Social Anxiety

Social Anxiety in Cornelia de Lange Syndrome

Evidence suggests there is heightened probability of shyness, social anxiety (worry) and selective mutism in Cornelia de Lange syndrome in comparison to other syndrome groups.

Social interactions can lead to noticeable behaviours such as fidgeting, avoiding eye contact, and trying to avoid social situations altogether.

Shyness and selective mutism

 You can hear parents’ accounts of these difficulties in the following video:

Research at the University of Birmingham (United Kingdom) has indicated that individuals with Cornelia de Lange syndrome group showed similar levels of sociability to individuals with autism in unfamiliar social situations.  People with autism typically have difficulties with social interaction.  Parents and carers reported significantly more shyness in individuals with Cornelia de Lange syndrome in unfamiliar social situations when compared to three other genetic syndromes (Angelman, Down and Rubinstein-Taybi syndromes).

Extreme shyness appeared characteristic of individuals with Cornelia de Lange syndrome, with between one and three children out of 10 showing extreme shyness in four unfamiliar situations.

The American Psychiatric Association (APA) describes selective mutism as a “consistent failure to speak in specific social situations (in which there is an expectation for speaking, e.g., at school) despite speaking in other situations”.  This lack of speech must last for at least one month, have an impact on achievement at school or work or affect social communication.  The onset of selective mutism typically occurs before five years of age and for some it may only last a few months, whilst for others, it can persist for a number of years.

Estimates from research suggest that approximately four out of ten children in the Cornelia de Lange syndrome group were selectively mute [2]. In contrast, in children without a genetic syndrome or intellectual disability, selective mutism is fairly rare; prevalence rates are between .03% and 2%.

Are shyness and selective mutism related to age?

Individuals with Cornelia de Lange syndrome aged between 12-18 years were reported to initiate social interaction less frequently with unfamiliar people than the under 12 years age group.

The results suggest a change in the age-related pattern of sociability with unfamiliar individuals in Cornelia de Lange syndrome.  Further investigations are needed to understand the unusually high prevalence of selective mutism in Cornelia de Lange syndrome.

A study conducted by Dr Hayley Crawford at the University of Birmingham (United Kingdom) looked at social differences in individuals with fragile X, Cornelia de Lange, and Rubinstein-Taybi syndrome.  To read more about this project click here.

Other insights into anxiety in Cornelia de Lange syndrome

Researchers at the University of Birmingham (United Kingdom) discovered that most individuals with CdLS display behaviour aligning with at least one type of anxiety, with symptoms remaining generally consistent throughout an individual’s life.

Difficulties with uncertainty, routine changes and responses to sensory stimuli (e.g. loud noises) can also trigger anxiety. To cope, some individuals with CdLS develop a strong preference for sameness and routines, which helps them to manage daily life.

Anxiety regarding uncertainty, routine changes and sensory stimuli appears to manifest similarly in CdLS to in autistic individuals.

Typical indicators of anxiety in people with CdLS involve avoiding situations and seeking reassurance from a familiar adult.

Evidence suggests that younger individuals with CdLS and those who struggle with everyday tasks show more behaviours associated with anxiety and greater responses to stress.

A study exploring anxiety in individuals with genetic syndromes, specifically fragile X syndrome (FXS) and CdLS, discovered that individuals with FXS and CdLS display higher levels of cortisol (a stress hormone) when experiencing anxiety compared to individuals without the syndrome.