Electrical Status Epilepticus in Sleep

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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.

Original languageEnglish (US)
Pages (from-to)50-60
Number of pages11
JournalSeminars in Pediatric Neurology
Volume15
Issue number2
DOIs
StatePublished - Jun 2008

Fingerprint

Status Epilepticus
Sleep
Landau-Kleffner Syndrome
Electroencephalography
Epilepsy
Seizures
Ketogenic Diet
Agnosia
Diet Therapy
Therapeutics
gamma-Globulins
Benzodiazepines
Anticonvulsants
Synapses
Language
Steroids

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Electrical Status Epilepticus in Sleep. / Nickels, Katherine C; Wirrell, Elaine C.

In: Seminars in Pediatric Neurology, Vol. 15, No. 2, 06.2008, p. 50-60.

Research output: Contribution to journalArticle

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