[Disorders of higher mental functions in children with the Lesch-Nyhan syndrome].

The authors analyzed higher psychic dysfunctions, the structure of intellectual impairments, the characteristics of the emotional-motivational sphere in four children with the classical and in 12 children with the psychopathological variant of hereditary purinosis. Disturbances of higher psychic functions are associated with damage to the profound portions and the limbic system of the brain. The described structure of higher mental functions along with the characteristic features of the emotional-motivational sphere may serve as sufficient clinical-psychopathological criteria for diagnosing Lesch-Nyhan's syndrome.