Cognitive, behavioral, and social functioning in children and adults with Dravet syndrome

Amy Brown; Marta Arpone; Amy L Schneider; Silvana Micallef; Vicki A Anderson; Ingrid E Scheffer

doi:10.1016/j.yebeh.2020.107319

Cognitive, behavioral, and social functioning in children and adults with Dravet syndrome

Epilepsy Behav. 2020 Nov:112:107319. doi: 10.1016/j.yebeh.2020.107319. Epub 2020 Aug 25.

Authors

Amy Brown¹, Marta Arpone², Amy L Schneider³, Silvana Micallef⁴, Vicki A Anderson⁵, Ingrid E Scheffer⁶

Affiliations

¹ Murdoch Children's Research Institute, Royal Children's Hospital, 50 Flemington Road, Parkville, 3052, Melbourne, Australia; Swinburne University, Hawthorn, Melbourne, Australia.
² Murdoch Children's Research Institute, Royal Children's Hospital, 50 Flemington Road, Parkville, 3052, Melbourne, Australia.
³ Epilepsy Research Centre, Department of Medicine, Austin Health, The University of Melbourne, Heidelberg, Australia.
⁴ Austin Health, Heidelberg, Australia.
⁵ Murdoch Children's Research Institute, Royal Children's Hospital, 50 Flemington Road, Parkville, 3052, Melbourne, Australia; Melbourne School of Psychological Science, University of Melbourne, Melbourne, Australia; Psychological Service, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, The University of Melbourne, Parkville, Australia.
⁶ Murdoch Children's Research Institute, Royal Children's Hospital, 50 Flemington Road, Parkville, 3052, Melbourne, Australia; Epilepsy Research Centre, Department of Medicine, Austin Health, The University of Melbourne, Heidelberg, Australia; Florey Institute of Neuroscience and Mental Health, Heidelberg, Australia; Department of Paediatrics, The University of Melbourne, Parkville, Australia. Electronic address: scheffer@unimelb.edu.au.

PMID: 32858363
DOI: 10.1016/j.yebeh.2020.107319

Abstract

Aim: The objective of the study was to delineate the cognitive, behavioral, psychological, and social functioning of individuals with Dravet syndrome.

Method: Cognitive, behavioral, and social functioning were assessed in patients with Dravet syndrome by comprehensive, age-appropriate standardized neuropsychological testing. Primary caregivers completed standardized measures regarding participants' behavior, psychological status, adaptive functioning, and social skills, including their involvement with intervention services.

Results: The cohort comprised 45 patients, aged 2-30 years. Intellectual functioning ranged from average intellect to profound intellectual disability, with a decrease in cognitive and adaptive functioning with age. Only 6 children were able to complete the entire neuropsychological battery and showed a range of cognitive profiles. Five of 6 participants scored within the average range on Affect Recognition and 5/6 on Motor Free Visual Perception tests. Twenty-one (58%) participants had deficits in social skills and 18/27 (67%) in social communication, with 10 participants, who did not yet have a diagnosis of autism spectrum disorder (ASD), screening positive for social communication deficits. Behavioral problems were frequently reported, with attention problems in 24 (65%) and atypicality in 25 (70%). Despite this, parents reported that psychological services were the least utilized health interventions.

Conclusions: Cognitive functioning varies markedly in individuals with Dravet syndrome, with some patients demonstrating global impairment while others have a discordant neuropsychological profile. Behavioral, psychological, social problems, and ASD are common. Social deficits should be reviewed to identify those who warrant ASD assessment. Early identification of behavioral and psychological disorders and targeted use of psychological intervention are essential components of holistic care in Dravet syndrome.

Keywords: Behavioral functioning; Cognitive assessment; Dravet syndrome; Neuropsychology; Social functioning.

Publication types

Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adult
Autism Spectrum Disorder*
Child
Child, Preschool
Cognition
Epilepsies, Myoclonic*
Humans
Social Interaction
Social Skills
Young Adult