Behavioral phenotypes of genetic syndromes with intellectual disability: comparison of adaptive profiles.

Abstract:

:The study of distinctive and consistent behaviors in the most common genetic syndromes with intellectual disability is useful to explain abnormalities or associated psychiatric disorders. The behavioral phenotypes revealed outcomes totally or partially specific for each syndrome. The aim of our study was to compare similarities and differences in the adaptive profiles of the five most frequent genetic syndromes, i.e. Down syndrome, Williams syndrome, Angelman syndrome, Prader-Willi syndrome, and Fragile-X syndrome (fully mutated), taking into account the relation with chronological age and the overall IQ level. The research was carried out using the Vineland Adaptive Behavior Scale (beside the Wechsler Intelligence scales to obtain IQ) with a sample of 181 persons (107 males and 74 females) showing genetic syndromes and mental retardation. Syndrome-based groups were matched for chronological age and mental age (excluding the Angelman group, presenting with severe mental retardation). Similarities and differences in the adaptive profiles are described, relating them to IQs and maladaptive behaviors. The results might be useful in obtaining a global index of adjustment for the assessment of intellectual disability level as well as for educational guidance and rehabilitative plans.

journal_name

Psychiatry Res

journal_title

Psychiatry research

authors

Di Nuovo S,Buono S

doi

10.1016/j.psychres.2011.03.015

subject

Has Abstract

pub_date

2011-10-30 00:00:00

pages

440-5

issue

3

eissn

0165-1781

issn

1872-7123

pii

S0165-1781(11)00238-1

journal_volume

189

pub_type

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