» What are the Cognitive and Behavioural Characteristics of Cornelia de Lange Syndrome

Summary section

Cognitive and behavioural recommendations:

R50: Hyper- and hyposensitivity and other sensory processing difficulties should be assessed, and support strategies should be implemented in individuals with CdLS throughout their lifespan.

R51: Increasing adaptive skills to enhance independence should remain a focus throughout the lifespan and should include personalised specific goals and teaching strategies.

R52: Additional developmental and educational support should be provided to individuals with CdLS to reach their maximum cognitive and educational potential, taking into account their specific cognitive impairments.

R53: Cognitive strengths and weaknesses of individuals with CdLS should be assessed in order to design educational and interventional strategies.

R54: To identify the cause of self-injurious behaviour in individuals with CdLS, medical assessment, specifically looking for sources of pain, should be followed by behavioural assessment of self-restraint then functional analysis.

R55: Treatment of self-injurious behaviour should include both medical and behavioural strategies.

R56: A clinical diagnosis of autism spectrum disorder (ASD) should be considered in all individuals with CdLS throughout life, taking into account atypical presentations.

R57: In addition to standardised ASD diagnostic tools, fine-grained observations should be carried out to accurately define the profile of social functioning in an individual with CdLS.

R58: ASD-specific interventions should be considered in all individuals with CdLS in combination with approaches that consider the broader social functioning profile of the syndrome.

R59: Interventions targeting problematic repetitive behaviour in individuals with CdLS should be sensitive to anxiety, sensory problems and social demands. These interventions should also consider environmental factors.

R60: Atypical presentation of anxiety and mood disorder should be considered when behaviour changes occur.

R61: As anxiety is common in individuals with CdLS during periods of environmental change/transitions, a planned program should be implemented.

R62: Treatment of anxiety and mood disorders in individuals with CdLS should be considered using psychosocial interventions (therapies) and pharmacotherapy (medication).

R63: When assessing communication, vision and hearing problems, speech impairments, intellectual disability, difficulties in social interaction and social anxiety should be considered. Video observations can be very useful.

R64: Developmentally appropriate communication strategies (such as speech therapy, augmented communication input) should be implemented within the first 18 months of life.

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