Table 4 Dysregulated behavior cluster recommendations

DB1. Dysregulated behaviors are common in TSC, have a major impact on the family, and require a systematic approach. Dysregulated behaviors, including aggression, temper tantrums, and self-injury, are common in individuals with TSC and can have a major impact on the family. Most of the time, dysregulated behaviors have a “meaning” or “function” and are not due to the individual being intentionally difficult. Many behaviors have specific triggers, purposes, or are reinforced by the responses of others to the behavior. Caregivers should therefore be supported to receive a systematic and comprehensive evaluation to understand the dysregulated behaviors

DB2. Understand the meaning/function of dysregulated behaviors to guide intervention. There may be many different reasons why individuals with TSC may present with dysregulated behaviors. These include (but are not limited to) difficulties with communication, overactive and impulsive behaviors, mood and anxiety problems, sensory sensitivities, avoidance of demand, cognitive inflexibility, or pain. The intervention plan for each of these reasons may be very different. For this reason, caregivers should be supported to complete a functional behavioral assessment to identify antecedents and consequences of specific behaviors as a way of understanding the reason, function, or meaning of a specific dysregulated behavior

DB3. Understand the intellectual and neuropsychological profile to inform the meaning/function of dysregulated behaviors and to guide intervention. Ensure a good understanding of the intellectual ability and neuropsychological profile of an individual with dysregulated behaviors as these may inform the reason, function, or meaning of such behaviors. Where necessary, perform formal assessments to evaluate current intellectual, language/communication, attention, executive, memory, and visuospatial skills so that goals and support can be adapted appropriately for each individual. Provide accommodations and resources to optimize communication (e.g., hand signs, picture cards, devices/apps) and other skills as required

DB4. Perform urgent physical work-up for sudden onset of dysregulated behaviors and/or rapid change in behaviors. Sudden onset or change in behaviors may indicate an underlying medical problem, including (but not limited to) seizures, subependymal giant cell astrocytoma (SEGA), physical illnesses, or adverse effects of medications. Look out for new or alarming physical or neurological symptoms such as lethargy, confusion, vomiting, or physical pain, and perform urgent medical/neurological workup to identify potential physical causes of dysregulated behaviors

DB5. Use non-pharmacological intervention strategies as first-line treatment for dysregulated behaviors. Once a functional analysis of behaviors has been performed and underlying medical causes for behaviors have been ruled out, non-pharmacological strategies should be implemented as a first-line treatment. These are determined by the meaning or function of the behavior, and typically include communication, behavioral, developmental, and environmental strategies. These non-pharmacological strategies are often more effective than medication and should always be part of treatment plans

DB6. Medications should only be used for dysregulated behaviors after a careful systematic evaluation and always alongside a non-pharmacological intervention plan. There are no medication strategies that will improve dysregulated behaviors in isolation. Where medications are considered, they should always be integrated in a broader management plan that includes non-pharmacological approaches. Medications should always be prescribed within the marketing authorization of the drug, for as short a period as possible, and with clear consent from the individuals with TSC or caregivers

DB7. Research is required to generate a TSC-specific evidence base for non-pharmacological interventions of dysregulated behaviors. There is a gap in research for TSC-specific behavioral and other non-pharmacological interventions. In the interim, we recommend the use of guidelines for the management of dysregulated behaviors in the general population