偏侧惊厥-偏瘫-癫癎综合征的临床特点及诊断

Clinical Characteristics and Diagnosis of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome

目的探讨偏侧惊厥-偏瘫-癫癎(HHE)综合征的临床特点及诊断。方法对5例HHE综合征患儿的临床资料进行回顾性分析,总结临床特征及其发病的危险因素,进行必要的辅助检查,包括头颅CT和(或)MRI、脑电图及诊断性智力测定或精神运动发育评价。结果4例4岁内起病,3例伴热性惊厥,最长惊厥时间均在2 h以上,并惊厥侧肢体偏瘫。5例均在偏瘫后2年内出现癫癎反复发作。4例为局限性运动发作,1例为精神运动性发作。5例均存在智力障碍或精神发育迟滞。头颅MRI检查4例发现左侧海马硬化,1例CT示右半球萎缩。脑电图均异常,4例见异常放电,1例示明显不对称。5例均予卡马西平为主的药物治疗,癫癎发作得到控制或部分控制。结论HHE综合征是持续偏侧惊厥导致的偏瘫-癫癎综合征,海马硬化可能是反复癫癎所致海马的继发性损伤,而非癫癎的起源灶。应提高对该病的认识,早期正确处理惊厥持续状态将减少HHE综合征发生。

Objective To summarize the clinical characteristics and diagnosis of hernieonvulsion - hemiplegia - epilepsy （ HHE ） syndrome. Methods The clinical data of 5 children with HHE syndrome were analyzed, Cranial magnetic resonance imaging MRI and/or computerized tomography （CT） and electroencephalogram （EEG） were done in all patients. Results The age of onset was less than 4 years in 4 of 5 patients and initial seizures were associated with fever in 3 eases. The longest duration of convulsive seizures lasted more than 2 hours in all eases, followed by the appearance of hemiplegia involving the side of the body where the convulsions were localized. Subsequent epilepsy developed within 2 years of the hemiplegia in all 5 eases, with 4 eases simple partial seizure and 1 case psychomotor seizures. All eases had mental retardation. Cranial MRI showed left hippoeampal sclerosis in 4 patients and CT brain scan showed right cerebral hemiatrophy in 1 ease. The EEG showed abnormality in all patients. Epileptiform discharges were found in 4 eases, and the damage hemisphere showed slowing and low voltage of background activity in 1 ease. All eases received mainly earbamazepine therapy, and the seizures were satisfactorily controlled or partly controlled. Conclusions HHE syndrome is recognized as hemiplegia - epilepsy syndrome after prolonged hemieonvulsion. The hippocampal sclerosis may be the consequence of repeated seizures, but not the cause. Better understanding of the syndrome, early and adequate treatment of prolonged seizures, especially of febrile convulsion, will reduce the incidence of HHE syndrome.