Tanaka Y, Hamano S, Nara T, Nakanishi Y
Division of Neurology, Saitama Children's Medical Center.
No To Hattatsu. 1994 Nov;26(6):528.
A case of 1-month-old female infant with hemimegalencephaly was reported. This disorder is a rare malformation characterized by congenital hypertrophy of one hemisphere and ipsilateral ventriculomegaly. Clinical signs of the patient included a left sided macrocephaly, intractable seizures and delayed development. Ictal single photon emission computed tomography (SPECT) on the 49th day using 99mTc-HMPAO showed hyperperfusion in the left occipital and frontal lobes, when ictal EEG showed a sharp wave-burst appearing continuously in the left occipital lobe that spread to the left frontal lobe. Interictal SPECT on the 48th day demonstrated and increased tracer accumulation in the left hemisphere, especially in the left frontal lobe. In this case, the megalencephalic hemisphere might have an important role for epileptogenesis and its spread according to the ictal SPECT findings.
报道了1例1月龄患有半侧巨脑症的女婴。这种疾病是一种罕见的畸形，其特征为一侧半球先天性肥大及同侧脑室扩大。该患者的临床症状包括左侧巨头畸形、顽固性癫痫发作和发育迟缓。在第49天使用99mTc-HMPAO进行发作期单光子发射计算机断层扫描（SPECT）显示，左侧枕叶和额叶血流灌注增加，此时发作期脑电图显示左侧枕叶持续出现尖波暴发并扩散至左侧额叶。第48天的发作间期SPECT显示左侧半球放射性示踪剂积聚增加，尤其是左侧额叶。在该病例中，根据发作期SPECT结果，巨脑半球可能在癫痫发作的发生及其扩散中起重要作用。
