Epileptic encephalopathy with electrical status epilepticus: An electroclinical study of 59 patients

Abstract

To evaluate the electroclinical features, treatment effectiveness, and outcome of 59 patients with epileptic encephalopathy with electrical status epilepticus during sleep. Methods: Medical-files of 59 patients with electrical status epilepticus during sleep were retrospectively evaluated for data concerning: history, physical and neurological examinations, sleep and awake EEG's, psychometric tests and brain MRI. Results: A total of 31 boys and 28 girls were identified. Patients were evaluated in two groups: symptomatic/structural and idiopathic group. There was no significant difference between the etiological groups in term of mean age at ESES onset, mean interval between the first seizure and the onset of ESES. The mean age at seizure onset was earlier in the symptomatic/structural group than the idiopathic ones. The mean follow-up time after the ESES onset was 4.5 years for all patients. The most effective antiepileptic drugs in our series were clobazam and levetiracetam. In refractory patients, steroid treatment was found effective during the early course of the disease. In the idiopathic group, cognitive decline has improved. However in the symptomatic group, patients did not respond to the treatment and cognitive deterioration did not improve in one third of the group. Conclusion:The long-term outcome of ESES is highly variable and usually depends on etiology and the duration of ESES. The most efficious antiepileptic drugs in our study are clobazam and levetiracetam.

Electrical status epilepticus in sleep (ESES)/continuous spikes and waves during slow sleep (CSWS) syndrome in children: An electroclinical evaluation according to the EEG patterns

“…Various degrees of intellectual deterioration were found in 18 of 22 patients (81%) in our cohort. Arhan et al [21] reported that 40.6% of their cohort showed cognitive decline in our country. Attention-deficit hyperactivity disorder was found in 9 of 44 patients in our cohort.…”

Benign epileptiform discharges of childhood and associated conditions

“…ДЭПД являются облигатными ЭЭГ-паттернами при ряде форм эпилептических энцефалопатий, особенно сопровождающихся постоянной продолженной эпилептиформной активностью в фазу медленного сна. Это синдромы псевдо-Леннокса, Ландау-Клеффнера, энцефалопатия с электрическим эпилептическим статусом медленного сна [14,16,28]. Общими признаками ДЭПД на ЭЭГ при всех данных синдромах являются крайне высокий индекс эпилептиформной активности с нарастанием в фазу медленного сна и тенденция к диффузному распространению ДЭПД.…”

“…Причины повреждающего воздействия продолженной эпилептиформной активности на функциональное состояние детского мозга точно не определены. В настоящее время предполагается несколько механизмов такого воздействия [16,25,28,46,89]:…”

Levetiracetam for the treatment of status epilepticus

“…A significant decrease in seizure frequency (>50%) was observed in 35 (70%) of the 50 patients with seizures at the start of LEV therapy, and a positive EEG response was observed during the first 3-4 months of LEV therapy in 32 (45%) of the 71 patients. In addition, Arhan et al [62] performed a retrospective study to assess the efficacy of AED therapy in 59 children with ESES and demonstrated that LEV is one of the two most effective anti-ESES drugs.…”