Literature DB >> 29536582

Attenuated behaviour in Cornelia de Lange and fragile X syndromes.

L Bell1, C Oliver2, A Wittkowski3, J Moss2, D Hare4.   

Abstract

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.
© 2018 The Authors. Journal of Intellectual Disability Research published by MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disibilities and John Wiley & Sons Ltd.

Entities:  

Keywords:  attenuated behaviour; autism; catatonia; cornelia de lange syndrome; fragile X; movement disorder

Mesh:

Year:  2018        PMID: 29536582     DOI: 10.1111/jir.12481

Source DB:  PubMed          Journal:  J Intellect Disabil Res        ISSN: 0964-2633


  1 in total

1.  Repetitive and Self-injurious Behaviors in Children with Cornelia de Lange Syndrome.

Authors:  Siddharth Srivastava; Bennett Clark; Colleen Landy-Schmitt; Elizabeth A Offermann; Antonie D Kline; Samuel T Wilkinson; Marco A Grados
Journal:  J Autism Dev Disord       Date:  2021-05
  1 in total

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