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目的总结偏侧惊厥-偏瘫(HH)综合征和偏侧惊厥-偏瘫-癫(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 hemifacial-hemiplegia (HH) syndrome and hemifacial-hemiparalyzed-epilepsy (HHE) syndrome. Methods The clinical data of 11 HH or HHE children admitted to the First Hospital of Peking University from 1995 to 2003 were analyzed. Eleven patients underwent cranial MRI and / or CT examination, electroencephalogram (EEG) examination and blood biochemical tests, and 6 patients were screened for urinary metabolic disease. Results Eight cases were diagnosed as HHE and three cases were diagnosed as HH. Age of onset from 1 month to 8 years and 5 months, an average of 2 years and 9 months. Four patients had concomitant fever before the first convulsion. 5 cases of hemifacial state before convulsions. 5 cases of right hemiplegia, 6 cases of left hemiplegia. Head imaging showed 2 cases of normal, 7 cases of cerebral hemispheres and clinical symptoms, 1 case of diffuse lesions, 1 case detected unrelated lesions. All 11 cases of EEG were abnormal. Among them, 8 cases of EEG showed obvious asymmetry in bilateral hemispheres and 4 cases of abnormal discharge in 11 cases. 6 cases of metabolic disease screening were negative, 1 case of blood lactate, pyruvate increased, 1 case of elevated blood ammonia. Seven patients treated with carbamazepine-based antiepileptic drugs. Conclusions HHE is a recognized sequelae of convulsive persistence. Clinically, improving the understanding of the disease and active treatment intervention will help to improve its prognosis.