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首页> 外文期刊>European journal of paediatric neurology: EJPN : official journal of the European Paediatric Neurology Society >Evolution of cortical metabolic abnormalities and their clinical correlates in Sturge-Weber syndrome.
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Evolution of cortical metabolic abnormalities and their clinical correlates in Sturge-Weber syndrome.

机译：Sturge-Weber综合征中皮质代谢异常的演变及其临床相关性。

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BACKGROUND: The natural course of Sturge-Weber syndrome (SWS) is poorly understood, although neurological symptoms are often progressive. AIMS: To track longitudinal changes in brain glucose metabolism measured with positron emission tomography (PET) and their relation to clinical changes during the early course of SWS. METHODS: Fourteen children (age 3 months to 3.9 years at enrollment) with SWS and unilateral leptomeningeal angioma underwent two consecutive glucose metabolism PET scans with a mean follow-up time of 1.2 years. Longitudinal changes of the extent of cortical glucose hypometabolism on the angioma side were measured and correlated with age, clinical seizure frequency and hemiparesis. RESULTS: An increase in the size of the hypometabolic cortex was seen in 6 children, coinciding with an age-related increase in cortical glucose metabolism measured in unaffected contralateral cortex. These 6 patients were younger both at the initial (mean age 0.75 vs. 2.8 years; p<0.001) and the second scan (mean age 1.8 vs. 4.2 years; p=0.001) than those with no change in the extent of hypometabolic cortex (n=6). The area of cortical hypometabolism decreased in the two remaining children, and this was associated with resolution of an initial hemiparesis in one of them. Seizure frequency between the two scans was higher in children who showed progressive enlargement of cortical hypometabolism, as compared to those with no progression (p=0.008). CONCLUSIONS: In SWS, detrimental metabolic changes occur before 3 years of age coinciding with a sharp increase of developmentally regulated cerebral metabolic demand. Progressive hypometabolism is associated with high seizure frequency in these children. However, metabolic abnormalities may remain limited or even partially recover later in some children with well-controlled seizures. Metabolic recovery accompanied by neurological improvement suggests a window for therapeutic intervention in children with unilateral SWS.
机译：背景：尽管神经系统症状通常是进行性的，但人们对Sturge-Weber综合征（SWS）的自然病程知之甚少。目的：追踪SWS早期过程中用正电子发射断层扫描（PET）测量的脑部葡萄糖代谢的纵向变化及其与临床变化的关系。方法：十四名SWS和单侧软脑膜血管瘤患儿（入组年龄在3个月至3.9岁之间）接受了两次连续的糖代谢PET扫描，平均随访时间为1。2年。测量了血管瘤侧的皮质葡萄糖低代谢程度的纵向变化，并与年龄，临床癫痫发作频率和偏瘫相关。结果：6名儿童的代谢不足皮层大小增加，这与年龄相关的对侧皮层皮质糖代谢的增加有关。这6例患者在初次（平均年龄为0.75 vs. 2.8岁; p <0.001）和第二次扫描（平均年龄为1.8 vs. 4.2岁; p = 0.001）时都比低代谢皮质的年龄年轻。 （n = 6）。其余两个孩子的皮质低代谢区域减少，这与其中一个孩子最初的偏瘫缓解有关。与没有进展的儿童相比，显示皮质渐进代谢逐渐增加的儿童两次扫描之间的癫痫发作频率更高（p = 0.008）。结论：在SWS中，有害的代谢变化发生在3岁之前，与发育调节的脑代谢需求的急剧增加相吻合。在这些儿童中，进行性低代谢与癫痫发作频率高有关。但是，对于某些癫痫发作控制良好的儿童，代谢异常可能仍然有限，甚至部分恢复。代谢恢复并伴有神经功能改善，为单侧SWS患儿的治疗干预开辟了一个窗口。

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