Grachev V V
Zh Nevrol Psikhiatr Im S S Korsakova. 2001;101(1):22-6.
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.
雷特综合征（RS）是一种幼儿期的严重疾病，主要影响女孩，表现为自闭症症状、严重智力衰退和运动功能障碍。本研究的目的是分析RS临床症状学的早期阶段以及脑电图相关性。对65例年龄在2.5至13岁的典型RS患者进行了检查。结果发现，在约50%的病例中，情感和自闭症表现是RS第一阶段的主要症状。根据运动功能障碍和行为紊乱的程度描述了两种典型RS变体：伴有运动障碍的RS；伴有精神分裂症样障碍的RS。对62例处于疾病Ⅲ - d期的患者的145份脑电图进行分析，发现节律性θ活动焦点的位置与临床表现及其预后价值之间存在一些相关性。因此，RSⅢ - d期患者节律性θ活动指数的降低与注意力、行为和社交活动的改善显著相关，而其升高与高级皮质功能的恶化以及运动障碍的进展相关。
