Developmental Profiles and Mentality in Preschool Children with Prader-Willi Syndrome: A Preliminary Study

Background: A majority of the children with Prader-Willi syndrome (PWS) have globaldevelopmental delay and mental delay. The aim of this study was to investigatethe developmental profiles and mental assessments among preschoolchildren with PWS.Methods: Ten children with PWS between the ages of 15 months to 6 years, and 11children with typical development were enrolled. Developmental profiles interms of their developmental quotient (DQ) for the eight domains of theChinese Children Developmental Inventory (CCDI) and mental assessmentsin terms of intelligence quotient (IQ) and developmental index (DI) werecarried out for all children.Results: The DQs of all eight domains, including gross motor, fine motor, expressivelanguage, concept comprehension, situation comprehension, self help, personal-social and general development, in the PWS group were lower than theDQs of the children from the typical development group (p < 0.01). Childrenwith PWS had better DQs in the fine motor domain than in the gross motordomain and in the receptive language domain than in the expressive languagedomain. Furthermore, their verbal IQ were better than their performanceIQ and their mental DI was better than their psychomotor DI.Conclusions: These findings suggest that the children with PWS show an uneven globaldevelopmental delay together with an uneven mental delay. The results ofthis study should allow clinicians to better understand the developmentalfunctioning of children with PWS and this will help with the planning oftreatment strategies.