title: Apert syndrome: factors involved in the cognitive development.
abstract: 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.
authors.affiliation: Department of Craniofacial Surgery, Hospital de Reabilitação de Anomalias Craniofaciais, University of Sao Paulo, Bauru, SP, Brazil. adriano@yacubian.com.br
authors.forename: Adriano
authors.initials: A
authors.lastname: Yacubian-Fernandes
authors.forename: Aristides
authors.initials: A
authors.lastname: Palhares
authors.forename: Alcir
authors.initials: A
authors.lastname: Giglio
authors.forename: Roberto C
authors.initials: RC
authors.lastname: Gabarra
authors.forename: Silvio
authors.initials: S
authors.lastname: Zanini
authors.forename: Luis
authors.initials: L
authors.lastname: Portela
authors.forename: Mateus Violin
authors.initials: MV
authors.lastname: Silva
authors.forename: Gimol Bezaquen
authors.initials: GB
authors.lastname: Perosa
authors.forename: Dagma
authors.initials: D
authors.lastname: Abramides
authors.forename: José Píndaro P
authors.initials: JP
authors.lastname: Plese
mesh_terms: D000168:Acrocephalosyndactylia / Q000150:complications* / Q000601:surgery; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D004522:Educational Status; D005260:Female; D006801:Humans; D007223:Infant; D007360:Intelligence; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D011247:Pregnancy; D012959:Socioeconomic Factors