偏侧惊厥-偏瘫和偏侧惊厥-偏瘫-癫综合征11例临床分析  

Clinical characteristics of hemiconvulsion-hemiplegia syndrome and hemiconvulsion-hemiplegia-epilepsy syndrome in 11 patients.

作　　者：杨志仙[1] 秦炯[1] 常杏芝[1] 包新华[1] 张月华[1] 刘晓燕[1] 姜玉武[1] 杨艳玲[1] 

机构地区：[1]北京大学第一医院儿科,北京100034

出　　处：《中国实用儿科杂志》2006年第7期522-525,共4页Chinese Journal of Practical Pediatrics

基　　金：卫生部临床学科重点项目基金(20010912)资助

摘　　要：目的总结偏侧惊厥-偏瘫（HH）综合征和偏侧惊厥-偏瘫-癫痈（HHE）综合征的临床特征。方法对北京大学第一医院儿科1995—2003年收治的11例HH或HHE患儿的临床资料进行分析。11例均做了头颅MRI和（或）CT检查、脑电图（EEG）检查及血生化测定，6例做了尿代谢病筛查。结果8例诊断为HHE，3例诊断为HH。发病年龄1个月至8岁5个月，平均为2岁9个月。4例首次惊厥前伴有发热。5例偏瘫前有惊厥持续状态。5例为右侧偏瘫，6例为左侧偏瘫。头颅影像学显示2例正常，7例大脑半球病变与临床症状相对应，1例为弥漫性病变，1例查出无关病变。11例EEG均为异常，其中8例EEG示双侧半球明显不对称，11例中4例有异常放电。6例代谢病筛查均为阴性，1例血乳酸、丙酮酸增高，1例血氨增高。7例采用了以卡马西平为主的抗癫痈药治疗。结论HHE为一种公认的惊厥持续状态导致的后遗症，临床上提高对该病的认识并积极进行治疗干预，有助于改善其预后。Objective To summarize the clinical characteristics of hemiconvulsion-hemiplegia syndrome （ HH ） and hemiconvulsion-hemiplegia-epilepsy syndrome （ HHE ）. Methods The clinical data of 11 children with HH or HHE admitted to the hospital from 1995 to 2003 were analyzed. Cranial MRI and / or CT,EEG and analysis of serum were done in all patients. Urinary organic acids were analysed in 6 patients. Results The 11 patients were all male. Eight cases were diagnosed as HHE,while 3 cases were not observed the epilepsy and diagnosed as HH. The age of onset was from one month to eight years and five months, averaged two years and nine months. There were fever symptoms before initial convulsion in 4 cases. Choky happened during birth process in 3 cases. One patient suffered from vitamin K scarcity at one month old and intercurrent encephalic hemorrhage. One case had a history of head trauma. Status epilepicus occured in 5 cases before hemiplegy appeared. Another 3 cases twitched frequently one day which could not be affirmed status epilepicus because of simpe note. Five cases had hemiplegy symptoms in the right body, while 6 cases in the left body. Cranial MRI or/and CT was normal in 2 patients,7 cases had cerebrum hemisp.here damage or marked unilateral predominance damage which was corresponding to the clinical symptoms. The brain damage was diffuse in one case. One case had right cyst which might be not related to the disease. Brain atrophy was observed in 5 cases. The EEG was abnormal in all patients. The waves in double cerebrum hemisphere were obviously asymmetrical in 8 cases. The damage hemisphere showed slowing and low voltage of background activity. Epileptiform discharges were found in 4 cases. Urinary organic acids analysis was all negative in 6 cases. One patient had elevated lactic and pyruvic acid and another one had ammonia increase in plasma. Most patients mainly received carbamazepine therapy. Conclusion HHE is one of the recognized sequelae of convulsive status epilepticus. Further recognizing this

关 键 词：侧惊厥-偏瘫综合征 偏侧惊厥-偏瘫-癫痫综合征 脑电图 

分 类 号：R72[医药卫生—儿科]