Clinical And EEG Follow-up Study Of Benign Childhood Epilepsy With Central-temporal Spikes With Electrical Status Epilepticus During Sleep

Background and purpose: Benign childhood epilepsy with central-temporal sp-ikes is the most common benign childhood epilepsy syndrome. It has specific clinicalsymptoms and EEG discharge characteristics and its prognosis is good. However, withthe development of medicine, the growing body of research demonstrated that the pro-gnosis of part of BCECTS was not ideal, not always benign. Some children showed c-ognitive and behavioral disorders, attention deficit hyperactivity disorder (ADHD), s-ome children were associated with sleep epilepsy with electrical status (ESES), Land-au-Kleffner syndrome (LKS). ESES is a special EEG phenomenon. It mainly refers tocontinuous spike and wave distribution during the NREM period of sleep and is oftenaccompanied by varying degrees of neuropsychological impairment. Therefore, earlydiagnosis and effective treatment of ESES can improve its long-term prognosis. In thisstudy, we retrospectively analyzed the clinical and EEG characteristics, treatment res-ponse and prognosis of11children with BCECTS with ESES in order to provide a re-ference for the clinical diagnosis and treatment of BCECTS with ESES.Materials and Methods: The clinical, EEG and therapeutic response in11chil-dren who were diagnosed as BCECTS with ESES in pediatric epilepsy clinic in theChina-Japan Union Hospital of Jilin University between January2005and October2011were followed up clinically. Diagnostic criteria:①BCECTS: BCECTS diagnos-tic criteria proposed by the ILAE in1989.②ESES: Spike-Wave Index(SWI)≥85%.The calculation method of SWI: Duration of total spike slow wave complexes(min)/NREM sleep time(min). We recorded the general information (including gender, birthhistory, history of febrile seizures or a family history of epilepsy, the situation of neur-opsychological development), the clinical data before and after the diagnosis of BCE- CTS (including age of onset,the characteristic of clinical onset, EEG features, neuroi-maging characteristics, the situation of neuropsychological development, treatment a-nd its efficacy), the clinical data before and after the time that ESES was finding (incl-uding the time that ESES was finding, the change of characteristic of clinical onset, E-EG features while the children were diagnosed as having ESES, the change of neuroi-maging characteristics), the data of treatment and follow-up for ESES (including trea-tment,clinical situation, EEG improvement, the recovery of neuropsychological impa-irrment and follow-up time) of all children. We had summarized and analyzed all theresults.Results：(1)4males and7females.(2) Age of onset was9years1month to9y-ears8months, average age was6.8±1.2years. When children were diagnosed as B-CECTS, three of them were partial seizures and eight of them were partial seizures w-ith secondary generalized seizures. A typical central temporal spikes was seen in inte-rictal EEG while awake and sleep in six children,and only in the sleep in five children;the development of neuropsychological and motor of all the children were normal；theneuroimaging of all the children were normal; all patients used AEDs monotherapy,attack was completely controlled in six children, and three children had a demonstrab-le effect, and two children had a effect.(3) the age that ESES appeared was6years10months to11years8months, and the average age was7.9±1.4years. In the diseaseprocess, seizure frequency was increased and new seizure types appeared in five chil-dren，seizure frequency was increased in six children；high amplitude spike and waveclose to the persistent was seen in the whole guide or central temporal during NREMsleep period in interictal EEG, SWI≥85%; all the children accompanied by varying d-egrees of neuropsychological impairment after the occurrence of ESES.(4)Thefollow-up time was11months to6years and9months, the average time was2.7±1.8years. All children were applied to ADEs or ADEs joint oral prednisone,1childwith LEV monotherapy，4children with LEV joint VPA,1children with LEV joint T-PM,2children with VPA joint CZP,2children with VPA joint oral prednisone; attackwas completely controlled in five children, and four children had a demonstrable effe-ct, and two children had a effect; abnormal EEG discharge was completely controlled in one child, four children had a demonstrable effect, two children had a effect and fo-ur children had no effect; the neuropsychological impairment returned to normal in o-ne child, and improved in10children.Conclusions：1.Children with BCECTS with ESES offen accompanied by varying degreesof neuropsychological impairment, therapeutic purposes is not only to control the sei-zures, but also to improve the ongoing epileptiform discharges in order to promote therehabilitation of neuropsychological injury.2.BCECTS with ESES often need combination therapy, VPA joint CZP treatmentis effective, LEV showed efficacy, and some children needed adrenocortical hormonetherapy.