output: Contribution to journalArticle

Authors

  • Hayley Crawford
  • Lisa Nelson
  • Gursharan Singla

Colleges, School and Institutes

External organisations

  • Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Edgbaston, B15 2TT, UK. grovesl@bham.ac.uk.
  • Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Edgbaston, Birmingham, B15 2TT, UK. Electronic address: kate.eden1@gmail.com.
  • Institute of Cognitive Neuroscience, University College London, 17 Queen Square, London, WC1N 3AR, UK.
  • Centre of Applied Biological and Exercise Sciences, Faculty of Health and Life Sciences, Coventry University, Coventry CV1 5FB, United Kingdom.
  • Department of Obstetrics and Gynaecology, Royal Derby Hospital, Uttoxeter Road, Derby DE22 3NE, United Kingdom.
  • Warwick Medical School, University of Warwick, Coventry, United Kingdom

Abstract

BACKGROUND: Depressive symptomology and low affect are comparatively common in individuals with genetic disorders such as Cornelia de Lange syndrome. However, lifespan trajectories and associated person characteristics have not been examined. In this study, the trajectories for affect and associated behavioural characteristics were investigated in individuals with Cornelia de Lange syndrome with individuals with fragile X syndrome (FXS) comparable for chronological age and total number of behavioural indicators of ASD included for the purpose of contrast.

METHODS: A 7-year longitudinal study of affect (mood, interest and pleasure) was conducted in individuals with CdLS (n = 44) and FXS (n = 95). The trajectories of low affect were explored, as well as associations between Time 1 behavioural characteristics and affect at Time 1 and Time 3 (7 years later).

RESULTS: The CdLS group were lower in mood than the FXS group overall (p < .001). Interest and pleasure scores showed a significant decline over the lifespan for individuals with CdLS (p < .001) but not the FXS group. Lower level of ability at Time 1 was associated with lower mood at Time 1 and Time 3 in the FXS group only. Higher levels of ASD symptomology at Time 1 were associated with low mood and interest and pleasure in both syndrome groups at Time 1 and Time 3. Greater insistence on sameness at Time 1 was associated with lower mood at Time 1 in the FXS group and lower interest and pleasure at Time 1 and Time 3 in the CdLS group.

CONCLUSIONS: Low affect in specific genetic syndromes may be associated with differing lifespan trajectories and behavioural profiles. Specifically, individuals with CdLS appear at risk for experiencing declines in levels of interest and pleasure whereas individuals with FXS show no significant change in the level of affect with age.

Details

Original languageEnglish
Article number6
JournalJournal of Neurodevelopmental Disorders
Volume11
Issue number1
Publication statusPublished - 7 Jun 2019

Keywords

  • Affect, Cornelia de Lange syndrome, Fragile X syndrome, Mood, Trajectory