Study on the relationship between electroclinical characteristics and prognosis of epilepsy with myoclonic absence
10.3760/cma.j.issn.1673-4408.2024.09.010
- VernacularTitle:肌阵挛失神癫痫电临床特征与预后关系研究
- Author:
Jiaoyang LU
1
;
Yue NIU
;
Yuehua ZHANG
;
Zhixian YANG
Author Information
1. 北京大学人民医院儿科 100000
- Keywords:
Electroclinical characteristics;
Myoclonic absence seizure;
Epilepsy with myoclonic absence;
Prognosis
- From:
International Journal of Pediatrics
2024;51(9):621-627
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the electroclinical characteristics of patients with epilepsy with myoclonic absence（EMA）and analyze the relationship with prognosis.Methods:Clinical data of 25 patients with EMA monitored at the pediatric EEG monitoring centers of Peking University People's Hospital and Peking University First Hospital between January 2012 and December 2022 were retrospectively analyzed and divided into three groups according to development before and after the onset of the disease to analyze the electroclinical characteristics and the relationship with prognosis.Results:There were 14 males and 11 females in 25 cases，and the median age of epilepy onset was 48（26，74）months.Sixteen cases in the group with normal development before and after the onset of epilepsy（group A），5 cases in the group with normal development before the onset of epilepsy but retarded development after the onset of epilepsy（group B），and 4 cases in the group with retarded development before and after the onset of epilepsy（group C）.The median age at onset was 62（36，82）months，34（21，66）months，and 26（20，32）months in the three groups，with 3，3，and 4 cases of early onset in each group，respectively.The EEG background activity slowed down in 10 cases，with 6，1 and 3 cases in the three groups，respectively.Interictal EEG was normal in 1 and abnormal in 24 cases，which showed generalized discharges，of which 11 cases showed coexisting focal discharges and generalized discharges.Among the focal discharges，there were cases in all three groups，involving the anterior-posterior，temporal and Rolandic regions.Fifteen cases had myoclonic absence（MA）induced by hyperventilation，with 10 cases in group A，4 cases in group B and 1 case in group C.The most prevalent concomitant seizure was myoclonic seizure（MS），with 9，3 and 2 cases in each group respectively.Statistically significant differences were seen in early onset and refractory EMA among the three groups（both P<0.05）.In further two by two comparisons，the proportion of early onset and drug refractory cases was greater in children in group C than in group A，with statistically significant differences（both P<0.017），and the difference in concomitant MS among the three groups was not statistically significant（ P>0.05）. Conclusions:The MA seizures in children with EMA are sensitive to hyperventilation.The common accompanying seizure is MS.Some children with EMA present with early onset and refractory epilepsy，with a tendency towards developmental epileptic encephalopathy.
