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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(12): 862-868
DOI: 10.1590/0004-282X20170171
Article

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

Craniossinostoses sindrçmicas: habilidades neuropsicolinguísticas e análise por imagem do sistema nervoso central
Luciana Paula Maximino
1   Universidade de São Paulo, Faculdade de Odontologia de Bauru, Departamento de Fonoaudiologia e Audiologia, Bauru SP, Brasil
2   Universidade de São Paulo, Hospital para Reabilitação para Anomalias Craniofaciais, Bauru SP, Brasil
,
Luis Gustavo Ducati
4   Universidade Estadual Paulista, Faculdade de Medicina de Botucatu, Departamento de Neurologia, Psicologia e Psiquiatria, Botucatu SP, Brasil
,
Dagma Venturini Marques Abramides
1   Universidade de São Paulo, Faculdade de Odontologia de Bauru, Departamento de Fonoaudiologia e Audiologia, Bauru SP, Brasil
,
Camila de Castro Corrêa
3   Universidade Estadual Paulista, Faculdade de Medicina de Botucatu, Departamento de Oftalmotogia, Otorrinilaringologia e Cirurgia de Cabeça e Pescoço, Botucatu SP, Brasil
,
Patrícia Fernandes Garcia
1   Universidade de São Paulo, Faculdade de Odontologia de Bauru, Departamento de Fonoaudiologia e Audiologia, Bauru SP, Brasil
,
Adriano Yacubian Fernandes
1   Universidade de São Paulo, Faculdade de Odontologia de Bauru, Departamento de Fonoaudiologia e Audiologia, Bauru SP, Brasil
4   Universidade Estadual Paulista, Faculdade de Medicina de Botucatu, Departamento de Neurologia, Psicologia e Psiquiatria, Botucatu SP, Brasil
› Author AffiliationsFAPESP2000/080803 and CNPQ 307043/2008-8
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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.

RESUMO

Objetivo: Caracterizar as habilidades neuropsicolinguísticas de indivíduos com craniossinostoses sindrçmicas e apresentar esses achados com as anomalias do sistema nervoso central.

Métodos: Participaram do estudo 18 sujeitos com diagnóstico clínico de craniossinostose sindrçmica, 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 sindrçmicas, bem como o baixo desempenho cognitivo.

Keywords:

acrocephalosyndactylia - craniofacial dysostosis - central nervous system - neuropsychology - language

Palavras-chave:

acrocefalossindactilia - disostose craniofacial - sistema nervoso central - neuropsicologia - linguagem

Support:

FAPESP2000/080803 and CNPQ 307043/2008-8.




Publication History

Received: 05 August 2016

Accepted: 13 September 2017

Article published online:
01 September 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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