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Cri du Chat syndrome

Psychological and behavioural characteristics

The early reports on the syndrome suggested that profound learning disability was a cardinal feature of the syndrome, presenting in all individuals with a 5p deletion. However, recent findings indicate that in children with typical Cri du Chat, IQ predominantly falls into the moderate to severe learning disability range but that there is a crucial discrepancy in the pattern of language functioning with children displaying better receptive than expressive language (Cornish, Bramble et al 1999; Cornish and Munir 1998). These findings extend previous research that discovered 'language delay' to be a deviant feature of the syndrome by highlighting a particular strength within their cognitive profile. Even in children with very minimal speech, studies have shown that many can use basic sign or gestural language for communication (Carlin 1990).

Self-injurious behaviour appears to be very common in Cri du Chat syndrome (Dykens and Clarke 1997; Collins and Cornish 2001) most notably head banging, hitting the head against body parts, and self-biting all reaching a plateau in late childhood and then remaining constant throughout early adulthood. Clinical hyperactivity is also known to be over represented in children with the syndrome (Dykens and Clark 1997) and is further compounded by a high incidence of chronic sleep problems and restlessness.

View What are the symptoms? What are the symptoms?  |  Inheritance patterns and prenatal diagnosis View Inheritance patterns and prenatal diagnosis

Medical text written July 2001 by Dr K Cornish and Dr D Bramble, Consultant Child and Adolescent Psychiatrist, Nottingham University, Nottingham, UK. Last reviewed October 2005 by Professor K Cornish, Canada Research Chair in Neuropsychology and Education, McGill University, Montreal, Canada.

 

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