title: Cognitive and behavioral heterogeneity in genetic syndromes.
abstract: OBJECTIVE
  this study aimed to investigate the cognitive and behavioral profiles, as well as the psychiatric symptoms and disorders in children with three different genetic syndromes with similar sociocultural and socioeconomic backgrounds.
  
  
  METHODS
  thirty-four children aged 6 to 16 years, with Williams-Beuren syndrome (n=10), Prader-Willi syndrome (n=11), and Fragile X syndrome (n=13) from the outpatient clinics of Child Psychiatry and Medical Genetics Department were cognitively assessed through the Wechsler Intelligence Scale for Children (WISC-III). Afterwards, a full-scale intelligence quotient (IQ), verbal IQ, performance IQ, standard subtest scores, as well as frequency of psychiatric symptoms and disorders were compared among the three syndromes.
  
  
  RESULTS
  significant differences were found among the syndromes concerning verbal IQ and verbal and performance subtests. Post-hoc analysis demonstrated that vocabulary and comprehension subtest scores were significantly higher in Williams-Beuren syndrome in comparison with Prader-Willi and Fragile X syndromes, and block design and object assembly scores were significantly higher in Prader-Willi syndrome compared with Williams-Beuren and Fragile X syndromes. Additionally, there were significant differences between the syndromes concerning behavioral features and psychiatric symptoms. The Prader-Willi syndrome group presented a higher frequency of hyperphagia and self-injurious behaviors. The Fragile X syndrome group showed a higher frequency of social interaction deficits; such difference nearly reached statistical significance.
  
  
  CONCLUSIONS
  the three genetic syndromes exhibited distinctive cognitive, behavioral, and psychiatric patterns.
authors.affiliation: Department of Medical Psychology and Psychiatry, Faculty of Medical Sciences, Universidade de Campinas, Campinas, SP, Brazil; Pediatric Research Center, Department of Pediatrics, Faculty of Medical Sciences, Universidade de Campinas, Campinas, SP, Brazil. Electronic address: luizflp@fcm.unicamp.br.
authors.forename: Luiz F L
authors.initials: LF
authors.lastname: Pegoraro
authors.affiliation: Department of Medical Genetics, Faculty of Medical Sciences, Universidade de Campinas, Campinas, SP, Brazil.
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Steiner
authors.affiliation: Department of Medical Psychology and Psychiatry, Faculty of Medical Sciences, Universidade de Campinas, Campinas, SP, Brazil.
authors.forename: Eloisa H R V
authors.initials: EH
authors.lastname: Celeri
authors.affiliation: Department of Medical Psychology and Psychiatry, Faculty of Medical Sciences, Universidade de Campinas, Campinas, SP, Brazil.
authors.forename: Claudio E M
authors.initials: CE
authors.lastname: Banzato
authors.affiliation: Department of Medical Psychology and Psychiatry, Faculty of Medical Sciences, Universidade de Campinas, Campinas, SP, Brazil; Pediatric Research Center, Department of Pediatrics, Faculty of Medical Sciences, Universidade de Campinas, Campinas, SP, Brazil.
authors.forename: Paulo
authors.initials: P
authors.lastname: Dalgalarrondo
mesh_terms: D000293:Adolescent; D002648:Child; D003071:Cognition; D003072:Cognition Disorders / Q000235:genetics / Q000523:psychology*; D003430:Cross-Sectional Studies; D004522:Educational Status; D005260:Female; D005600:Fragile X Syndrome / Q000175:diagnosis / Q000523:psychology*; D006801:Humans; D007182:Income; D008607:Intellectual Disability / Q000235:genetics / Q000523:psychology*; D008297:Male; D001523:Mental Disorders / Q000235:genetics / Q000523:psychology*; D011218:Prader-Willi Syndrome / Q000175:diagnosis / Q000523:psychology*; D014888:Wechsler Scales; D018980:Williams Syndrome / Q000175:diagnosis / Q000523:psychology*
keywords: Behavior; Cognition; Cognição; Comportamento; Fragile X syndrome; Prader-Willi syndrome; Síndrome de Prader-Willi; Síndrome de Williams-Beuren; Síndrome do X-Frágil; Williams-Beuren syndrome