The profile of social communication in Dravet syndrome

Abstract

Dravet syndrome (DS) presents a multifaceted clinical picture marked by epilepsy, cognitive impairments and behavioral disorders that progresses throughout development. Behavioral disorders include impairments in social relationships and communication, with frequent diagnosis of autism spectrum disorder. This study focused on comprehensively evaluating and comparing social communication profiles among a group of 43 children with Dravet syndrome, 30 children with level 1 autism spectrum disorder, 36 with social (pragmatic) communication disorder, and 18 with intellectual disability. Using validated tools like the Childhood Autism Spectrum Test and Children’s Communication Checklist, distinct patterns of social communication deficits were delineated. Our findings indicate that children with Dravet syndrome experience challenges in social relationships, primarily due to difficulties in use of pragmatic language. Areas such as range of interests and social interaction are less affected compared to those with ASD, emphasizing differing profiles between the conditions. While children with DS and ID may have similar intellectual functioning, the different social communication deficits in DS indicate their role in the DS phenotype beyond ID. These results underscore the unique social communication profile of DS and emphasizes the importance of tailored interventions and deep phenotyping efforts for effective DS management.