Dr Hayley Crawford began the June CEDAR seminar by highlighting that autistic people and people with intellectual disabilities are at higher risk of anxiety than the general population. For example, for children and adolescents with intellectual disabilities prevalence rates are 3-21.9% in comparison to 3-6.5% for typically developing children and adolescents.

Dr Crawford’s research aims to improve identification of anxiety in non-verbal or minimally verbal autistic individuals and individuals with intellectual disabilities and gain an understanding of what is underpinning high rates of anxiety in these groups.

In her presentation, Dr Crawford explored topics including identification of anxiety through research using interviews, questionnaires, eye tracking, and behavioural observation, and investigated whether there are certain situations that exacerbate anxiety. This can help find the answer to what might be causing high anxiety as well as whether anxiety can be indicative of other conditions, such as intellectual disabilities.

Behavioural assessment of social anxiety

The first study discussed by Dr Crawford looked at a behavioural assessment of social anxiety and social motivation in individuals with Fragile X, Cornelia de Lange, and Rubinstein-Taybi syndromes. It examined the behaviours associated with social anxiety and social motivation across four social situations: no social interaction, voluntary social interaction, required social interaction, and performance.

The study suggested that certain behaviours reflect social anxiety rather than autistic symptoms. Although eye gaze, vocal length, and time to first utterance may be signs of a lack of social interest, they may be also a response to social anxiety. Social avoidance, discomfort, and negative emotional affect have been found to be indicators of social anxiety as well. These behaviours were unrelated to autism symptomatology in this study.

Dr Crawford also highlighted that in this study, heightened anxiety was seen in people with Fragile X syndrome for all four experimental situations. Social anxiety was displayed with both familiar and unfamiliar people. In people with Cornelia de Lange syndrome, heightened social anxiety may be related to the uncertainty, difficulties planning responses, and, especially with unfamiliar people, executive function impairment.

Dr Crawford continued by talking about the findings of the study on spontaneous discrimination of happy and disgusted facial expressions in autistic people and people with Fragile X syndrome. Looking patterns to the eyes and mouth were also examined. The study revealed that these two groups did not differ in terms of emotion discrimination. However, the participants with Fragile X syndrome looked at the eyes significantly less than the autistic and typically developing participants. Reduced looking at the eyes in individuals with Fragile X syndrome was unlikely to be related to autism symptomatology in this group.

Social attention

The other study by Dr Crawford and colleagues looked at social attention as well as its relationship with anxiety and autism indications. This eye-tracking study compared visual attention to naturalistic social scenes in males with Fragile X syndrome and typically developing children. These two groups did not differ on the overall amount of time spent viewing the background, body or face regions of the actors in the scenes. However, in males with Fragile X syndrome, increased looking at faces was related to heightened anxiety. This might be the case since for the individuals experiencing social anxiety faces may be seen as a more threatening part of the social scene.

Another eye-tracking study showed that autistic adolescents displayed shorter looking-time to social videos in comparison to non-social videos only when an actor or object was moving towards them. The participants in three genetic syndrome groups – Fragile X, Cornelia de Lange, and Rubinstein-Taybi syndromes – demonstrated similar looking-time. However, participants with Fragile X syndrome fixated on the social actor or object that moved toward them quicker. Participants with Cornelia de Lange syndrome fixated on this slower than other groups.

Social avoidance

Dr Crawford also presented the findings of the study on social avoidance in males with Fragile X syndrome from infancy through to young adulthood. Elevated and persistent social avoidance (e.g., reduced eye gaze), in social encounters was revealed in this group. Social avoidance displayed by over 80% of males with Fragile X syndrome was first seen during infancy. It became more intense throughout childhood and stabilized during adolescence and young adulthood. Although social avoidance in Fragile X syndrome is often attributed to autism or anxiety disorders, its profile is more complex.

The findings of the study on infant social avoidance were also mentioned. This was a longitudinal study using direct child assessments and parent-report questionnaires. In males with Fragile X syndrome, increased social avoidance across infancy and preschool predicted increased autism symptom severity, but reduced risk for anxiety. This is an important finding as autism is one of the most common disorders associated with Fragile X syndrome and few studies have identified early markers of autism in individuals with Fragile X syndrome.

Dr Crawford concluded by presenting an avoidance cycle of anxiety in a typical phobia situation. She used an example of a dog phobia in children. When feeling threatened, the child is experiencing physiological signs of anxiety, including hyperarousal. This leads to them being more vigilant for danger and thus, engaging in behaviours to escape any dogs. The avoidance gives them the experience of short-term relief. However, long-term it leads to an increase in anxiety and makes the whole situation repeat.

The way forward

The way forward is working towards being able to pilot an intervention and improve longer-term outcomes. The signs and behaviours may differ between the groups which increases the importance of identification of key markers related to anxiety. Better understanding of anxiety is crucial in developing effective early interventions to reduce it, and targeted support means improved quality of life for the individuals, their families and carers.

In relation to the avoidance cycle talked through by Dr Crawford, the presented topic is interesting since, as it was mentioned by the audience, there are recommended interventions. One of these is exposure therapy which involves slowly exposing the person to what they are scared of in a safe and controlled manner. In situations when the person is exposed to fear or anxiety provoking situations the avoidance is often observed. This avoidance of the feared situation might be elevated in autistic individuals, which leads to less exposure and decreases the chances to overcome the anxiety.

As it was mentioned by Dr Crawford, there has been an inevitable impact of the COVID-19 pandemic. Not only did it affect individuals’ mental health and behaviours but also restricted researchers’ possibilities of data collection. Heightened anxiety, due to the pandemic, in people with intellectual disabilities and their carers sometimes made researchers not able to collect data.

As the audience suggested, although this research would not necessarily change clinical assessment and case management from the clinical perspective, it is important to consider diagnostic overshadowing. For clinicians who are familiar with anxiety in people with intellectual disabilities, the discussed studies provide useful reminders about differences in presented behaviours that might indicate anxiety.

It is helpful to recognise that when it comes to anxiety-related behaviours, we should not necessarily expect facial expressions or focus on avoidance. Being aware that physiological markers might be signs of anxiety helps to overcome sometimes wrongly made assumptions that the person’s behaviour is part of their disability. Raising awareness about misattributing the behaviours indicating anxiety to other problems or conditions might help identify their anxiety, improve the care they receive, and enhance their wellbeing.

Dr Hayley Crawford Hayley leads the Neurodevelopmental Conditions Research @ WMS Lab at the University of Warwick which focusses on research into clinical outcomes for children and adults with neurodevelopmental disorders. Hayley is a Specialist Advisor to the Fragile X Society and Co-Director of the Cerebra Network for Neurodevelopmental Disorders.