Panayiotopoulos C P
Division of Neurology and Clinical Neurophysiology, King Khalid University Hospital, Riyadh, Saudi Arabia.
Ann Neurol. 1989 Jul;26(1):51-6. doi: 10.1002/ana.410260108.
Eighteen of 418 children who had onset of epilepsy before the age of 13 years showed clinical and electroencephalographic evidence of benign childhood epilepsy with occipital paroxysms. They represented one-fifth of all benign age- and localization-related idiopathic epilepsies seen. Some patients were followed as long as 15 years. There was a preponderance in females and peak age at onset of epilepsy was 5 years. In 16 children, the seizures were infrequent and sometimes prolonged and consisted mainly of tonic deviation of the eyes and vomiting, often with evolution to unilateral or generalized convulsions. Seizures were only nocturnal in 11 and nocturnal and diurnal in another 5 children. Prognosis was excellent; 5 children had only one fit. Remission usually occurred 1 to 2 years after onset and no seizures occurred after the age of 12 years. The remaining 2 children had frequent diurnal episodes consisting of visual hallucinations, postictal headache, and occasional nocturnal hemiconvulsions. Their prognosis was less favorable. Electroencephalographic abnormalities in all 18 patients consisted of repetitive spike and slow-wave discharges confined to the occipital regions and attenuated when the eyes were open. These outlasted clinical remission for many years, sometimes up to the age of 16. Fixation-off sensitivity was demonstrated frequently. Based on these findings, a unifying definition for benign childhood epilepsy with occipital paroxysms is proposed.
在418名13岁前发病的癫痫患儿中,有18名表现出伴有枕叶阵发的良性儿童癫痫的临床和脑电图证据。他们占所有所见良性年龄和定位相关特发性癫痫的五分之一。一些患者随访了长达15年。女性居多,癫痫发作的高峰年龄为5岁。16名儿童的癫痫发作不频繁,有时持续时间长,主要表现为眼球强直性偏斜和呕吐,常演变为单侧或全身性惊厥。11名儿童的癫痫仅在夜间发作,另外5名儿童的癫痫在夜间和白天均有发作。预后良好;5名儿童仅发作一次。缓解通常在发病后1至2年出现,12岁后无癫痫发作。其余2名儿童白天频繁发作,包括视幻觉、发作后头痛和偶尔的夜间偏瘫性惊厥。他们的预后较差。所有18例患者的脑电图异常均表现为局限于枕叶区域的重复性棘波和慢波放电,睁眼时减弱。这些异常在临床缓解后持续多年,有时直至16岁。经常表现出闭眼敏感。基于这些发现,提出了伴有枕叶阵发的良性儿童癫痫的统一定义。