Syndromic craniosynostosis: neuropsycholinguistic abilities and imaging analysis of the central nervous system



Document title: Syndromic craniosynostosis: neuropsycholinguistic abilities and imaging analysis of the central nervous system
Journal: Arquivos de neuro-psiquiatria
Database: PERIÓDICA
System number: 000414352
ISSN: 0004-282X
Authors: 1
2
1
2
1
1
Institutions: 1Universidade de Sao Paulo, Faculdade de Odontologia de Bauru, Bauru, Sao Paulo. Brasil
2Universidade Estadual Paulista "Julio de Mesquita Filho", Faculdade de Medicina de Botucatu, Botucatu, Sao Paulo. Brasil
Year:
Season: Dic
Volumen: 75
Number: 12
Pages: 862-868
Country: Brasil
Language: Inglés
Document type: Estadística o encuesta
Approach: Analítico
English abstract Objective: To characterize patients with syndromic craniosynostosis with respect to their neuropsycholinguistic abilities and to present these findings together with the brain abnormalities. Methods: Eighteen patients with a diagnosis of syndromic craniosynostosis were studied. Eight patients had Apert syndrome and 10 had Crouzon syndrome. They were submitted to phonological evaluation, neuropsychological evaluation and magnetic resonance imaging of the brain. The phonological evaluation was done by behavioral observation of the language, the Peabody test, Token test and a school achievement test. The neuropsychological evaluation included the WISC III and WAIS tests. Results: Abnormalities in language abilities were observed and the school achievement test showed abnormalities in 66.67% of the patients. A normal intelligence quotient was observed in 39.3% of the patients, and congenital abnormalities of the central nervous system were observed in 46.4% of the patients. Conclusion: Abnormalities of language abilities were observed in the majority of patients with syndromic craniosynostosis, and low cognitive performance was also observed
Portuguese abstract Objetivo: Caracterizar as habilidades neuropsicolinguísticas de indivíduos com craniossinostoses sindrômicas e apresentar esses achados com as anomalias do sistema nervoso central. Métodos: Participaram do estudo 18 sujeitos com diagnóstico clínico de craniossinostose sindrômica, 44,4% com a síndrome de Apert e 55,6% síndrome de Crouzon. Todos os sujeitos foram submetidos a avaliação fonoaudiológica, psicológica e exames de ressonância magnética do encéfalo. A avaliação fonoaudiológica foi contemplada pela Observação Comportamental da Linguagem, Teste Peabody (TVIP), Teste Token e Teste de Desempenho Escolar (TDE); enquanto a psicológica utilizou a WISC-III e a WAIS. Resultados: Observou-se alteração nas habilidades de linguagem em todos os protocolos utilizados, sendo o TDE o que apresentou maior porcentagem de alteração (66,67%).A avaliação cognitiva evidenciou quociente de inteligência dentro da média em 39,3% dos sujeitos, enquanto que 46,4% apresentaram malformações congênitas do sistema nervoso central. Conclusão: Constatou-se alterações nas habilidades de linguagem na maioria dos sujeitos com craniossinostoses sindrômicas, bem como o baixo desempenho cognitivo
Disciplines: Medicina
Keyword: Neurología,
Psicología clínica,
Malformaciones congénitas,
Craneosinostosis,
Acrocefalosindactilia,
Disostosis craneofacial,
Sistema nervioso central,
Neuropsicología,
Lenguaje,
Cognición
Keyword: Medicine,
Neurology,
Clinical psychology,
Congenital malformations,
Craniosynostosis,
Acrocephalosyndactylia,
Craniofacial disostosis,
Central nervous system,
Neuropsychology,
Language disorders,
Cognition
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