Dravet behavior, social issues emerge in toddlerhood, study finds
Challenges may emerge before age 3, worsen through early childhood
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Behavioral and social difficulties are common in young children with Dravet syndrome, often emerging in toddlerhood and worsening through early childhood, according to a natural history study.
The ENVISION (NCT04537832) study followed 58 children with Dravet syndrome, aged 6 months to 5 years, for up to two years. Results showed that attention issues, hyperactivity, aggressive behavior, social withdrawal, autism-like traits, and difficulties with socializing often emerged before age 3 and became clinically significant between ages 3 and 4.
Children with more severe behavioral difficulties were also more likely to show greater overall developmental delays, poorer communication skills, and more difficulty engaging socially.
“These data reshape the clinical picture of [Dravet syndrome] by establishing that the window for behavioral and social–emotional intervention opens well before school age,” the researchers wrote, emphasizing “the importance of early diagnosis to enable targeted management … of behavioral aspects and promote language and cognitive interventions early in the course of [the disease].”
The study, “Characterizing early behavioral and social–emotional problems in young children with SCN1A+ Dravet syndrome: Findings from the ENVISION prospective natural history study,” was published in Epilepsia.
Looking beyond diagnosed issues
Dravet syndrome is caused most often by mutations in the SCN1A gene. It is characterized by prolonged, hard-to-control seizures that begin in the first year of life and often persist despite treatment with antiseizure medications.
Families frequently report that behavioral and social-emotional difficulties can be as challenging as seizures. And while seizures often follow a more stable pattern after early childhood, behavioral problems may worsen as children approach adolescence, significantly affecting quality of life.
Previous research has mainly focused on formally diagnosed psychiatric conditions, showing that clinically significant behavioral difficulties are common in school-aged children and adults with Dravet. The researchers said this approach may overlook a broader range of behavioral issues that do not meet criteria for a specific diagnosis but may still meaningfully affect social and emotional development and quality of life.
To help address this gap, they followed 58 young children with Dravet syndrome caused by SCN1A mutations who were enrolled in the ENVISION study at 16 sites worldwide.
The children were evaluated every three months for up to two years using standardized questionnaires completed by their caregivers to assess behavior, emotions, and social development. At enrollment, 81% of participants had mild or no developmental delays.
By preschool age, many children had symptoms severe enough to be considered clinically significant. Common difficulties included problems with attention, hyperactivity, aggressive or oppositional behaviors, social withdrawal, and traits associated with autism.
In contrast, emotional problems like anxiety or depression were uncommon at this young age and showed little change over time.
Social development showed a similar trajectory. Social skills were below what would be expected for children of the same age at the study’s start and declined further behind age norms over time, particularly in younger children. Many had persistent trouble interacting with peers, understanding social cues, and managing everyday social situations.
Behavioral issues were significantly linked to children’s communication abilities, overall developmental status, and social functioning. This means that children with more severe behavioral problems were more likely to have weaker language skills, greater developmental delays, and more difficulty engaging socially.
In contrast, seizure burden showed little to no consistent relationship with behavioral or social-emotional difficulties.
When researchers accounted for multiple factors, age was the strongest predictor of worsening behavior. As children grew older, issues related to attention, hyperactivity, aggression, and social difficulties tended to increase, regardless of sex, type of SCN1A mutation, seizure characteristics, or antiseizure medication.
The results highlight “the need to diagnose and manage” behavioral and social challenges in children with Dravet early, the researchers wrote, adding that “left unaddressed, these early deficits contribute to greater challenges as children grow, potentially impacting long-term social–emotional development.”
