Apert syndrome: Factors involved in the cognitive development

• Autor(a) principal: Yacubian-Fernandes, Adriano [UNESP]
• Data de Publicação: 2005
• Outros Autores: Palhares Neto, Aristides Augusto [UNESP], Giglio, Alcir, Gabarra, Roberto Colichio [UNESP], Zanini, Silvio, Portela, Luis, Silva, Mateus Violin, Perosa, Gimol Benzaquen [UNESP], Abramides, Dagma, Plese, José Píndaro P.
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UNESP
• Texto Completo: http://dx.doi.org/10.1590/S0004-282X2005000600011; http://hdl.handle.net/11449/68562
• topic: Apert syndrome; Magnetic resonance; Mental development; acrocephalosyndactyly; adolescent; adult; brain malformation; child; classification; clinical article; cognitive development; controlled study; correlation analysis; education; family; family attitude; female; human; intelligence quotient; male; mental development; neuropsychological test; parent; parental attitude; qualitative analysis; social aspect; Acrocephalosyndactylia; Adolescent; Adult; Child; Child, Preschool; Cognition Disorders; Educational Status; Female; Humans; Infant; Intelligence; Magnetic Resonance Imaging; Male; Neuropsychological Tests; Pregnancy; Socioeconomic Factors

Apert syndrome is characterized by craniosynostosis, symmetric syndactyly and other systemic malformations, with mental retardation usually present. The objective of this study was to correlate brain malformations and timing for surgery with neuropsychological evaluation. We also tried to determine other relevant aspects involved in cognitive development of these patients such as social classification of families and parents' education. Eighteen patients with Apert syndrome were studied, whose ages were between 14 and 322 months. Brain abnormalities were observed in 55.6% of them. The intelligence quotient or developmental quotient values observed were between 45 and 108. Mental development was related to the quality of family environment and parents' education. Mental development was not correlated to brain malformation or age at time of operation. In conclusion, quality of family environment was the most significant factor directly involved in mental development of patients with Apert syndrome.