儿童良性枕叶癫癎的临床及脑电图特点

Clinical and electroencephalographic features in children with benign occipital epilepsy

摘要:

目的：分析比较Panayiotopoulos型及Gastaut型儿童良性枕叶癫癎（BOE）的临床、脑电图特点。方法：回顾性分析16例Panayiotopoulos型及7例Gastaut型BOE患儿的临床资料。结果：Panayiotopoulos型BOE平均起病年龄4.5岁，Gastaut型9.1岁，前者临床特征为发作期的呕吐，惊厥形式多为头眼偏转发作，以夜间为多，常泛化为全身发作，持续时间较长，但发作频率较低；Gastaut型枕叶癫癎视觉症状更常见，发作频率高, 均为白天发作，发作较少全身泛化，6例伴有明显的头痛。23例患儿脑电图均显示枕区为主的后头部高幅癎性放电,常为闭眼诱发，睁眼抑制。Panayiotopoulos型6例及Gastaut型5例患儿进行了睁闭眼试验，Panayiotopoulos型和Gastaut型各4例显示睁眼抑制现象。11 例Panayiotopoulos型及所有7例Gastaut型患儿接受抗癫癎单药治疗，11 例Panayiotopoulos型患儿均对药物反应良好，Gastaut型2例无效，2例伴认知功能障碍。结论：Panayiotopoulos型及Gastaut型儿童BOE在起病年龄、发作频率、持续时间、昼夜分布、视觉症状及对治疗的反应等方面均有差别。［中国当代儿科杂志，2010，12（7）：527-529］

Abstract:

OBJECTIVE: To study and compare the clinical and electroencephalography (EEG) features in children with benign occipital epilepsy (BOE) of Gastaut and Panayiotopoulos types. METHODS: The clinical data of 23 children with BOE (16 Gastaut type and 7 Panayiotopoulos type) were retrospectively studied. RESULTS: The mean age of onset in the Panayiotopoulos and Gastaut groups were 4.5 and 9.1 years, respectively. The children in the Panayiotopoulos group were characterized by ictal vomiting, frequent deviation of eyes and head, frequent nocturnal seizures, and secondary generalized seizures with longer duration but less frequency. By comparison, the children in the Gastaut group were characterized by visual symptoms as ictal events, higher seizure frequency, shorter seizure duration and more frequent diurnal seizures. The EEG showed that the majority of both groups had occipital spike-wave discharges when the eyes were opened. Eleven children in the Panayiotopoulos group and all of 7 children in the Gastaut group received antiepileptic mono-drug therapy. All of the 11 children in the Panayiotopoulos group responded to the therapy, but 2 cases in the Gastaut group did not respond and 2 cases had subtle cognitive deficits. CONCLUSIONS: There are differences in the age of onset, clinical symptoms, seizure frequency and duration, and therapeutic responses between children with Panayiotopoulos and Gastaut type BOE.［Chin J Contemp Pediatr, 2010, 12 (7):527-529］