EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPY

Electrical status epilepticus of slow sleep (ESES) is a sleep EEG pattern with a continuous (85-100%) diffuse epileptiform activity. The morphology of these epileptiform complexes is identical to benign epileptiform discharges of childhood (BEDC). Epilepsy with ESES (or Penelope Syndrome) is a form...

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Main Author: A. A. Kholin
Format: Article
Language:Russian
Published: IRBIS LLC 2018-05-01
Series:Эпилепсия и пароксизмальные состояния
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Online Access:https://www.epilepsia.su/jour/article/view/393
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author A. A. Kholin
author_facet A. A. Kholin
author_sort A. A. Kholin
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description Electrical status epilepticus of slow sleep (ESES) is a sleep EEG pattern with a continuous (85-100%) diffuse epileptiform activity. The morphology of these epileptiform complexes is identical to benign epileptiform discharges of childhood (BEDC). Epilepsy with ESES (or Penelope Syndrome) is a form of age-dependent epileptic encephalopathies with continuous spike-wave activity during slow wave sleep. This group of epilepsies also includes Pseudo-Lennox syndrome, Landau-Kleffner syndrome, autistic epileptiform regression and some others. Usually, the ESES pattern correlates with the severity of cognitive deficit in epileptic children. The list of anti-epileptic drugs (AEDs) used in pharmacotherapy of epileptic encephalopathies includes valproates, levetiracetam, ethosuximide, sulthiame, topiramate, zonisamide and benzodiazepines; those are given in monotherapy or rationalized combined therapy. Levetiracetam was highly effective in 67.6% of patients with ESES (n=23); among them, two cases of clinical and EEG remission during the pre-puberty period, nine cases of clinical remission and 12 cases with a significant decrease in seizures and epileptiform discharges. In 20.6% of patients (n=7), the pharmacotherapy showed low efficacy. In 11.8% of patients (n=4), aggravation of the disease was noted. Combinations of levetiracetam with valproates and levetiracetam with ethosuximide were found the most effective AEDs. In the cases of epileptogenic structural defect that underlines drug-resistant epilepsy with ESES, neurosurgical intervention is recommended.
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spelling doaj-art-1e722fde62114584ba91ae583d43c2a32025-08-20T03:57:32ZrusIRBIS LLCЭпилепсия и пароксизмальные состояния2077-83332311-40882018-05-01101637110.17749/2077-8333.2018.10.1.063-071373EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPYA. A. Kholin0N. I. Pirogov Russian National Research Medical University, Ministry of Health of Russia; Russian Children Clinical Hospital, Ministry of Health of RussiaElectrical status epilepticus of slow sleep (ESES) is a sleep EEG pattern with a continuous (85-100%) diffuse epileptiform activity. The morphology of these epileptiform complexes is identical to benign epileptiform discharges of childhood (BEDC). Epilepsy with ESES (or Penelope Syndrome) is a form of age-dependent epileptic encephalopathies with continuous spike-wave activity during slow wave sleep. This group of epilepsies also includes Pseudo-Lennox syndrome, Landau-Kleffner syndrome, autistic epileptiform regression and some others. Usually, the ESES pattern correlates with the severity of cognitive deficit in epileptic children. The list of anti-epileptic drugs (AEDs) used in pharmacotherapy of epileptic encephalopathies includes valproates, levetiracetam, ethosuximide, sulthiame, topiramate, zonisamide and benzodiazepines; those are given in monotherapy or rationalized combined therapy. Levetiracetam was highly effective in 67.6% of patients with ESES (n=23); among them, two cases of clinical and EEG remission during the pre-puberty period, nine cases of clinical remission and 12 cases with a significant decrease in seizures and epileptiform discharges. In 20.6% of patients (n=7), the pharmacotherapy showed low efficacy. In 11.8% of patients (n=4), aggravation of the disease was noted. Combinations of levetiracetam with valproates and levetiracetam with ethosuximide were found the most effective AEDs. In the cases of epileptogenic structural defect that underlines drug-resistant epilepsy with ESES, neurosurgical intervention is recommended.https://www.epilepsia.su/jour/article/view/393epileptic encephalopathieselectrical status epilepticus in slow wave sleepesesbenign epileptiform discharges of childhoodbedcantiepileptic drugsaed
spellingShingle A. A. Kholin
EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPY
Эпилепсия и пароксизмальные состояния
epileptic encephalopathies
electrical status epilepticus in slow wave sleep
eses
benign epileptiform discharges of childhood
bedc
antiepileptic drugs
aed
title EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPY
title_full EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPY
title_fullStr EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPY
title_full_unstemmed EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPY
title_short EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPY
title_sort epileptic encephalopathies with electrical status epilepticus of slow wave sleep eses diagnosis and pharmacotherapy
topic epileptic encephalopathies
electrical status epilepticus in slow wave sleep
eses
benign epileptiform discharges of childhood
bedc
antiepileptic drugs
aed
url https://www.epilepsia.su/jour/article/view/393
work_keys_str_mv AT aakholin epilepticencephalopathieswithelectricalstatusepilepticusofslowwavesleepesesdiagnosisandpharmacotherapy