Abstract
BACKGROUND
The neurocognitive effects of NF1 include social cognition deficits, which are significant, because cause cognitive and social functioning. Social cognition is defined as cognitive processes related to the perception and understanding of cues in the environment that communicate social and interpersonal information and the ability to use those cues to interpret the thoughts and reactions of others and to modify one’s own behaviors to fit the social context. The purpose of the study was to describe cognitive deficits and behavioral phenotype of NF1, as well as looking for connections between deficits of social cognition and socio-emotional status in NF1.
METHODS
Psychological repeated testing and long-term observation was performed in 100 patients with NF1. Age at psychological diagnosis: 3 - 18 years. Full psychological outcome assessed cognitive, verbal and affective problems associated with manifestations of NF1. The patients were examined using battery of neuropsychological methods, psychological interview and analysis of medical history. For research of social functioning used subscales of Vineland Adaptive Behavior Scales (VABS-II) and Child Behavior Checklist (CBCL). We assessed also maladaptive behavior as a risk of social rejection. The main point of the assessment was to discover link between social cognition and challenging behaviors.
RESULTS
In the sample of patients with NF1 we observed specific social difficulties associated with emotional disturbances and social rejection. This specific behavioral phenotype and social cognition deficits disturbed learning process and their social life. Patients with NF1 were distinctly ineffective in interpersonal communications and establishing relations in the peer group as well as following the rules of social convention.
CONCLUSIONS
The results of our study suggest that social cognition deficits, are responsible for socio-emotional status of patients with NF1. Draft recommendations for diagnosis of specific emotional problems typical for NF1 and program of social development stimulation in NF1 will be presented and discussed.