Chiron C, Bulteau C, Loc'h C, Raynaud C, Garreau B, Syrota A, Mazière B
INSERM U 29 and Neuropediatric Department, Hôpital Saint Vincent de Paul, Paris, France.
J Nucl Med. 1993 Oct;34(10):1717-21.
A dopamine deficiency has been implicated in Rett syndrome, a progressive encephalopathy in girls that involves movement, tonus and cognitive disorders. To test the hypothesis that striatal D2 receptors increase in number in early stages of the disease, we measured the binding potential of 123I-Iodolisuride, a specific D2 ligand, in eleven Rett children aged 4-15 yr (7.9 +/- 3.5 yr) (mean +/- s.d.) and eight control subjects aged 3.5-13 yr (8.1 +/- 3.8 yr) who exhibited other neurological disorders. Regional cerebral blood flow (rCBF) was also measured with SPECT using 133Xe. The binding potential for 123I-ILIS and D2 receptors was significantly higher in Rett (0.45) than in controls (0.23) (p < 0.01). An increase in 123I-ILIS binding due to increased rCBF in patients' striata was excluded. Our results are consistent with a higher density of D2 receptors in young patients suffering from Rett syndrome because of reduced dopaminergic neurotransmission.
多巴胺缺乏与雷特综合征有关，雷特综合征是一种发生在女童中的进行性脑病，涉及运动、肌张力和认知障碍。为了验证疾病早期纹状体D2受体数量增加这一假说，我们测量了11名年龄在4至15岁（平均7.9±3.5岁）的雷特患儿以及8名年龄在3.5至13岁（平均8.1±3.8岁）患有其他神经系统疾病的对照受试者中特异性D2配体123I-碘利舒脲的结合潜能。还使用133Xe单光子发射计算机断层扫描（SPECT）测量了局部脑血流量（rCBF）。雷特患儿中123I-ILIS与D2受体的结合潜能（0.45）显著高于对照组（0.23）（p<0.01）。排除了因患者纹状体rCBF增加导致123I-ILIS结合增加的情况。我们的结果与雷特综合征年轻患者中由于多巴胺能神经传递减少导致D2受体密度较高一致。
