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

被引:25
作者
Brown, Amy [1 ,2 ]
Arpone, Marta [1 ]
Schneider, Amy L. [3 ]
Micallef, Silvana [4 ]
Anderson, Vicki A. [1 ,6 ,7 ,8 ]
Scheffer, Ingrid E. [1 ,3 ,5 ,8 ]
机构
[1] Royal Childrens Hosp, Murdoch Childrens Res Inst, 50 Flemington Rd, Melbourne, Vic 3052, Australia
[2] Swinburne Univ, Melbourne, Vic, Australia
[3] Univ Melbourne, Epilepsy Res Ctr, Dept Med, Austin Hlth, Heidelberg, Vic, Australia
[4] Austin Hlth, Heidelberg, Vic, Australia
[5] Florey Inst Neurosci & Mental Hlth, Heidelberg, Vic, Australia
[6] Univ Melbourne, Melbourne Sch Psychol Sci, Melbourne, Vic, Australia
[7] Royal Childrens Hosp, Psychol Serv, Melbourne, Vic, Australia
[8] Univ Melbourne, Dept Paediat, Parkville, Vic, Australia
关键词
Dravet syndrome; Behavioral functioning; Cognitive assessment; Social functioning; Neuropsychology; DORSAL; GENE; COMORBIDITIES; EPILEPSY; AUTISM; IMPACT;
D O I
10.1016/j.yebeh.2020.107319
中图分类号
B84 [心理学]; C [社会科学总论]; Q98 [人类学];
学科分类号
03 ; 0303 ; 030303 ; 04 ; 0402 ;
摘要
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. (C) 2020 Elsevier Inc. All rights reserved.
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页数:7
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