LANGUAGE AND BEHAVIOR IN CHILDREN WITH SOTOS SYNDROME

To examine language and behavior in children with Sotos syndrome, an o vergrowth syndrome involving advanced bone age, characteristic facies, and developmental disability. Method: Twenty-seven children with Soto s syndrome were compared with 20 children with overgrowth, intellectua l disability, and facies not characteristic of Sotos syndrome. Ages ra nged from 5 to 16 years. Direct assessment was undertaken with standar dized measures of intelligence and language abilities. Behavior was ex amined by parent and teacher report. Results: Children with Sotos synd rome had levels of intelligence in the severely disabled to average ra nge, with the majority falling in the borderline range. Mean level of intelligence was significantly higher than that observed for children in the comparison group. Language abilities were developed to a level consistent with overall level of intelligence. Rates of parent- and te acher-reported behavior problems were significantly higher than normal , but, with the exception of temper tantrums, did not differ from thos e observed in children in the comparison group. Attention-deficit hype ractivity disorder was observed in 38% of children with Sotos syndrome . They were more irritable and had more stereotypic behavior and inapp ropriate speech than is expected in children with intellectual disabil ities, and they were more withdrawn and had more stereotypic behavior than children in the comparison group. Conclusions: Assessment of lang uage abilities revealed no specific language impairment. High rates of behavior problems were observed, but these were not higher than those observed for other large, delayed, dysmorphic children.