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Cognitive functioning in children with typical cri du chat (5p−) syndrome

Published online by Cambridge University Press:  01 April 1999

K M Cornish
Affiliation:
Section of Developmental Psychiatry, Division of Psychiatry, Faculty of Medicine, Medical School, University of Nottingham, Queens Medical Centre, Nottingham, UK.
D Bramble
Affiliation:
Section of Developmental Psychiatry, Division of Psychiatry, Faculty of Medicine, Medical School, University of Nottingham, Queens Medical Centre, Nottingham, UK.
F Munir
Affiliation:
Neuropsychology of Genetic Disorders Research Unit, Faculty of Medicine, University of Nottingham, Queens Medical Centre, Nottingham NG7 2UH, UK.
J Pigram
Affiliation:
Neuropsychology of Genetic Disorders Research Unit, Faculty of Medicine, University of Nottingham, Queens Medical Centre, Nottingham NG7 2UH, UK.
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Abstract

This study is the first attempt to assess systematically the cognitive functioning in children diagnosed with typical cri du chat syndrome (CDCS) using neuropsychological test measures. Twenty-six children aged between 6 years 4 months and 15 years 5 months (mean 8 years 3 months) completed a battery of tasks measuring IQ level, receptive and expressive language skills, and articulation. Twenty-four children were in the severe learning-disability range with no specific verbal or performance profile. Using more finely tuned measures of cognition, however, a clear discrepancy in the pattern of language functioning was found with better receptive than expressive language skills. One implication of these findings is that parents and professionals should be more optimistic about the capacities of children with CDCS to understand more complex verbal commands than their expressive language skills would suggest.

Type
Original Articles
Copyright
© 1999 Mac Keith Press

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