title: Behaviour in Cornelia de Lange syndrome: a systematic review.
abstract: Careful study and accurate description of behaviour are important to understand developmental challenges for individuals with Cornelia de Lange syndrome (CdLS). Here we present a systematic review of current understanding of behaviour in CdLS.
  
  
  
  A systematic search was performed for articles published between January 1946 and December 2015 evaluating autism, self-injury, and/or cognition in CdLS. After study-selection, 43 papers were included. The Cochrane quality criteria were adjusted to assign quality scores to the included studies.
  
  
  
  Participants were mostly categorized in the severe/profound developmental level. Methodology and quality were very heterogeneous, as well as reporting occurrence of autism. Self-injurious behaviour was reported in 15 papers. Physical conditions were reported in 21 studies, mostly related to hearing and vision. Only nine studies mentioned details about medication.
  
  
  
  Comparison of presented results was hindered by heterogeneous assessment methods. Improving our understanding of behavioural characteristics in CdLS requires more uniform methodology. We propose a criterion standard of instruments that can ideally be used in assessment of behaviour and development. This will improve understanding of behaviour in the context of developmental level and daily functioning.
authors.affiliation: Autism Team Northern-Netherlands, Jonx Department of Youth Mental Health and Autism, Lentis Psychiatric Institute, Groningen, The Netherlands.
authors.forename: Paul A
authors.identifier: 0000-0001-9489-4022
authors.initials: PA
authors.lastname: Mulder
authors.affiliation: Department of Paediatrics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
authors.forename: Sylvia A
authors.initials: SA
authors.lastname: Huisman
authors.affiliation: Department of Paediatrics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
authors.forename: Raoul C
authors.initials: RC
authors.lastname: Hennekam
authors.affiliation: Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK.
authors.forename: Chris
authors.initials: C
authors.lastname: Oliver
authors.affiliation: Autism Team Northern-Netherlands, Jonx Department of Youth Mental Health and Autism, Lentis Psychiatric Institute, Groningen, The Netherlands.
authors.forename: Ingrid D C
authors.initials: ID
authors.lastname: van Balkom
authors.affiliation: Autism Team Northern-Netherlands, Jonx Department of Youth Mental Health and Autism, Lentis Psychiatric Institute, Groningen, The Netherlands.
authors.forename: Sigrid
authors.initials: S
authors.lastname: Piening
mesh_terms: D016206:Databases, Bibliographic / Q000706:statistics & numerical data; D003635:De Lange Syndrome / Q000150:complications* / Q000523:psychology*; D006801:Humans; D001523:Mental Disorders / Q000209:etiology*; D011788:Quality of Life