Developmental profiles and mentality in preschool children with Prader-Willi syndrome: a preliminary study
Chien-Min Chen1, Chia-Ling Chen, Jia-Woei Hou
1Department of Physical Medicine and Rehabilitation, Chang Gung Memorial Hospital at Chiayi, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Insights
Children with Prader-Willi syndrome (PWS) exhibit uneven global developmental and mental delays across various domains. Fine motor and receptive language skills were notably better than gross motor and expressive language, respectively.
Area of Science:
- Pediatric Neurology
- Developmental Psychology
- Genetics
Background:
- Prader-Willi syndrome (PWS) is frequently associated with global developmental delay and cognitive impairments.
- Understanding the specific developmental trajectories in children with PWS is crucial for targeted interventions.
Purpose of the Study:
- To investigate the detailed developmental profiles and mental assessments in preschool-aged children diagnosed with Prader-Willi syndrome.
- To compare the developmental functioning of children with PWS against a typically developing cohort.
Main Methods:
- A cohort of ten children with PWS (15 months–6 years) and eleven typically developing children were assessed.
- The Chinese Children Developmental Inventory (CCDI) was used to evaluate developmental quotients (DQ) across eight domains.
- Intelligence quotient (IQ) and developmental index (DI) were measured for comprehensive mental assessment.
Main Results:
- Children with PWS demonstrated significantly lower DQs across all eight CCDI domains compared to controls (p < 0.01).
- Within the PWS group, fine motor skills surpassed gross motor skills, and receptive language exceeded expressive language abilities.
- Verbal IQ was higher than performance IQ, and mental DI was superior to psychomotor DI in children with PWS.
Conclusions:
- Children with Prader-Willi syndrome present with a distinct pattern of uneven global developmental and mental delays.
- These findings provide valuable insights for clinicians to better understand and plan individualized treatment strategies for children with PWS.
Background:
A majority of the children with Prader-Willi syndrome (PWS) have global developmental delay and mental delay. The aim of this study was to investigate the developmental profiles and mental assessments among preschool children with PWS.
Methods:
Ten children with PWS between the ages of 15 months to 6 years, and 11 children with typical development were enrolled. Developmental profiles in terms of their developmental quotient (DQ) for the eight domains of the Chinese Children Developmental Inventory (CCDI) and mental assessments in terms of intelligence quotient (IQ) and developmental index (DI) were carried out for all children.
Results:
The DQs of all eight domains, including gross motor, fine motor, expressive language, concept comprehension, situation comprehension, self help, personal- social and general development, in the PWS group were lower than the DQs of the children from the typical development group (p < 0.01). Children with PWS had better DQs in the fine motor domain than in the gross motor domain and in the receptive language domain than in the expressive language domain. Furthermore, their verbal IQ were better than their performance IQ and their mental DI was better than their psychomotor DI.
Conclusions:
These findings suggest that the children with PWS show an uneven global developmental delay together with an uneven mental delay. The results of this study should allow clinicians to better understand the developmental functioning of children with PWS and this will help with the planning of treatment strategies.
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