FDG-PET study of patients with Leigh syndrome
FDG-PET study of patients with Leigh syndrome
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DOI：
10.1016/j.jns.2016.02.008
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发表时间：
2016-03-15
影响因子：
4.4
通讯作者：
Kure, Shigeo
中科院分区：
文献类型：
--
作者：
Haginoya, Kauzhiro;Kaneta, Tomohiro;Kure, Shigeo
我们对5名Leigh综合征患者（中位年龄11（范围4-13）岁）进行了一项[F-18]氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）研究，以评估其对于了解这种疾病的功能性脑功能障碍的有用性和未来的药物试验。发现4例患者报告了线粒体DNA基因突变。脑T2加权磁共振成像（MRI）显示高信号区在壳核双侧5例，尾状核双侧4例，丘脑双侧2例，脑干1例。在两名老年患者中观察到小脑萎缩。对于疾病控制，选择了7名年龄匹配的癫痫患者，他们的MRI和FDG-PET检查正常。对于F-18-FDG摄取减少的病灶的半定量分析，计算每个脑结构中感兴趣区域（ROI）的平均标准摄取值（SUV）。我们比较了Leigh综合征患者和对照组9个节段（额叶、颞叶、顶叶和枕叶、丘脑、基底节、中脑、脑桥和小脑）的SUV。小脑和基底节区葡萄糖摄取明显减少，这可以解释Leigh综合征患者的共济失调和肌张力障碍。虽然本研究有一定的局限性，但FDG-PET可能有助于评价Leigh综合征患者的脑功能障碍和新药治疗效果。在得出结论之前，需要对更多患者使用先进方法定量葡萄糖摄取进行进一步研究。(C)© 2016 Elsevier B. V.版权所有。
We conducted a [F-18]fluorodeoxyglucose positron emission tomography (FDG-PET) study in five patients (median age 11 (range 4-13) years) with Leigh syndrome to evaluate its usefulness for understanding the functional brain dysfunction in this disease and in future drug trials. Four patients were found to have reported mitochondrial DNA gene mutations. The brain T2-weighted magnetic resonance imaging (MRI) showed high-intensity areas in the putamen bilaterally in five patients, caudate bilaterally in four, thalamus bilaterally in two, and brainstem in one. Cerebellar atrophy was observed in older two patients. For disease control, seven age matched epilepsy patients who had normal MRI and FDG-PET studies were selected. For semiquantitative analysis of the lesions with decreased F-18-FDG uptake, the mean standard uptake value (SUV) was calculated in regions of interest (ROIs) placed in each brain structure. We compared the SUV of nine segments (the frontal, temporal, parietal, and occipital lobes, thalami, basal ganglia, mid-brain, pons, and cerebellum) between patients with Leigh syndrome and controls. The glucose uptake was decreased significantly in the cerebellum and basal ganglia, which could explain the ataxia and dystonia in patients with Leigh syndrome. Although this study had some limitations, FDG-PET might be useful for evaluating the brain dysfunction and treatment efficacy of new drugs in patients with Leigh syndrome. Further study with more patients using advanced methods to quantify glucose uptake is needed before drawing a conclusion. (C) 2016 Elsevier B.V. All rights reserved.