Social and emotional problems in children with neurofibromatosis type 1: evidence and proposed interventions.

OBJECTIVE

To describe social and emotional problems in children and adolescents with neurofibromatosis type 1 (NF1) and propose interventions. Our hypothesis is that children with NF1 will have significantly more social and emotional problems, compared with their unaffected siblings and children in the general population.

STUDY

DESIGN

Forty-three children with NF1 and 22 unaffected siblings (ages 5 to 18 years) were assessed with a standardized test completed by parents and teachers (the Child Behavior Checklist).

RESULTS

As with other aspects of NF1, there was variable expressivity. However, when rated by parents, children with NF1 had significantly more problems in comparison with test norms or unaffected siblings on 7 of 8 scales: Social Problems, Attention Problems, Anxiety/Depression, Withdrawal, Thought Problems, Somatic Complaints, and Aggressive Behavior. Children with NF1 also scored lower than unaffected siblings on measures assessing sports and other activities. Teachers reported fewer differences.

CONCLUSIONS

We propose interventions in the form of information for parents; early screening and treatment for speech, motor, and cognitive problems; and an increased level of intervention to prevent and treat psychologic problems, including systematic screening with standardized tests.