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

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

Amy Brown  1 , Marta Arpone  2 , Amy L Schneider  3 , Silvana Micallef  4 , Vicki A Anderson  5 , Ingrid E Scheffer  6
Affiliations

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

Amy Brown et al. Epilepsy Behav. 2020 Nov.

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.

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Conflict of interest statement

Declaration of competing interest Ingrid Scheffer serves/has served on the editorial boards of the Annals of Neurology, Neurology, Epileptic Disorders and Epilepsy Currents; may accrue future revenue on pending patent WO2009/086591: Diagnostic And Therapeutic Methods For EFMR (Epilepsy And Mental Retardation Limited To Females); has a patent for SCN1A testing held by Bionomics Inc and licensed to various diagnostic companies (WO/2006/133508); she has a patent for a molecular diagnostic/therapeutic target for benign familial infantile epilepsy (BFIE) [PRRT2] WO/2013/059884 with royalties paid. She has served on scientific advisory boards for UCB, Eisai, GlaxoSmithKline, BioMarin, Nutricia, Rogcon and Xenon Pharmaceuticals; has received speaker honoraria from GlaxoSmithKline, UCB, BioMarin, Biocodex and Eisai; has received funding for travel from UCB, Biocodex, GlaxoSmithKline, Biomarin and Eisai; has served as an investigator for Zogenix, Zynerba, Ultragenyx, GW Pharma, UCB, Eisai, Anavex Life Sciences and Marinus; and has consulted for Zynerba Pharmaceuticals, Atheneum Partners, Ovid Therapeutics and UCB. She receives/has received research support from the National Health and Medical Research Council of Australia, Health Research Council of New Zealand, CURE, Australian Epilepsy Research Fund, March of Dimes and NIH/NINDS.

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