Video EEG Of Seizures In Children With Angelman Syndrome

Objective:By analyzing the video electroencephalograms(EEG)in children with Angelman syndrome(AS),the characteristics of ictal and interictal EEG were studied to further clarify the type of seizures.Methods:The EEG were retrospetively reviewed,these children were genetically diagnosed with Angelman syndrome,and were from the section of Pediatric Neurology,between October 2008 to October 2017,special interest was focused to seizures,ictal and interictal EEG,and other factors including age,gender,genetics and type of seizures was studied.Results:A total of 17 childhood cases with Angelman syndrome were diagnosed by molecular genetics,8 were boys,9 cases were girls.At least one EEG records were performed,totally 33 records collected.Chromosomal microarray showed 15q11-13 maternal deletion.Ages in EEG monitoring: 0-12 months in 7 cases(21.2%),age 1-3 years in 20 cases(60.6%),age 3-7years in 6 cases(18.2%).184 seizure episodes,including at wake 83 episodes(45.0%),101 episodes during sleep(55.0%)were captured.There were45 episodes of generalized seizures: atypical absences,myoclonus,atonic;focal seizures119 times: focal myoclonic seizures,frontal lobe absence seizures,hypermotor seizures,and other types of focal seizures.Focal seizures were origin clear,the origin of the frontal pole in 49 times(41.2%),posterior temporal origin in 41(34.5%),the central origin of the14(11.8%),combined frontal and occipital regions of origin in 15(12.5%).20 times epileptic spasms were recorded.The EEG of all types of seizures was different from the background,and the different types of seizures had corresponding EEG features.The interictal EEG was mainly manifested in the frontal and occipital temporal regions,including the sharp(spike)waves,the sharp(spike)and slow waves,paroxysmal waves in mixed pattern persisting medium to long periods intermittently,and the electrical status epilepticus during sleep(ESES)background was seen during sleep.Conclusion:Through the analysis of 33 records of EEG in 17 children,the following conclusions were reached.1.There are various types of epileptic seizures in children with AS,which have both generalized and focal origin.Previous studies have shown that atypical absence and myoclonic seizures in children with AS are the main seizure type.This study found that there are more focal seizures.Therefore,we need further research on the types and significance of AS seizures in the future.2.The origin of focal seizures is diverse,suggesting that brain dysfunction is widespread.