Behavior and Anxiety in Angelman Syndrome

Quick Overview

In this panel discussion on Behavior and Anxiety in Angelman Syndrome, Dr. Anjali Sadhwani, Dr. Anne Wheeler, and Dr. Chris Keary provide an overview of the challenging behaviors commonly seen in individuals with Angelman Syndrome. They discuss the functions of behavior and the importance of conducting a functional behavior analysis to understand the triggers and consequences of behavior. The panelists also highlight the prevalence of behavior problems based on molecular subtype, with differences observed in anxiety, aggression, hyperactivity, and attention span. They emphasize the need for behavioral strategies, such as identifying the function of behavior, implementing sensory interventions, and using visual schedules and timers. Additionally, they discuss the role of medications in addressing behavior problems, including the use of serotonin-based medications for anxiety, as-needed medications for aggression, and non-stimulant medications for hyperactivity. The panelists also touch on the impact of sleep problems on behavior and provide strategies for addressing sleep challenges. They conclude by announcing a forthcoming study to gather more information on effective strategies and medications for behavior and anxiety in Angelman Syndrome.

Good afternoon, I’m Dr. Anjali Sadhwani, and I’m pleased to be here with Dr. Anne Wheeler and Dr. Chris Carey to discuss the Behavior and Anxiety panel. In this session, we will provide an overview of challenging behaviors in Angelman syndrome and discuss both behavioral and medical strategies to address these behaviors.

Common Behavior Problems in Angelman Syndrome

Behavior problems are common in individuals with Angelman syndrome and can interfere with their ability to interact well in social environments. The frequency and severity of behavior problems can vary based on the molecular subtype. The most common behavior problems include mounting behaviors, aggression, irritability, hyperactivity, and sleep disturbances.

Understanding the Functions of Behavior

It is important to understand the functions of behavior in order to develop effective strategies for managing challenging behaviors. All behavior serves a function, which can include escape, attention-seeking, obtaining something tangible, or seeking sensory input. In a study, parents reported that communication was the main reason for disruptive behaviors, followed by attention-seeking, sensory input, and avoidance.

Conducting a Functional Behavior Analysis

A functional behavior analysis, also known as an ABC analysis, can help identify the antecedents, behaviors, and consequences of challenging behaviors. This analysis helps determine the triggers and context of the behavior, as well as the function it serves. Understanding the function of the behavior is crucial for developing appropriate strategies to address it.

Behavioral Strategies for Challenging Behaviors

Behavioral strategies can be effective in managing challenging behaviors in Angelman syndrome. These strategies include:

  • Ignoring or redirecting undesirable behavior while giving attention to desired behavior
  • Teaching and reinforcing appropriate attention-seeking behaviors
  • Providing frequent opportunities and breaks for individuals who seek escape
  • Using alternative communication methods, such as AAC devices, to address communication needs
  • Working with an occupational therapist to identify calming inputs for sensory concerns
  • Implementing environmental modifications, such as consistent routines and visual schedules, to reduce challenging behaviors

Medical Strategies for Challenging Behaviors

While behavioral interventions are often the first line of treatment for behavior problems, medications can also be considered in more severe cases. Medications commonly used for anxiety, such as buspirone and selective serotonin reuptake inhibitors (SSRIs), can be helpful. As-needed medications like benzodiazepines or propranolol can also be used for anxiety-related behaviors.

For aggression and irritability, medications like antipsychotics (risperidone, aripiprazole) or mood stabilizers (valproic acid) may be considered. Stimulant medications like methylphenidate or amphetamines can be used for hyperactivity and inattention, but non-stimulant medications like guanfacine or clonidine are often preferred in individuals with Angelman syndrome due to their lower risk of side effects.

Addressing Sleep Challenges

Sleep problems are common in individuals with Angelman syndrome. Strategies for addressing sleep challenges include:

  • Establishing consistent bedtime routines and sleep schedules
  • Creating a quiet and cool sleep environment
  • Using calming sensory interventions, such as chewy tubes or fidget toys
  • Considering the use of melatonin or other sleep medications under medical supervision

Participate in Research

We are conducting a study to better understand behavior and anxiety in Angelman syndrome. We are seeking participants for interviews and surveys to gather information about challenging behaviors and effective strategies. If you are interested in participating, please reach out to us for more information.

In conclusion, behavior problems and anxiety are common in Angelman syndrome, but there are effective strategies and medications available to help manage these challenges. By understanding the functions of behavior and implementing appropriate interventions, individuals with Angelman syndrome can improve their quality of life and social interactions.

Talk details

  • Title: Behavior and Anxiety in Angelman Syndrome
  • Author(s): Anjali Sadhwani, Anne Wheeler, Chris Keary
  • Author(s)’ affiliation: Boston Children’s Hospital; RTI International; Massachusetts General Hospital for Children
  • Publication date: 2023-07-07
  • Collection: 2023 ASF Virtual Conference