Rett's syndrome: diagnostic aspects

Rett's syndrome (RS) is a severe disease of an early childhood, affecting g irls mainly and manifesting in autistic symptoms, severe mental regression and motor dysfunction. The aim of the study was to analyze both early stage s of clinical symptomatology and EEC correlates of RS. 65 patients aged 2,5 -13 years with classic RS were examined. It was found that affective and au tistic manifestations were the main symptoms during the 1-st stage of RS in approximately 50% of the cases. Two variants of classic RS were described according to the degree of motor dysfunction and behavioral disturbances: R S with motor disorders; RS with schizophrenoform disorders. Analysis of 145 EEG of 62 patients with the III-d PS stage revealed a presence of some cor relations between location of the focus of the rhythmic theta -activity and clinical manifestations as well as their prognostic value. Thus, the decre ase of the index of the rhythmic theta -activity in the patients an the III -d stage of RS correlated significantly with the improvement of the attenti on, behavioral and social activity,while its increase correlated with both the change for the worse of the higher cortex functions and advance of the motor disorders.