Badr G G, Witt-Engerström I, Hagberg B
Department of Neurophysiology, University of Goteborg, Sweden.
Brain Dev. 1987;9(5):517-22. doi: 10.1016/s0387-7604(87)80076-1.
Six females with Rett syndrome (RS)--all seriously motor disabled with clinical symptomatology indicating not only brain but also spinal cord impairment--were investigated using auditory and somatosensory evoked responses techniques. In all patients the responses representing the pathways through the upper spinal cord were delayed suggesting an impairment of the central conduction time. The findings mean an involvement of the sub-cortical structures, the brain stem and the cervical spinal cord in the disease process. From the natural clinical course and the present findings, it is concluded that RS is also characterized by spinal cord impairment appearing with increasing age and stage of diseases.
六名患有雷特综合征（RS）的女性——均严重运动功能残疾，临床症状表明不仅存在脑部损伤，还存在脊髓损伤——采用听觉和体感诱发电位技术进行了研究。在所有患者中，代表通过上脊髓通路的反应均延迟，提示中枢传导时间受损。这些发现意味着皮质下结构、脑干和颈脊髓参与了疾病过程。从自然临床病程和目前的研究结果可以得出结论，雷特综合征的特征还包括随着年龄增长和疾病阶段的发展而出现的脊髓损伤。
