关键词: 偏侧惊厥-偏瘫-癫痫综合征；　颅脑磁共振成像；　儿童

Abstract: 　Objective　To summarize the clinical characteristics and diagnosis of hemiconvulsion -hemiplegia-epilepsy (HHE) syndrome. Methods　The clinical data of 3 children with HHE admitted in the Department of Pediatrics, the Affiliated Hospital of Qingdao University from 2017 to 2019 were retrospectively analyzed with related literatures reviewed. Results　All the 3 cases were female. The age of onset was 3 years old, 1 year and 2 months old, 6 years and 11 months old, respectively. All the first convulsions were accompanied by fever and convulsive status epilepticus. Right hemiplegia occurred in 2 cases and left hemiplegia in 1 case. Abnormal liver function and myocardial enzymes occurred in 1 case. Abnormal changes in cranial magnetic resonance imaging or cranial CT were observed in all 3 children which is in accordance with clinical symptoms. After hemiplegia occurred, 3 cases had recurrent epileptic seizures which were completely controlled temporarily after being treated with oxcarbazepinebased antiepileptic drugs. Gene detection was performed in case 1 and case 2. In case 1, 9.1Mb deletion in chr5q33.3q34 including a large number of functional genes such as GABRA1 and GABRA2 were detected by chromosome microarray. In case 2, no abnormality was found. Conclusion　HHE syndrome is a rare consequence of convulsive status epilepticus in children. The occurrence of HHE syndrome can be effectively reduced through further understanding of the syndrome and early appropriate treatment of status convulsion.

Key words: hemiplegia-hemiplegic-epilepsy syndrome;　brain magnetic resonance imaging;　child