al-Essa M A, al-Shamsan L A, Ozand P T
Department of Pediatrics and Biological and Medical Research, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Eur J Paediatr Neurol. 1999;3(3):125-7. doi: 10.1016/s1090-3798(99)90100-9.
We report a 2-year-old boy with ethylmalonic aciduria and vasculopathy syndrome evaluated by 18fluoro-2-deoxyglucose positron emission tomographic (18FDG PET) brain scan, with intense uptake of 18FDG in the caudate nucleus and putamen bilaterally but with no morphological changes on magnetic resonance imaging (MRI). A repeat 18FDG PET brain scan 1 year later showed a significant bilateral decreased uptake of glucose in the putamen and the head of the caudate nucleus as well as a decreased uptake in the frontal lobes. On MRI, there was atrophy and watershed infarcts in the basal ganglia, explaining the loss of glucose uptake. These results reflect a selective vulnerability of the basal ganglia, their functional derangement, and ultimate degeneration.
我们报告了一名患有乙基丙二酸尿症和血管病变综合征的2岁男孩，通过18氟-2-脱氧葡萄糖正电子发射断层扫描（18FDG PET）脑部扫描进行评估，结果显示双侧尾状核和壳核有强烈的18FDG摄取，但磁共振成像（MRI）上无形态学改变。1年后重复进行的18FDG PET脑部扫描显示，壳核和尾状核头部双侧葡萄糖摄取显著降低，额叶摄取也减少。MRI显示基底节萎缩和分水岭梗死，这解释了葡萄糖摄取的丧失。这些结果反映了基底节的选择性易损性、其功能紊乱以及最终的变性。
