Shahar E, Desatnik H, Brand N, Straussberg R, Hwang P A
Child Neurology Unit, Rambam Medical Center, Haifa, Israel.
Clin Neurol Neurosurg. 1996 Aug;98(3):237-41. doi: 10.1016/0303-8467(96)00033-9.
The ictal manifestations, EEG, CT, and MRI correlates, as well as the management and outcome of 11 children with epileptic blindness are presented. Seven males and four females, aged 3 months to 12 years, experienced single or recurrent episodes of acute visual obscuration. Ictal blindness was the solitary epileptic phenomenon in only two children. The rest had other focal or generalized motor epileptic manifestations. Six children had either focal motor phenomena and/or unilateral EEG disturbances, with a normal head CT. The drug of choice in this group was carbamazepine and all became asymptomatic. Two patients had structural abnormalities of the brain, of which one had a low-grade occipital astrocytoma which was resected. His blindness abated shortly following initiation of carbamazepine, even prior to surgery. Status epilepticus amauroticus and focal motor seizures, secondary to focal cortical dysplasia, was detected in another 3-month-old infant. These required cortical resection and she regained full vision. Three patients had generalized epileptiform discharges on EEG, of which two were photic-induced. Blindness was accompanied with motor seizures and myoclonic jerks. Full seizure control could be achieved in only one child.
our data suggest a relatively benign nature and a favorable outcome in most children with ictal blindness. Resection of a secondary temporo-parietal focus, as occurred in an infant with status epilepticus amauroticus which originated in the occipital region, may result in complete cessation of seizures and visual recovery.
本文介绍了11例癫痫性失明患儿的发作表现、脑电图(EEG)、计算机断层扫描(CT)和磁共振成像(MRI)相关性,以及治疗和预后情况。7名男性和4名女性,年龄3个月至12岁,经历了单次或反复发作的急性视力模糊。发作性失明仅在2名儿童中是唯一的癫痫现象。其余患儿有其他局灶性或全身性运动性癫痫表现。6名儿童有局灶性运动现象和/或单侧脑电图异常,头部CT正常。该组患儿的首选药物是卡马西平,所有患儿均无症状。2例患儿有脑部结构异常,其中1例有低级别枕叶星形细胞瘤,已行切除。他在开始使用卡马西平后不久失明减轻,甚至在手术前。另一名3个月大的婴儿检测到由局灶性皮质发育不良继发的癫痫性黑蒙状态和局灶性运动性发作。这些情况需要进行皮质切除术,她恢复了完全视力。3例患儿脑电图有全身性癫痫样放电,其中2例由光刺激诱发。失明伴有运动性发作和肌阵挛。仅1名患儿的癫痫发作得到完全控制。
我们的数据表明,大多数发作性失明患儿的病情性质相对良性,预后良好。如一名起源于枕叶的癫痫性黑蒙状态婴儿那样,切除继发的颞顶叶病灶可能导致癫痫发作完全停止和视力恢复。