Chen H L, Wang P J, Tu Y K, Tseng S H, Yao Y T, Young C, Shen Y Z
Department of Pediatrics, National Taiwan University Hospital, Taipei, R.O.C.
J Formos Med Assoc. 1994 Nov-Dec;93(11-12):961-6.
Hemimegalencephaly is a rare congenital anomaly of the brain characterized by unilateral hypertrophy, usually with an abnormal gyri pattern and derangement of the cortical architecture. We report 2 patients with hemimegalencephaly who presented with early-onset seizures, hemiparesis and developmental delay. An electroencephalogram showed hemispheric continuous spikes and spike-and-waves in one patient and repetitive spike-and-waves in the other. Magnetic resonance imaging showed left hemimegalencephaly in both cases and in case 1 pachygyria and heterotopia. A functional hemispherectomy was done on case 1 at the age of 33 months and on case 2 at 7 months due to difficulty in seizure control with antiepileptic drugs. The frequency of seizure dramatically decreased and there was an obvious improvement in neurologic development after surgical intervention. Pathology revealed disorganized lamination of the cortical layers with increased neuron size and bizarre-shaped neurons in both cases. Heterotopia of neurons and glia in the subarachnoid space was noted in case 1. Hemispherectomy should be performed as soon as possible when medical treatment fails to control seizures.
半侧巨脑畸形是一种罕见的先天性脑异常，其特征为单侧脑肥大，通常伴有异常的脑回模式和皮质结构紊乱。我们报告2例半侧巨脑畸形患者，他们表现为早发性癫痫、偏瘫和发育迟缓。脑电图显示，1例患者为半球持续性棘波和棘慢波，另1例为重复性棘慢波。磁共振成像显示，2例均为左侧半侧巨脑畸形，病例1还伴有巨脑回和异位症。由于抗癫痫药物难以控制癫痫发作，病例1在33个月大时、病例2在7个月大时进行了功能性大脑半球切除术。术后癫痫发作频率显著降低，神经发育有明显改善。病理检查显示，2例患者均有皮质层排列紊乱，神经元大小增加，神经元形态怪异。病例1还发现蛛网膜下腔存在神经元和胶质细胞异位。当药物治疗无法控制癫痫发作时，应尽快进行大脑半球切除术。
