A Study On Pathogenesis And Clinical Efficacy Of Electrical Status Epilepticus During Sleep In Children

Objective:Electrical status epilepticus during sleep (ESES) is a specific electroencephalography (EEG) phenomenon with a characteristic EEG pattern consists of continuous and diffused spike waves mainly at 1.5 Hz to 3.0 Hz during slow sleep. Spike-wave index (SWI) has a central function in the diagnosis of ESES. ESES syndrome is a rare and serious epileptic encephalopathy characterized by epilepsy, neuropsychological impairment and a specific electroencephalographic pattern of ESES. The present study analyzed the etiology, pathogenesis, clinical manifestations and AEDs efficacy of ESES patients from multiple clinical centers in China, aiming to provide assistance for the diagnosis and therapy of ESES.Part one:Syndrome of Electrical Status Epilepticus during Sleep:an epileptic encephalopathy related with brain development.Method:The etiologies of ESES in patients from 5 clinical centers from 2009 to 2013 were retrospectively analyzed. Patients were classified into idiopathic group and symptomatic group. Demographics of all the ESES patients were analyzed. The ages at onset of epilepsy and onset of ESES were compared with the brain development graph reported by Insel, et al. In addition, the age at onset of epilepsy, the age at onset of ESES and the median time between the onset of first seizures and onset of ESES were compared between idiopathic and symptomatic groups.Result:The etiologies of ESES in 75 patients were mainly involving atypical benign childhood epilepsy with centrotemporal spikes (BECT), Landau-Kleffner syndrome (LKS), polymicrogyria, migrational disorders, inappropriate use of antiepileptic drugs and autoimmune disorders, etc. The ages at onset of epilepsy did not show any regularity. The age at onset of epilepsy in idiopathic group was significantly older than that in symptomatic group (P<0.05). However, the ages at onset of ESES tended to concentrate in 6 to 9 years of age. No significant difference was found regarding to the age at onset of ESES between the idiopathic and symptomatic groups.Conclusion:ESES is an Epileptic Encephalopathy which relates with the development of brain. The occurrence of ESES presents obvious age dependence.Part two:Efficacy of levetiracetam in Electrical Status Epilepticus during Sleep of children:a multicenter experience.Method:A multicenter, retrospective, open-label study was taken from 2009 to 2013 in 7 clinical centers. Patients were recruited based on the inclusion and exclusion criteria. Levetiracetam (LEV) was administrated to all patients either as monotherapy or add-on therapy. The response rates of LEV in reducing seizures and improving EEG among all ESES patients were analyzed. The differences of response rates between idiopathic and symptomatic groups, monotherapy and add-on therapy groups were compared.Results:After a mean treatment period of 19 months (range:6 to 24 months), 33 (63.5%) of 52 patients became seizure-free or had experienced remarkable reduction in seizures. The electrical status epilepticus of 41 (56.2%) of 73 patients disappeared off their electroencephalography. The electroencephalography efficacy of levetiracetam treatment was noted in the monotherapy (61.9%) and add-on (53.9%) groups. The clinical (67.7%) and electroencephalography (64.3%) response rates of the idiopathic group were better than those of the symptomatic group (57.1% and 45.2%, respectively). No patient discontinued the trial because of intolerability of side effects.Conclusion:Levetiracetam is effective in individuals with electrical status epilepticus during sleep with tolerable side effects.