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

Self-injurious and aggressive behaviours

Self-injurious behaviour refers to non-accidental behaviours that have the potential to cause damage, such as reddening of the skin, bruising, bleeding, hair loss, etc. Self-injurious behaviour is common in individuals with CdLS and includes behaviours such as self-hitting, head banging or self-biting (156), although it is not an inevitable consequence of the syndrome. Some behaviours shown in CdLS can be identical to self-injurious behaviour but do not cause any bodily damage. These behaviours may develop into self-injurious behaviour over time (157).

There are several risk markers for self-injurious behaviours in CdLS. Individuals with more severely impaired cognitive abilities, communication skills and adaptive behaviours are more likely to display self-injurious behaviour. Risk markers may also include CdLS caused by an NIPBL gene mutation, and increased levels of impulsivity, repetitive behaviours and characteristics associated with autism spectrum disorder (156).

Approximately half of individuals with CdLS display clinically significant self-injurious behaviour (158). Usually this is directed towards the individual's hands (159). Self-injurious behaviour can result in physical injury, the severity of which is dependent on the amount of damage and functional loss (156). Sometimes it may be necessary to use restraints to prevent permanent damage (107).

Self-injurious behaviour in CdLS may be a sign of or response to pain and has been associated with common medical conditions in CdLS, such as gastrointestinal problems, ear infections, constipation, dental disease or hip problems. It is important that the cause of self-injurious behaviour in individuals with CdLS be identified. This often requires medical assessment to specifically look for the sources of pain, as well as behavioural assessment and consideration of the individual’s environment. Treatment or intervention strategies can then be matched to the function of self-injurious behaviour. Treatment should include both medical and behavioural strategies (R54, R55).

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