Behavior and Anxiety in Angelman Syndrome

Quick Overview

This discussion explores behavioural issues and anxiety in individuals with Angelman Syndrome (AS). Behavioural problems are common in AS, varying in frequency and severity based on the molecular subtype. Common issues include mouthing behaviour, short attention span, and aggression. Anxiety is also prevalent, presenting differently from typical anxiety disorders. Observable behaviours associated with anxiety include irritability, agitation, and restlessness. Understanding these issues is crucial for developing effective strategies, such as communication supports, sensory interventions, and environmental modifications. In severe cases, medication may be used. Sleep disturbances and pica are also common in AS, and can be managed with medication and occupational therapy. Consistency is key in behaviour management, and working with behavioural specialists can help develop strategies. Managing specific behaviours involves redirection, positive reinforcement, and providing distractions. Understanding these issues is essential for developing effective interventions, improving the overall well-being of individuals with AS.


In this panel discussion, we explore the common behavioral issues and anxiety experienced by individuals with Angelman Syndrome (AS). We delve into the frequency, severity, and underlying causes of behavior problems, as well as strategies for managing them. Additionally, we examine the prevalence of anxiety in individuals with AS and discuss effective interventions.

Behavior Problems in Angelman Syndrome

Behavior problems are prevalent in individuals with AS and can significantly impact their social interactions. The frequency and severity of these problems vary based on the molecular subtype. According to reports from parents, the most common behavior problems include mouthing behavior, short attention span, and aggressive behaviors. As individuals with AS age, irritability and hyperactivity tend to increase, particularly in those with non-deletion.

Anxiety in Angelman Syndrome

Anxiety is a highly penetrant clinical feature in AS, although it may present differently from typical anxiety disorders. Individuals with non-deletion are more prone to anxiety compared to those with deletion. Observable behaviors associated with anxiety in AS include irritability, agitation, restlessness, repetitive behaviors, crying, aggression, and somatic concerns. Possible sources of anxiety in AS include separation from a preferred caregiver, new or unexpected situations, exceeding ability levels, and sensory overload.

Understanding and Addressing Behavior Problems

Understanding the underlying causes of behavior problems is crucial for developing effective strategies. Functional behavior analysis can help identify triggers and consequences of behavior. Strategies for addressing behavior concerns include communication supports, sensory interventions, environmental modifications, and behavioral therapy. In severe cases that do not respond to behavioral interventions, medication can be considered.

Medication for Behavior and Anxiety Issues

Medication can be used to address severe behavior and anxiety issues in individuals with AS. Serotonin reuptake inhibitors, buspirone, antipsychotics, and antiseizure medications are commonly prescribed. However, it is important to work closely with a clinician to find the right medication and dosage, as well as to monitor for side effects. Stimulant medications like Ritalin and Adderall are generally not effective for hyperactivity in individuals with AS and should be used cautiously due to side effects. Serotonergic medications may help with hyperactivity and are generally better tolerated. Antipsychotic medications may be considered as a last resort for severe hyperactivity accompanied by irritability, but their side effects should be carefully considered.

Sleep Disturbances and Pica

Sleep disturbances are common in individuals with AS, and medications such as melatonin, clonidine, and trazodone can help regulate sleep. Pica, the ingestion of non-food items, can be related to constipation, medication side effects, or sensory-seeking behaviors. Occupational therapy consultation may be beneficial in managing pica.

Managing Anxiety and Agitation

For anxiety and agitation on an as-needed basis, medications like scopolamine, lorazepam, propranolol, and Benadryl can be considered. Consistency is key in behavior management, but it can be challenging to maintain consistency outside of the home. Working with behavioral specialists, such as ABA therapists or psychologists, can help develop strategies and involve other caregivers and educators. Accessing ABA therapy for individuals with AS who do not have an autism diagnosis may be difficult, but referrals to behavioral psychologists or therapists with expertise in working with individuals with intellectual disabilities can be helpful.

Strategies for Specific Behavior Issues

Managing aggressive behaviors in young children with AS involves redirecting, using positive reinforcement, and providing distractions. Consistency and involving other caregivers and educators in behavior plans are crucial. Teeth grinding, often a sensory-seeking behavior, can be managed through strategies such as using a chewy item, introducing alternative movements like clicking, and implementing a behavioral reward system.

In conclusion, understanding the behavioral issues and anxiety experienced by individuals with AS is essential for developing effective interventions. By employing a combination of behavioral strategies and medication, individuals with AS can lead more fulfilling lives and improve their overall well-being.

Talk details

  • Title: Behavior and Anxiety in AS
  • Author(s): Christopher Keary, Anne Wheeler, Anjali Sadhwani, Cesar Ochoa-Lubinoff
  • Author(s)’ affiliation: Massachusetts General Hospital; RTI International; Boston Children’s Hospital; Rush University Medical Center
  • Publication date: 2022-08-17
  • Collection: 2022 ASF Family Conference