Dysregulated behaviors, including aggression, temper tantrums, and self-injury, represent the greatest concerns and burdens to families who live with TSC. These behaviors are therefore a common reason for referral to specialist services. However, there may be many different reasons for or “pathways” to dysregulated behaviors in TSC.

For example, they may be driven by communication difficulties, impulsivity, anxiety, sensory sensitivities, demand avoidance, cognitive inflexibility, trauma, and/or pain. Dysregulated behaviors may also emerge because of the physical manifestations of TSC, such as growing SEGA, seizures, or as an adverse effect of medications.

For this reason, there is no single intervention approach to this cluster of difficulties, and, equally, no single or simple medication that should be used to “manage” these behaviors. There are also no behavioral treatment studies for dysregulated behavior specifically in people with TSC. However, there is moderate support for real interventions for individuals with intellectual disabilities without TSC, which should inform practice.

Non-pharmacological/behavioral interventions may include speech/language work to support communication difficulties, cognitive-behavioral work to support anxiety behaviors and cognitive inflexibility, a range of sensory strategies to support the sensory sensitivities that may trigger dysregulated behaviors, and a range of environmental strategies such as visual schedules to increase predictability and support transitions during daily activities.

The consensus panel recommends the following:

Dysregulated behavior cluster recommendations

  • Dysregulated behaviors are common in TSC, have a major impact on the family and require a systematic approach.
  • It is important to understand the meaning/function of dysregulated behaviors to guide intervention.
  • It is also important to understand the intellectual and neuropsychological profile to inform the meaning/function of dysregulated behaviors and to guide intervention.
  • Perform urgent physical work-up for sudden onset of dysregulated behavior and/or rapid change in behaviors.
  • Use non-pharmacological intervention strategies as first-line treatment for dysregulated behaviors.
  • Medications should be used after a careful systematic evaluation and always alongside a non-pharmacological intervention plan.
  • Research is required to generate a TSC-specific evidence base for non-pharmacological interventions of dysregulated behaviors.
Reviewed by Ashley Pounders, MSN, FNP-C, November 2023.

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