Blue M E, Naidu S, Johnston M V
Kennedy Krieger Institute, and Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Ann Neurol. 1999 Apr;45(4):541-5. doi: 10.1002/1531-8249(199904)45:4<541::aid-ana21>3.0.co;2-2.
To determine whether a disorder of excitatory neurotransmission plays a role in the pathophysiology of Rett syndrome (RS), N-methyl-D-aspartate (NMDA), adenosine monophosphate acid (AMPA), kainate, and metabotropic types of glutamate receptors were labeled autoradiographically in the superior frontal gyrus (SFG) from 9 RS patients and 10 female controls. The results showed a trend for the densities of NMDA, AMPA, gamma-aminobutyric acid, and metabotropic glutamate receptors to be higher in younger patients than in controls and for densities in older patients to fall below those of controls. The age-related changes in SFG NMDA receptor density may be correlated with the shift from psychomotor regression and seizures in younger stage II/III RS girls to the less epileptic plateau stage in older girls.
为了确定兴奋性神经传递障碍是否在雷特综合征（RS）的病理生理学中起作用，对9例RS患者和10例女性对照者的额上回（SFG）进行了放射自显影标记，以检测N-甲基-D-天冬氨酸（NMDA）、单磷酸腺苷（AMPA）、海人藻酸以及代谢型谷氨酸受体。结果显示，较年轻患者的NMDA、AMPA、γ-氨基丁酸和代谢型谷氨酸受体密度有高于对照者的趋势，而年龄较大患者的受体密度则低于对照者。SFG中NMDA受体密度的年龄相关变化可能与II/III期较年轻的RS女孩从精神运动性退化和癫痫发作向年龄较大女孩癫痫发作较少的平台期转变有关。
