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


Anxiety is common in individuals with CdLS. Anxiety in CdLS usually presents as social anxiety (excessive worry about daily life events with no obvious reason for worry), separation anxiety (excessive fear about separation from home or a parent/carer) or selective mutism (not speaking in social situations where there is an expectation for speaking, e.g. at school) (168,148).

Anxiety in CdLS can lead to increased repetitive behaviour, mood-related symptoms or disruptive, aggressive and self-injurious behaviours (109). It is important that any intervention targeting problematic repetitive behaviour in an individual with CdLS be sensitive to anxiety, sensory problems and social demands. Interventions should also consider environmental factors (R59).

Social interactions can also provoke anxiety in individuals with CdLS and lead to behavioural responses that can be observed, such as fidgeting, avoiding eye gaze and active avoidance (152,175). Individuals with CdLS often have an increased preference for sameness, meaning many individuals have difficulty adjusting to changes in their routine. This can make transitional periods more challenging and provoke anxiety (148,162,174). During transitional periods, plans can be put in place to help individuals adjust to changes and reduce levels of anxiety (R61).

Assessing anxiety in CdLS can be difficult, particularly in individuals who display challenging behaviour such as self-injury, aggression, shouting and screaming (137). Often, anxiety and mood disorders can be identified by observing behavioural changes in individuals with CdLS (R60). Treatment of anxiety and mood disorders may include psychosocial interventions (therapy to improve health and well-being) and/or medication (R62).

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