Adaptive and maladaptive behavior in children with Smith-Magenis Syndrome.

HIV and AIDS Malignancy Branch, National Cancer Institute, Bethesda, MD 20892-8200, USA.
Journal of Autism and Developmental Disorders (Impact Factor: 3.34). 06/2006; 36(4):541-52. DOI: 10.1007/s10803-006-0093-2
Source: PubMed

ABSTRACT Children with Smith-Magenis Syndrome (SMS) exhibit deficits in adaptive behavior but systematic studies using objective measures are lacking. This descriptive study assessed adaptive functioning in 19 children with SMS using the Vineland Adaptive Behavior Scales (VABS). Maladaptive behavior was examined through parent questionnaires and the Childhood Autism Rating Scale. Cognitive functioning was evaluated with an age-appropriate test. Children scored below average on VABS Communication, Daily Living Skills, and Socialization scales. Learning problems and hyperactivity scales on the Conner's Parent Rating Scale were elevated, and girls were more impulsive than boys. Stereotypic and self-injurious behaviors were present in all children. Cognitive functioning was delayed and consistent with communication and daily living skills, while socialization scores were higher than IQ.

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    ABSTRACT: This natural observation study was designed to evaluate hypothesized elevated 'attention-seeking' and preference for adult attention in Smith-Magenis syndrome. Ten children with Smith-Magenis syndrome were observed across one school day, together with an age matched sample of 10 children with Down syndrome. Levels of attention given to, and vigilance for, adults and peers were recorded and compared. Sequences of behaviour were analyzed to evaluate the temporal relationships between giving and receiving attention during adult-child interactions. Compared to children with Down syndrome, children with Smith-Magenis syndrome gave preferential attention to adults and looked towards adults significantly more than they looked towards peers. Sequential analyses revealed that while children with Smith-Magenis syndrome did not initiate interactions with adults more than children with Down syndrome did, reciprocity between child and adult social behaviours in Smith-Magenis syndrome within interactions was compromised. This less synchronous sequence of child and adult interactions in Smith-Magenis syndrome may be the result of children with Smith-Magenis syndrome attempting to initiate interaction at times when it is unavailable. The marked preference for interacting with adults over peers in Smith-Magenis syndrome indicates atypicality of social interaction in this syndrome.
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