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Cognitive functioning in children with typical cri du chat (5p-) syndrome.
K M Cornish1, D Bramble, F Munir
1Neuropsychology of Genetic Disorders Research Unit, Medical School, University of Nottingham, Queens Medical Centre, UK.
Developmental Medicine and Child Neurology
|June 4, 1999
Summary
Children with cri du chat syndrome (CDCS) show better receptive than expressive language skills. This suggests they can understand more complex commands than previously thought, despite learning disabilities.
Area of Science:
- Neuropsychology
- Developmental Pediatrics
- Genetics
Background:
- Cri du chat syndrome (CDCS) is a genetic disorder associated with intellectual disability.
- Cognitive functioning in CDCS has not been systematically assessed using neuropsychological measures.
- Understanding language capabilities is crucial for educational and therapeutic interventions.
Purpose of the Study:
- To systematically assess cognitive functioning in children with typical CDCS.
- To evaluate IQ level, receptive and expressive language skills, and articulation.
- To identify specific cognitive profiles, particularly in language functioning.
Main Methods:
- Neuropsychological testing battery administered to 26 children with CDCS (ages 6-15).
- Assessment included measures of IQ, receptive and expressive language, and articulation.
- Analysis focused on identifying patterns and discrepancies in cognitive performance.
Main Results:
- Most children (24/26) fell within the severe learning disability range.
- A significant discrepancy was observed between receptive and expressive language skills.
- Receptive language skills were found to be better than expressive language skills.
Conclusions:
- Children with CDCS demonstrate a distinct language profile with stronger comprehension than production.
- This highlights potential for understanding complex verbal commands despite expressive language limitations.
- Parents and professionals should adjust expectations regarding communication capacities in CDCS.
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