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

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Lifespan trajectory of affect in Cornelia de Lange syndrome : towards a neurobiological hypothesis. / Groves, Laura; Moss, Joanna; Crawford, Hayley; Nelson, Lisa; Stinton, Chris; Singla, Gursharan; Oliver, Chris.

In: Journal of Neurodevelopmental Disorders, Vol. 11, No. 1, 6, 07.06.2019.

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@article{5e00195e194148c7beda67eedf1ed9b5,
title = "Lifespan trajectory of affect in Cornelia de Lange syndrome: towards a neurobiological hypothesis",
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.",
keywords = "Affect, Cornelia de Lange syndrome, Fragile X syndrome, Mood, Trajectory",
author = "Laura Groves and Joanna Moss and Hayley Crawford and Lisa Nelson and Chris Stinton and Gursharan Singla and Chris Oliver",
year = "2019",
month = "6",
day = "7",
doi = "10.1186/s11689-019-9269-x",
language = "English",
volume = "11",
journal = "Journal of Neurodevelopmental Disorders",
issn = "1866-1955",
publisher = "Springer",
number = "1",

}

RIS

TY - JOUR

T1 - Lifespan trajectory of affect in Cornelia de Lange syndrome

T2 - towards a neurobiological hypothesis

AU - Groves, Laura

AU - Moss, Joanna

AU - Crawford, Hayley

AU - Nelson, Lisa

AU - Stinton, Chris

AU - Singla, Gursharan

AU - Oliver, Chris

PY - 2019/6/7

Y1 - 2019/6/7

N2 - 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.

AB - 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.

KW - Affect

KW - Cornelia de Lange syndrome

KW - Fragile X syndrome

KW - Mood

KW - Trajectory

U2 - 10.1186/s11689-019-9269-x

DO - 10.1186/s11689-019-9269-x

M3 - Article

VL - 11

JO - Journal of Neurodevelopmental Disorders

JF - Journal of Neurodevelopmental Disorders

SN - 1866-1955

IS - 1

M1 - 6

ER -