title: Executive functions in intellectual disabilities: a comparison between Williams syndrome and Down syndrome.
abstract: Executive functions are a set of high cognitive abilities that control and regulate other functions and behaviors and are crucial for successful adaptation. Deficits in executive functions are frequently described in developmental disorders, which are characterized by disadaptive behavior. However, executive functions are not widely examined in individuals with intellectual disability. The present study is aimed at evaluating the etiological specificity hypotheses pertaining to executive functions by comparing individuals with intellectual disability of different etiology, as Williams syndrome and Down syndrome, on different aspects of executive functions. To this aim a battery evaluating attention, short-term and working memory, planning, categorization, shifting and inhibition, was administered to 15 children, adolescents and adults with Williams syndrome, to 15 children, adolescents and adults with Down syndrome and to 16 mental-age-matched typically developing children. The two groups with intellectual disability showed impairment in a set of executive functions, as auditory sustained attention, visual selective attention, visual categorization and working memory, and preserved visual sustained attention, auditory selective attention and visual inhibition. However, a distinctive profile has been found between the two syndromic groups on other executive functions. While participants with Down syndrome were poor in shifting and verbal aspects of memory and inhibition, those with Williams syndrome were poor in planning. The specific weakness and straights on executive functions may support the etiological specificity hypothesis accounting for distinctive cognitive development syndrome-specific.
authors.affiliation: Department of Neuroscience, Bambino Gesù Children's Hospital, Piazza Sant'Onofrio 4, I-00165 Rome, Italy.
authors.forename: Floriana
authors.initials: F
authors.lastname: Costanzo
authors.forename: Cristiana
authors.initials: C
authors.lastname: Varuzza
authors.forename: Deny
authors.initials: D
authors.lastname: Menghini
authors.forename: Francesca
authors.initials: F
authors.lastname: Addona
authors.forename: Tiziana
authors.initials: T
authors.lastname: Gianesini
authors.forename: Stefano
authors.initials: S
authors.lastname: Vicari
mesh_terms: D000293:Adolescent; D000328:Adult; D001288:Attention / Q000502:physiology; D002648:Child; D003071:Cognition / Q000502:physiology; D004314:Down Syndrome / Q000503:physiopathology*; D056344:Executive Function / Q000502:physiology*; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000503:physiopathology*; D007360:Intelligence / Q000502:physiology; D008297:Male; D008570:Memory, Short-Term / Q000502:physiology; D009483:Neuropsychological Tests; D018980:Williams Syndrome / Q000503:physiopathology*; D055815:Young Adult