[The progression of a case in the cri-du-chat syndrome: otolaryngological, cognitive and language characteristics].
This article presents the results of the ORL, cognitive and speech examinations of a boy aged 12 years, 9 months with cat cry syndrome. The laryngeal function has normalized but a small mandibular deficiency persists. The cognitive function corresponds to a mental age of less than 3 years with a complete blockade for abstraction but a good adaptation for concrete and practical situations. The linguistic examination was performed in two complementary pragmatic contexts: standard testing and semi-induced play. The referential and expressive functions are relatively good but with a poor adaptation to the interlocutors. The cognitive-semantic function of J.M. in naming and for space relation markers corresponds to about 4 1/2 years, which is better than what could be expected based on his cognitive level on the intelligence scales. Nevertheless, the child's syntactic characteristics are those of a 3-year-old child. Therefore the disparity between the cognitive-semantic, the syntactic and the nonlinguistic cognitive functions is negligible. Only the pragmatic function of the child remains much weaker than that achieved for the other facets of his language. The linguistic results of this case of cat cry syndrome are compared with those of children of the same age and mental level with trisomy 21 or other moderate mental handicaps.
Piérart
B
B
Université de Louvain, Service d'ORL, Bruxelles, Belgique.
Remacle
M
M
D002648:Child; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D002895:Chromosomes, Human, Pair 5; D003072:Cognition Disorders / Q000175:diagnosis; D003410:Cri-du-Chat Syndrome / Q000235:genetics; D006801:Humans; D007361:Intelligence Tests; D007806:Language Disorders / Q000175:diagnosis; D008297:Male