Electrical Status Epilepticus and Sleep-Related Encephalopathies

Electrical status epilepticus during sleep (ESES) is a paediatric epileptic encephalopathy defined by continuous or near-continuous bilateral spike–wave discharges occupying a high proportion of non-rapid eye movement (NREM) sleep. Clinically, it presents with relatively infrequent overt seizures but profound cognitive, behavioural and motor regression. Subtypes include continuous spike–wave during sleep (CSWS), developmental and epileptic encephalopathy with spike–wave activation in sleep (DEE-SWAS) and focal aphasic variants such as Landau–Kleffner syndrome. Onset typically occurs between three and eight years of age, occasionally following earlier epilepsy syndromes. Underlying causes span monogenic channelopathies, copy number variants and structural lesions. Pathophysiology implicates aberrant thalamocortical oscillations and disrupted sleep-related synaptic plasticity. Management comprises high-dose corticosteroids, benzodiazepines or other antiseizure medications, immunotherapy and, in selected cases, resective surgery. Early diagnosis and targeted intervention are vital to prevent long-term neurodevelopmental impairment.

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Electrical Status Epilepticus and Sleep-Related Encephalopathies publication trend

The graph below shows the total number of articles in electrical status epilepticus and sleep-related encephalopathies across all publications each year (not limited to Nature Index journals).

Technical terms

Electrical status epilepticus during sleep (ESES): An epileptic encephalopathy characterised by continuous or near-continuous spike–wave discharges during NREM sleep, leading to cognitive and behavioural decline.

Continuous spike–wave during sleep (CSWS): A sleep-associated EEG pattern of persistent spike–wave activity often used interchangeably with ESES, associated with developmental regression.

Spike–wave index (SWI): The proportion of slow-wave sleep time occupied by spike–wave discharges, used to quantify ESES severity.

Non-rapid eye movement (NREM) sleep: The phase of sleep characterised by slow-wave brain activity, during which pathological spike discharges are most prominent.

Developmental and epileptic encephalopathy (DEE): A group of severe epilepsy syndromes in which ongoing epileptiform activity contributes to developmental impairment beyond that expected from the underlying pathology alone.

References

1. Solving the Etiology of Developmental and Epileptic Encephalopathy with Spike–Wave Activation in Sleep (D/EE‐SWAS). Annals of Neurology (2024).
2. Genetic Etiologies in Developmental and/or Epileptic Encephalopathy With Electrical Status Epilepticus During Sleep: Cohort Study. Frontiers in Genetics (2021).
3. Effectiveness of perampanel in the treatment of pediatric patients with focal epilepsy and ESES: A single-center retrospective study. Frontiers in Pharmacology (2022).
4. Sleep and Epilepsy Link by Plasticity. Frontiers in Neurology (2020).
5. Corticosteroids versus clobazam in epileptic encephalopathy with ESES: a European multicentre randomised controlled clinical trial (RESCUE ESES*). Trials (2020).
6. Examining the Utility of Resective Epilepsy Surgery in Children With Electrical Status Epilepticus in Sleep: Long Term Clinical and Electrophysiological Outcomes. Frontiers in Neurology (2020).
7. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia (2022).