Hanai Sae, Okazaki Ken-ichi, Fujikawa Yoshinao, Nakagawa Eiji, Sugai Kenji, Sasaki Masayuki, Otsuki Taisuke
Departments of Child Neurology, National Center Hospital for Neurology and Psychiatry, National Center of Neurology and Psychiatry, 4-1-1 Ogawahigashi-cho, Kodaira Tokyo 187-8551, Japan.
Brain Dev. 2010 Jun;32(6):499-501. doi: 10.1016/j.braindev.2009.06.005. Epub 2009 Jul 22.
We present a male infant with hemifacial seizures refractory to antiepileptic medication. Hemifacial spasms around the left eye were frequent during wakefulness and sleep since birth. He also had mild psychomotor retardation. Magnetic resonance imaging (MRI) revealed a large tumor in the left middle cerebellar peduncle. Ictal single photon emission computed tomography (SPECT) and ictal (18)F-fluorodeoxyglucose [(18)F-FDG] positron emission tomography (PET) revealed hyperperfusion and hyper glucose metabolism at the tumor. Total removal of the tumor resulted in complete disappearance of hemifacial seizures and improved psychomotor development, indicating that the cerebellar tumor caused hemifacial seizures. A histopathological study confirmed that the tumor was a ganglioglioma. This case and the literature on similar cases indicated that this was a new epileptic syndrome originating in the cerebellum. Early diagnosis and early complete removal of the epileptogenic lesion should be recommended for this syndrome.
我们报告一名患有抗癫痫药物难治性半侧面部癫痫发作的男婴。自出生以来，其左眼周围频繁出现半侧面部痉挛，清醒和睡眠时均有发作。他还伴有轻度精神运动发育迟缓。磁共振成像（MRI）显示左中脑桥小脑角有一个大肿瘤。发作期单光子发射计算机断层扫描（SPECT）和发作期（18）F - 氟脱氧葡萄糖[（18）F - FDG]正电子发射断层扫描（PET）显示肿瘤部位有血流灌注增加和葡萄糖代谢增高。肿瘤全切后，半侧面部癫痫发作完全消失，精神运动发育改善，表明小脑肿瘤导致了半侧面部癫痫发作。组织病理学研究证实该肿瘤为神经节胶质瘤。此病例及类似病例的文献表明这是一种起源于小脑的新型癫痫综合征。对于该综合征，建议早期诊断并尽早完全切除致痫灶。
