Tags

Type your tag names separated by a space and hit enter

Electrical status epilepticus in sleep.
Semin Pediatr Neurol. 2008 Jun; 15(2):50-60.SP

Abstract

Electrical status epilepticus in sleep (ESES) describes an electroencephalographic pattern showing significant activation of epileptiform discharges in sleep. The terms continuous spike wave in slow-wave sleep (CSWS) and Landau-Kleffner syndrome (LKS) describe the clinical epileptic syndromes seen with ESES. Although there is an overlap between these 2 syndromes, children with CSWS present with a more global regression have more problematic epilepsy and have EEG foci located predominantly in frontotemporal or frontocentral regions. In contrast, children with LKS present with an acquired auditory agnosia, fewer seizures, and EEG foci in the posterotemporal regions. ESES requires a high degree of clinical suspicion because slow-wave sleep must be recorded to confirm this diagnosis. Treatment of ESES extends beyond just control of the seizures; amelioration of the continuous epileptiform discharge must occur to improve neuropsychological outcome. Although there is little evidence to guide treatment, conventional antiepileptic drugs play only a minimal role. Steroid therapy and high-dose benzodiazepines are most commonly used, but other therapies including intravenous gamma-globulin, the ketogenic diet, and surgical therapy with multiple subpial transaction have shown efficacy in small case series. Although epilepsy resolves with time in most cases, many children are left with significant cognitive or language impairment. Longer duration of ESES appears to be the major predictor of poor outcome; markedly abnormal neuronal activity during a critical period for synaptogenesis may result in aberrant synapse formation, explaining the poorer neuropsychological outcome. Early recognition and effective therapy are necessary to improve long-term prognosis in this condition.

Authors+Show Affiliations

Department of Neurology, Mayo Clinic, Rochester, MN 55902, USA.No affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

18555191

Citation

Nickels, Katherine, and Elaine Wirrell. "Electrical Status Epilepticus in Sleep." Seminars in Pediatric Neurology, vol. 15, no. 2, 2008, pp. 50-60.
Nickels K, Wirrell E. Electrical status epilepticus in sleep. Semin Pediatr Neurol. 2008;15(2):50-60.
Nickels, K., & Wirrell, E. (2008). Electrical status epilepticus in sleep. Seminars in Pediatric Neurology, 15(2), 50-60. https://doi.org/10.1016/j.spen.2008.03.002
Nickels K, Wirrell E. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol. 2008;15(2):50-60. PubMed PMID: 18555191.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Electrical status epilepticus in sleep. AU - Nickels,Katherine, AU - Wirrell,Elaine, PY - 2008/6/17/pubmed PY - 2008/8/20/medline PY - 2008/6/17/entrez SP - 50 EP - 60 JF - Seminars in pediatric neurology JO - Semin Pediatr Neurol VL - 15 IS - 2 N2 - Electrical status epilepticus in sleep (ESES) describes an electroencephalographic pattern showing significant activation of epileptiform discharges in sleep. The terms continuous spike wave in slow-wave sleep (CSWS) and Landau-Kleffner syndrome (LKS) describe the clinical epileptic syndromes seen with ESES. Although there is an overlap between these 2 syndromes, children with CSWS present with a more global regression have more problematic epilepsy and have EEG foci located predominantly in frontotemporal or frontocentral regions. In contrast, children with LKS present with an acquired auditory agnosia, fewer seizures, and EEG foci in the posterotemporal regions. ESES requires a high degree of clinical suspicion because slow-wave sleep must be recorded to confirm this diagnosis. Treatment of ESES extends beyond just control of the seizures; amelioration of the continuous epileptiform discharge must occur to improve neuropsychological outcome. Although there is little evidence to guide treatment, conventional antiepileptic drugs play only a minimal role. Steroid therapy and high-dose benzodiazepines are most commonly used, but other therapies including intravenous gamma-globulin, the ketogenic diet, and surgical therapy with multiple subpial transaction have shown efficacy in small case series. Although epilepsy resolves with time in most cases, many children are left with significant cognitive or language impairment. Longer duration of ESES appears to be the major predictor of poor outcome; markedly abnormal neuronal activity during a critical period for synaptogenesis may result in aberrant synapse formation, explaining the poorer neuropsychological outcome. Early recognition and effective therapy are necessary to improve long-term prognosis in this condition. SN - 1071-9091 UR - https://www.unboundmedicine.com/medline/citation/18555191/Electrical_status_epilepticus_in_sleep_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1071-9091(08)00026-0 DB - PRIME DP - Unbound Medicine ER -