Salih M A, Kabiraj M, Al-Jarallah A S, El Desouki M, Othman S, Palkar V A
Department of Paediatrics, College of Medicine, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.
Childs Nerv Syst. 1997 May;13(5):257-63. doi: 10.1007/s003810050079.
Six patients (4 boys and 2 girls) with hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome are described. They had prolonged seizures, lasting from 30 min to 12 h, at ages 1-4 years. These took the form of hemiconvulsion in three of the children and generalized tonic-clonic seizures in the others, being preceded by hemifacial twitching or head and eye deviation in two. They were followed by hemiplegia, which cleared with time in five patients, apart from subtle pyramidal tract signs. One child had spastic quadriparesis, choreiform movements, contracture deformities and severe mental retardation following repeated status epilepticus. Subsequent epilepsy developed in five patients and was satisfactorily controlled with carbamazepine and/or phenobarbitone. Cerebral hemiatrophy was documented in all patients by cranial computed tomography and/or magnetic resonance imaging. Single photon emission computed tomography (done in 4 patients) showed ipsilateral hypoperfusion (of the damaged hemisphere). Electroencephalography showed ipsilateral slowing and low voltage of background activity. Epileptiform discharges were found on the ipsilateral side in two cases and the contralateral side (the undamaged hemisphere) in one.
本文描述了6例患有偏瘫-偏瘫-癫痫（HHE）综合征的患者（4名男孩和2名女孩）。他们在1至4岁时出现了持续30分钟至12小时的长时间癫痫发作。其中3名儿童表现为偏瘫性惊厥，其他儿童表现为全身性强直-阵挛性惊厥，其中2名儿童在发作前出现半侧面部抽搐或头部和眼睛偏斜。随后出现偏瘫，5名患者的偏瘫随着时间推移逐渐恢复，仅遗留轻微锥体束征。1名儿童在反复癫痫持续状态后出现痉挛性四肢瘫、舞蹈样动作、挛缩畸形和严重智力障碍。5名患者随后发展为癫痫，使用卡马西平和/或苯巴比妥可得到满意控制。所有患者均通过头颅计算机断层扫描和/或磁共振成像记录到脑半球萎缩。4名患者进行了单光子发射计算机断层扫描，显示受损半球同侧灌注减低。脑电图显示同侧背景活动减慢和低电压。2例患者在同侧发现癫痫样放电，1例在对侧（未受损半球）发现癫痫样放电。
