Wenk G L
Division of Neural Systems, Memory and Aging, Arizona Research Laboratories, University of Arizona, Tucson 85724, USA.
Neuropediatrics. 1995 Apr;26(2):123-5. doi: 10.1055/s-2007-979741.
Rett syndrome is a neurological disorder associated with cortical atrophy, stereotyped hand movements, dementia, and extrapyramidal dysfunction. Endogenous levels of dopamine and its metabolites are decreased throughout the neocortex and basal ganglia and the number of dopamine type 2 receptors are decreased in the putamen. The present study investigated changes in dopamine uptake sites and dopamine type-1 receptors in the brains of eleven Rett syndrome patients (4-30 yrs) and ten normal female controls (2.5-20 yrs). The number of dopamine type 1 receptors within the caudate nucleus were unchanged. The density of dopamine reuptake sites were unchanged in the cingulate and midfrontal gyri but decreased within the caudate nucleus and putamen. The results of the present study suggest that: 1) in the basal ganglia of Rett syndrome patients, dopamine receptive neurons are intact whereas the number and activity of dopamine terminals are decreased, and 2) in the midfrontal and cingulate cortex, dopaminergic neuronal activity may be increased in order to compensate for fewer terminals that contain less dopamine.
瑞特综合征是一种与皮质萎缩、刻板手部动作、痴呆和锥体外系功能障碍相关的神经疾病。整个新皮质和基底神经节中多巴胺及其代谢产物的内源性水平降低，壳核中多巴胺2型受体数量减少。本研究调查了11名瑞特综合征患者（4 - 30岁）和10名正常女性对照（2.5 - 20岁）大脑中多巴胺摄取位点和多巴胺1型受体的变化。尾状核内多巴胺1型受体数量未变。扣带回和额中回中多巴胺再摄取位点的密度未变，但尾状核和壳核内的密度降低。本研究结果表明：1）在瑞特综合征患者的基底神经节中，多巴胺感受神经元完好无损，而多巴胺终末的数量和活性降低；2）在额中回和扣带回皮质中，多巴胺能神经元活性可能增加，以补偿含有较少多巴胺的终末数量减少的情况。
