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首页> 外文期刊>European journal of paediatric neurology: EJPN : official journal of the European Paediatric Neurology Society >Iomazenil hyperfixation in single photon emission computed tomography study of malformations of cortical development during infancy.
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Iomazenil hyperfixation in single photon emission computed tomography study of malformations of cortical development during infancy.

机译：婴儿期皮层发育畸形的单光子发射计算机断层扫描研究中的伊马西尼超固定。

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摘要

We present 2 cases of malformations of cortical development and early onset epilepsy. The first case is of a patient with left hemimegalencephaly who developed focal epilepsy at the age of 2 days and cluster spasms at 1.5 months. After left functional hemispherectomy, seizures originated from the contralateral hemisphere, which had shown normal signals in the preoperative magnetic resonance imaging study. The second case is of a patient with lissencephaly, caused by a missense mutation in the doublecortin gene, who developed West syndrome at the age of 5 months. In both the cases, (123)I-iomazenil single photon emission computed tomography performed during infancy showed significant hyperfixation in the dysplastic lesions. This finding indicates the immaturity of the affected neurons and a gamma-aminobutyric acidergic involvement in epileptogenesis associated with malformations of cortical development during infancy.
机译：我们目前有2例皮质发育畸形和早期发作性癫痫。第一例是左半巨脑患者，在2天时出现局灶性癫痫，在1.5个月时出现丛集性痉挛。左功能性半球切除术后，癫痫发作来自对侧半球，在术前磁共振成像研究中显示正常信号。第二例是由双皮质素基因的错义突变引起的小脑畸形患者，他在5个月大时发展为West综合征。在这两种情况下，婴儿期进行的（123）I-iomazenil单光子发射计算机断层扫描显示出增生性病变中的明显过度固定。该发现表明受影响的神经元的不成熟和在婴儿期皮质发育异常相关的癫痫发生中的γ-氨基丁酸能。

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