Tagawa T, Itagaki Y, Kobayashi M, Sano T, Sumi K
Department of Pediatrics, Osaka Kouseinenkin Hospital, Japan.
Pediatr Neurol. 1999 Aug;21(2):579-82. doi: 10.1016/s0887-8994(99)00043-0.
A 9-year-old male with congenital bilateral perisylvian syndrome is described. He had pseudobulbar palsy, mental retardation, and intractable epilepsy. Computed tomography and magnetic resonance images of the brain demonstrated bilateral perisylvian malformations and a diffuse pachygyric appearance. At 8 years of age, he had episodes of excessive drooling, fluctuating impairment of consciousness, unsteady sitting, and frequent head drop that lasted several days. The electroencephalogram demonstrated continuous diffuse slow spike and waves. These findings suggested atypical absence status epilepticus. Intravenous administration of diazepam resulted in transient improvement of clinical and electroencephalographic findings. Status epilepticus recurred within several minutes after diazepam administration. Although no patient has been reported to have a history of status epilepticus among those affected by this syndrome, it seems that atypical absence status can occur more frequently than expected, as seen in Lennox-Gastaut syndrome. After recognition and confirmation of nonconvulsive status epilepticus, immediate treatment must be attempted.
本文描述了一名患有先天性双侧外侧裂周综合征的9岁男性。他患有假性球麻痹、智力障碍和顽固性癫痫。脑部计算机断层扫描和磁共振成像显示双侧外侧裂周畸形以及弥漫性巨脑回外观。8岁时，他出现了流涎过多、意识波动障碍、坐姿不稳和频繁头部下垂的症状，持续了几天。脑电图显示持续弥漫性慢棘波和慢波。这些发现提示为非典型失神癫痫持续状态。静脉注射地西泮使临床和脑电图表现暂时改善。地西泮给药后几分钟内癫痫持续状态复发。尽管在受该综合征影响的患者中尚未有报告称有癫痫持续状态病史，但似乎非典型失神状态比预期更频繁发生，如在Lennox-Gastaut综合征中所见。在识别并确认非惊厥性癫痫持续状态后，必须立即尝试进行治疗。
