De Volder A G, Jaeken J, Van den Berghe G, Bol A, Michel C, Cogneau M, Goffinet A M
Positron Tomography Laboratory, University of Louvain, Louvain-la-Neuve, Belgium.
Pediatr Res. 1988 Aug;24(2):238-42. doi: 10.1203/00006450-198808000-00020.
Regional brain glucose utilization was investigated by positron emission tomography with fluorodeoxyglucose in three children with adenylosuccinase deficiency. A consistent pattern was found in the three patients, namely a marked decrease of fluorodeoxyglucose uptake in all gray structures, with the exception of the cerebellum, which was minimally affected. Anomalies predominated in the cerebral cortex, particularly in the anterior regions; they were less pronounced in thalamus and basal ganglia. The observations suggest that positron emission tomography may be a useful tool for the localization of the deleterious effects of metabolic diseases and for the investigation of their pathophysiologic mechanisms.
采用氟代脱氧葡萄糖正电子发射断层扫描技术，对3例腺苷琥珀酸酶缺乏症患儿的局部脑葡萄糖利用情况进行了研究。在这3例患者中发现了一种一致的模式，即除小脑受影响最小外，所有灰质结构中的氟代脱氧葡萄糖摄取均显著减少。异常主要出现在大脑皮层，尤其是前部区域；在丘脑和基底神经节中则不太明显。这些观察结果表明，正电子发射断层扫描可能是一种有用的工具，可用于定位代谢性疾病的有害影响及其病理生理机制的研究。
