title: [Rett's syndrome: diagnostic issues].
abstract: Rett's syndrome (RS) is a severe disease of an early childhood, affecting girls mainly and manifesting in autistic symptoms, severe mental regression and motor dysfunction. The aim of the study was to analyze both early stages of clinical symptomatology and EEG correlates of RS. 65 patients aged 2.5-13 years with classic RS were examined. It was found that affective and autistic 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: RS 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 correlations between location of the focus of the rhythmic theta-activity and clinical manifestations as well as their prognostic value. Thus, the decrease of the index of the rhythmic theta-activity in the patients on the III-d stage of RS correlated significantly with the improvement of the attention, 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.
authors.forename: V V
authors.initials: VV
authors.lastname: Grachev
mesh_terms: D000293:Adolescent; D002540:Cerebral Cortex / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008297:Male; D011596:Psychomotor Disorders / Q000175:diagnosis; D015518:Rett Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D012720:Severity of Illness Index; D012737:Sex Factors
vernacular_title: Sindrom Retta: voprosy diagnostiki.