Focal glucose hypermetabolism in interictal state of West syndrome
Focal glucose hypermetabolism in interictal state of West syndrome
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DOI：
10.1016/j.pediatrneurol.2005.05.019
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发表时间：
2006-01-01
影响因子：
3.8
通讯作者：
Ito, M
中科院分区：
文献类型：
--
作者：
Kumada, T;Okazawa, H;Ito, M
这份报告涉及两个兄弟姐妹从四胞胎，他们都有西方综合征与非典型的结果，正电子发射断层扫描使用[F-18]氟脱氧葡萄糖。一个表现为脑室周围白质脑病，另一个由于胎儿窘迫和新生儿期脑实质出血而表现为脑室周围白质脑病和脑穿通畸形。他们在9个月大时患上了韦斯特综合征。氟脱氧葡萄糖-正电子发射断层扫描研究后，他们的癫痫发作停止显示葡萄糖代谢增加。相应区域在[C-11]氟马西尼正电子发射断层扫描中呈现低水平蓄积。患者在1个月以上的时间里没有癫痫发作，他们的脑电图只是偶尔显示零星的阵发性放电。由于这些病变中苯二氮卓受体密度降低，兴奋性神经元系统的活性可能过度表达抑制性神经元系统的活性，从而导致病变的癫痫发生。这表明，氟脱氧葡萄糖和氟马西尼-正电子发射断层扫描显示功能异常，这些患者的癫痫发生仍然是活跃的，即使当患者是无脑电和脑电图上有轻微的致痫性放电。(c)2006年，Elsevier Inc. All rights reserved.
This report concerns two siblings from a tetrad, both of whom had West syndrome with atypical findings on positron emission tomography using [F-18] fluorodeoxyglucose. One manifested periventricular leukoencephalopathy, and the other had periventricular leukoencephalopathy as well as porencephaly because of fetal distress and brain parenchymal hemorrhage in the neonatal period. They developed West syndrome at the age of 9 months. Fluorodeoxyglucose-positron emission tomography study performed after cessation of their seizures revealed an increase in glucose metabolism. The corresponding region presented low-level accumulation in [C-11]flumazenil positron emission tomography. The patients remained seizure-free for more than 1 month, and their electroencephalograms only occasionally disclosed sporadic paroxysmal discharges. Because of the decreased density of benzodiazepine receptor in these lesions, the activity of the excitatory neuron system may overexpress that of the inhibitory neuron system, thus resulting in epileptogenesis of the lesions. It is suggested that fluorodeoxyglucose and flumazenil-positron emission tomography revealed functional abnormalities and that epileptogenesis of these patients is still active even when the patient is seizure-free and there are mild epileptogenic discharges on electroencephalogram. (c) 2006 by Elsevier Inc. All rights reserved.