The Characteristics of Neurocognitive and Psychosocial function in Girls with Turner Syndrome

The Characteristics of Neurocognitive and Psychosocial function in Girls with Turner Syndrome

The Characteristics of Neurocognitive and Psychosocial function in Girls with Turner Syndrome

(구연):
Release Date : 2017. 10. 26(목)
Bonglim Joo1,2, Ahreum Kwon2,3, Junghwan Suh2,3, Hyun Wook Chae2,4, Duk Hee Kim5 , Ho-Seong Kim2,3
Yonsei Univeresity Severance Children's Hospital Pediatric Psychology1
Yonsei Univeresity College of Medicine Pediatrics2
Yonsei Univeresity Severance Children's Hospital Pediatric Endocrinology3
Yonsei Univeresity Gangnam Severance Hospital Pediatric Endocrinology4
Sowha Children's Hospital Pediatric Endocrinology5
주봉림1,2, 권아름2,3, 서정환2,3, 채현욱2,4, 김덕희5 , 김호성2,3
연세의대 세브란스 어린이병원 소아심리1
연세대학교 의과대학 소아청소년과2
연세의대 세브란스 어린이병원 소아청소년과 소아내분비3
연세의대 강남세브란스병원 소아청소년과 소아내분비4
소화아동병원 소아청소년과 소아내분비5

Abstract

Introduction: Turner syndrome (TS) is a disorder, associated with loss of part or all of one X chromosome in females. In previous literature review, TS has been identified as having an increased risk for nonverbal learning disorder (NLD). The intellectual function and verbal abilities are usually intact, while relative weakness in visuospatial, executive function and social domain are observed in TS patients. Because there are a few studies in neuropsychological profiles in TS patients, the aim of this study was to identify neurocognitive profile and psychosocial function in Korean girls with TS and understand their characteristics. Methods: Participants were recruited and seventeen girls with TS (aged 5-14years) were performed neuropsychological evaluation as follows. (a) K-WISC-IV for intelligence, (b) VMI and Rey copy for visuospatial, (c) CAT for attention, (d) Rey-Kim memory test for memory, (e) CCTT for executive function, (f) Child Behavior Check list (CBCL), Pediatric Quality of Life ( PedsQL), Children’s Depression Index (CDI), Revised Children Manifest Anxiety Scale (RCMAS) and Self-Concept Inventory (SCI) for psychosocial. Results: The intellectual impairments were observed in six (35.3%) of the participants, even though mean full scales intelligence quotient (FSIQ) showed average. The score of verbal domain was the highest in the intelligence quotient, and it was significantly higher than performance domain, which is related perceptual reasoning. The social, attention, working memory scores in intelligence quotient subtest were significantly lower than population mean. The mean Memory quotient (MQ) was average, while the scores of seven subjects (41.2%) were lower than norm in visuospatial function. In addition, Executive/Attention function showed often deficient in TS patients. Although most of participants reported the good total self-concept, mood, behavior and quality of life, it showed partially low social competence, math/physical self-esteem. Conclusions: The study results revealed that TS had cognitive weakness for NLD and risk for social cognitive difficulties. This study offers clinical neurocognitive data in Korean girls with TS and it may contribute to understand their characteristics. In addition, the present results will support to development of psychological programs that ultimately improve the quality of life of TS.

Keywords: Turner syndrome, cognition, psychology