Komaki H, Sugai K, Maehara T, Shimizu H
Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, 4-1-1 Ogawahigashi-cho, Kodaira, Tokyo 187-8551, Japan.
Brain Dev. 2001 Nov;23(7):727-31. doi: 10.1016/s0387-7604(01)00287-x.
A surgically treated case of early-infantile epileptic encephalopathy (EIEE) with suppression-bursts associated with focal cortical dysplasia is reported. Tonic-clonic seizures followed by a series of spasms occurred at age of a few days. Interictal electroencephalogram (EEG) revealed a suppression-burst pattern and magnetic resonance imaging suggested focal cortical dysplasia in the left prefrontal area. Combination therapies of antiepileptic treatments showed only partial efficacy. The patient underwent lesionectomy at 4 months of age and the spasms decreased to zero to two series daily. At age 2 years, his seizures increased in number and EEG showed that residual left hemisphere was the main epileptogenic focus. Modified functional hemispherectomy of the left hemisphere was applied at age 3 years. The patient, now 5 years old, is free from seizure and gaining psychomotor development gradually. In cases of EIEE with focal cortical dysplasia, surgical treatment may have beneficial effects on both psychomotor development and seizure control.
本文报告了一例经手术治疗的早期婴儿癫痫性脑病(EIEE),伴有与局灶性皮质发育异常相关的抑制爆发。患儿在出生几天后出现强直阵挛发作,随后是一系列痉挛发作。发作间期脑电图(EEG)显示为抑制爆发模式,磁共振成像提示左侧前额叶区域存在局灶性皮质发育异常。抗癫痫联合治疗仅显示部分疗效。患儿在4个月大时接受了病灶切除术,痉挛发作减少至每天零至两次。2岁时,其癫痫发作次数增加,脑电图显示残余的左半球是主要的致痫灶。3岁时对患儿实施了左侧改良功能性半球切除术。该患儿现在5岁,已无癫痫发作,并逐渐获得精神运动发育。对于伴有局灶性皮质发育异常的EIEE病例,手术治疗可能对精神运动发育和癫痫控制均有有益影响。