Emotion Dysregulation in Fragile X Syndrome

By Mya Jones

Authors:  Rebecca C Shaffer, Debra L Reisinger, Lauren M Schmitt, Martine Lamy, Kelli C Dominick, Elizabeth G Smith, Marika C Coffman, Anna J Esbensen

Summary:

A large portion of individuals with Fragile X Syndrome (FXS) experience the inability to change how strongly they feel an emotional experience or how they respond to the experience, referred to as emotion dysregulation. Cincinnati Children’s Fragile X Center team reviewed the surrounding and relevant research conducted to examine emotion dysregulation in FXS and other genetic disorders commonly associated with intellectual disabilities to better understand the current state of the research.

Findings indicate emotion dysregulation in FXS can include irritability, temper tantrums, crankiness, reactivity, big reactions to small problems, anxiety, and verbal/ physical aggression. Due to the loss of the Fragile X protein, individuals with FXS demonstrate differences in their neural structures and function, including difficulties with sensory processing, social and emotional identification and processing, and executive function. Recent studies suggest that these neural changes can be a risk factor for emotion dysregulation. Additionally, research suggests that hyperexcitability in FXS may be a risk factor for emotion dysregulation.

Research for emotion dysregulation in FXS is relatively new. To date, only one current parent report form is being modified to be valid for use in the assessment of irritability in FXS. Currently, there are no research trials examining behavioral therapy for emotion dysregulation in FXS, but there have been some targeting pharmacological interventions. Atypical antipsychotics have been studied for the treatment of irritability, including the examination of aripiprazole which saw significant clinical improvements on outcomes measures.

Through this review, clear patterns were established, such as the FXS population with comorbid ASD demonstrate more self-injurious behaviors than in populations without the co-morbidities of FXS and ASD. The FXS population that has co-occurring ASD and intellectual disability (ID) showed the highest rates of aggression and irritability in comparison to those without these co-occurrences.

Why this matters:

This review gave us a better look into how emotion dysregulation presents itself in the FXS population, as well as what this experience can look like for some with FXS and comorbid ASD as well as suggestions for next steps to further explore emotion dysregulation in FXS. Further evidence discussed that while there is a wide range in symptoms manifested in ED, the FXS population experienced more anxiety symptoms when emotion dysregulation was present. It also brought up the possible role that emotion dysregulation can play well into adulthood with co-occurring conditions, such as tremor/ataxia syndrome, while more information is needed about the stability of symptoms into adulthood to get a clearer picture. While research into emotion dysregulation in FXS is new, the behaviors and difficulties associated with emotion dysregulation in FXS are not new. Many families cite symptoms like irritability, temper tantrums, crankiness, reactivity, big reactions to small problems, anxiety, and verbal/ physical aggression as major challenges. Understanding emotion dysregulation in FXS will help researchers and clinicians understand what types of treatments may be needed and help with the process of developing those treatments.

Next steps:

Given this review was broad across several genetic disorders commonly associated with intellectual disability, we must thoroughly examine individuals with FXS separately to get a better idea of the full picture of emotion dysregulation in FXS. Further, research needs to be expanded beyond youth with FXS to include the developmental lifespan. Similar to other behavioral challenges in FXS, emotion dysregulation will likely present differently across ages and require different supports or interventions based on age. Additionally, a common definition and assessment tool of emotion dysregulation is needed to better understand the impact of emotion dysregulation in FXS and consistently track outcomes across research trials. Lastly, a needed area of research includes psychological interventions for emotion dysregulation outside of pharmacological trials for related emotion dysregulation symptoms.

FOR MORE DETAILS VISIT:

The full publication of this research can be found here in the Journal of the American Academy of Child & Adolescent Psychiatry.