Lo S T, Collin P J L, Hokken-Koelega A C S
Department of Pediatric Endocrinology, Dutch Growth Research Foundation, Rotterdam, The Netherlands.
Department of Child Psychiatry, de Koraalgroep, Sittard, The Netherlands.
J Intellect Disabil Res. 2015 Sep;59(9):827-34. doi: 10.1111/jir.12197. Epub 2015 Apr 14.
Prader-Willi syndrome (PWS) is characterised by hypotonia, hypogonadism, short stature, obesity, behavioural problems, intellectual disability, and delay in language, social and motor development. There is very limited knowledge about visual-motor integration in children with PWS.
Seventy-three children with PWS aged 7-17 years were included. Visual-motor integration was assessed using the Beery Visual-motor Integration test at the start of the study and after 2 years. The association between visual-motor integration and age, gender, genetic subtype and intelligence was assessed.
Children with PWS scored 'very low' (-3 standard deviations) in visual-motor integration and 'below average' (-1 standard deviation) in visual perception and motor coordination compared with typically developing children. Visual-motor integration was higher in children with a deletion (β = -0.170, P = 0.037), in older children (β = 0.222, P = 0.009) and in those with a higher total IQ (β = 0.784, P < 0.001). Visual perception was higher with a deletion (β = -0.193, P = 0.044) and higher IQ (β = -0.618, P < 0.001), but motor coordination was only higher with a higher total IQ (β = 0.429, P = 0.001). Visual perception and motor coordination were not associated with age or gender. There was a trend for visual-motor integration decline over the 2 year follow-up period (P = 0.099). Visual perception and motor coordination did not change over the follow-up period.
Visual-motor integration is very poor in children with PWS. Children scored higher on the time-limited subtests for visual perception and motor coordination than the combined test for visual-motor integration. Separation of visual-motor integration tasks into pure visual or motor tasks and allowing sufficient time to perform the tasks might improve daily activities, both at home and at school.
普拉德-威利综合征(PWS)的特征为肌张力减退、性腺功能减退、身材矮小、肥胖、行为问题、智力残疾以及语言、社交和运动发育迟缓。关于PWS患儿的视动整合,人们了解的知识非常有限。
纳入73名年龄在7至17岁的PWS患儿。在研究开始时和2年后使用贝利视动整合测试对视动整合进行评估。评估视动整合与年龄、性别、基因亚型和智力之间的关联。
与正常发育儿童相比,PWS患儿在视动整合方面得分“极低”(-3个标准差),在视觉感知和运动协调方面得分“低于平均水平”(-1个标准差)。基因缺失患儿(β=-0.170,P=0.037)、年龄较大的患儿(β=0.222,P=0.009)以及总智商较高的患儿(β=0.784,P<0.001)的视动整合水平更高。基因缺失患儿(β=-0.193,P=0.044)和智商较高的患儿(β=-0.618,P<0.001)视觉感知水平更高,但只有总智商较高的患儿运动协调能力更高(β=0.429, P=0.001)。视觉感知和运动协调与年龄或性别无关。在2年随访期内,视动整合有下降趋势(P=0.099)。随访期间视觉感知和运动协调能力未发生变化。
PWS患儿视动整合能力非常差。患儿在视觉感知和运动协调的限时子测试中的得分高于视动整合综合测试。将视动整合任务分解为纯视觉或纯运动任务,并给予足够时间完成任务可能会改善患儿在家和在学校的日常活动。