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Attenuated behaviour in Cornelia de Lange and fragile X syndromes

Bell, L., Oliver, C., Wittkowski, A., Moss, J. and Hare, Dougal 2018. Attenuated behaviour in Cornelia de Lange and fragile X syndromes. Journal of Intellectual Disability Research 62 (6) , pp. 486-495. 10.1111/jir.12481

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Background: Catatonia‐like presentations in people with autism have been increasingly recognised within research and diagnostic guidelines. The recently developed Attenuated Behaviour Questionnaire has identified that attenuated behaviour [autistic catatonia] is very prevalent in people with autism spectrum disorders (ASDs) and associated with repetitive behaviour. In the current study, we investigated attenuated behaviour within two genetic syndromes associated with ASD and examined ASD and repetitive behaviour as longitudinal predictors of attenuated behaviour. Method: The Attenuated Behaviour Questionnaire was completed by parents/carers of 33 individuals with Cornelia de Lange syndrome (CdLS) and 69 with fragile X syndrome (FXS). Information collected from the same informants 4 years previously was utilised to examine ASD and repetitive behaviour as predictors of later attenuated behaviour, controlling for age, gender and ability. Results: Catatonia‐like attenuated behaviour was reported for individuals with CdLS (30.3%) and FXS (11.6%). Slowed movement was more prevalent in people with CdLS. No other phenotypic differences were observed. Across the two groups, repetitive behaviour predicted the presence of attenuated behaviour 4 years later, after controlling for age, gender and ability. Conclusions: Attenuated behaviour can be identified in individuals with CdLS and FXS and may have an effect on both adaptive behaviour and quality of life. Repetitive behaviours predicted subsequent risk within both groups and should be assessed by services as part of a pro‐active strategy of support.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Psychology
Publisher: Blackwell Publishing
ISSN: 0964-2633
Date of First Compliant Deposit: 21 March 2018
Date of Acceptance: 5 February 2018
Last Modified: 28 Jul 2020 01:24

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