Language and behavior in children with Sotos syndrome.

OBJECTIVE: To examine language and behavior in children with Sotos syndrome, an overgrowth syndrome involving advanced bone age, characteristic facies, and developmental disability. METHOD: Twenty-seven children with Sotos syndrome were compared with 20 children with overgrowth, intellectual disability, and facies not characteristic of Sotos syndrome. Ages ranged from 5 to 16 years. Direct assessment was undertaken with standardized measures of intelligence and language abilities. Behavior was examined by parent and teacher report. RESULTS: Children with Sotos syndrome had levels of intelligence in the severely disabled to average range, 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 teacher-reported behavior problems were significantly higher than normal, but, with the exception of temper tantrums, did not differ from those observed in children in the comparison group. Attention-deficit hyperactivity disorder was observed in 38% of children with Sotos syndrome. They were more irritable and had more stereotypic behavior and inappropriate speech than is expected in children with intellectual disabilities, and they were more withdrawn and had more stereotypic behavior than children in the comparison group. CONCLUSIONS: Assessment of language 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.

Midtrimester amniocentesis: obstetric outcome and neonatal neurobehavioral status.

The possible effects of midtrimester genetic amniocentesis on neurobehavioral status were studied in newborn infants of women who had had the procedure (N = 100) and in newborn infants of women who had declined the test (N = 56). Brazelton's Neonatal Behavioral Assessment Scale was administered to newborn infants born at term and did not reveal consequences of amniocentesis on neonatal orientation, range of state, motor ability, autonomic regulation, regulation of state, response decrement, or reflexes. Information on obstetric complications also was obtained. The findings raised questions regarding the temporal relationship between amniocentesis and fetal loss and focused attention on preterm birth as a potential risk that warrants further investigation. This study provides the foundation for our prospective longitudinal follow-up in which the cohort will be reassessed later in infancy and in childhood.

Two subgroups of gender-problem children.

Gender-referred children who met the DSM-III diagnostic criteria for gender identity disorder of childhood (n = 21) and gender-referred children who did not meet these criteria (n = 15) were compared with regard to demographic, gender role, and behavioral disturbance information. As judged by parental questionnaires and behavioral tests, the cross-gender role behavior of gender-referred children who met the DSM-III criteria was generally more extreme than that of their non-DSM-III counterparts. These two diagnostically defined subgroups did not, however, differ in the degree of their behavioral and emotional disturbance. The DSM-III children were significantly younger than the non-DSM-III children, but the extent to which the disparity in age accounted for the gender role differences appeared equivocal. The implications of these findings for the diagnosis and assessment of gender-referred children are discussed.

Sex-typed play in gender-disturbed children: a comparison to sibling and psychiatric controls.

Gender-disturbed children (n = 14) were compared to their preadolescent siblings (n = 16) and psychiatric controls (n = 13) on a sex-typed free-play task previously shown to differentiate gender-disturbed boys from normal boys. On three separate trials totaling 20 minutes, the gender-disturbed children played for a significantly longer period of time with cross-sex toys and for a significantly shorter period of time with same-sex toys than did the two control groups. The gender-disturbed children also showed greater trial-to-trial consistency in their play preferences than the other two groups. The utility of this task in the assessment of childhood gender disturbance is discussed. In addition, the results are discussed in relation to a number of perspectives regarding both typical and atypical gender identity development in childhood.

Patterns of intellectual functioning and spatial ability in boys and gender identity disorder.

Preadolescent boys with Gender Identity Disorder (N = 13), their brothers (N = 8) and boys referred for psychiatric assessment (N = 10) were administered age-appropriate Wechsler intelligence scales. Four hypotheses regarding patterns of intellectual functioning and spatial ability were tested. A previous suggestion that the development of boyhood femininity is associated with higher than average IQ was not supported, since the IQs of the feminine boys and their brothers did not differ. A report of enhanced verbal ability as compared with perceptual organization also was not replicated in these feminine boys. On Kaufman's "Freedom from Distractibility" factor, the feminine boys and their brothers obtained scores as low as the psychiatric controls; that is, all three groups were equally distractible. It was suggested that anxiety contributed to poor performance on this factor. Unlike the control groups, the feminine boys obtained low scores on a test of spatial ability (Block Design) as compared to their scores on a verbal task (Vocabulary). The association between a feminine gender role and relatively poor spatial ability was discussed.

Gender identity problems of children and adolescents: the establishment of a special clinic.

Theoretical accounts of the origins of gender identity disturbance are reviewed and then followed by a description of the establishment of a child and adolescent gender identity clinic. Clinical impressions of 16 gender disturbed patients are presented and the position is taken that most patients manifested a confused, as opposed to fixed, core gender identity.