Rationale:
Developmental and epileptic encephalopathies (DEE), including Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS), are marked by early-onset, pharmacoresistant seizures and a progressive decline in cognitive, behavioral, and executive domains.
This study aimed to characterize and compare executive function profiles in children with LGS and DS through the Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2).
Methods:
An observational, cross-sectional, and analytical design was applied to a cohort of 36 patients aged 6–17 years (32 with LGS and 4 with DS). Caregivers served as informants, rating their children’s executive functioning in daily life contexts.
Results:
Findings revealed that patients with LGS showed pronounced executive impairments, most notably in emotional and behavioral regulation, with clinically significant elevations across all BRIEF-2 indices. Conversely, patients with DS displayed a more heterogeneous executive pattern, with relatively greater challenges in metacognitive processes such as working memory and cognitive flexibility.
Multivariate analyses indicated that LGS diagnosis and the presence of SCN1A mutations were significant predictors of executive dysfunction. Moreover, structural etiologies of epilepsy were associated with deficits in emotional and behavioral self-regulation.
Conclusions:
These results underscore the clinical relevance of BRIEF-2 as a sensitive tool for neurobehavioral profiling in DEE and highlight its potential to guide individualized neuropsychological interventions.
Funding: Camelice Scholarship