Continuous spike and wave during slow wave sleep (CSWS) is an epileptic encephalopathy that presents with neurocognitive regression and clinical seizures, and that demonstrates an electroencephalogram (EEG) pattern of electrical status epilepticus during sleep, as defined by the Commission on Classification and Terminology of the International League Against Epilepsy 1989. CSWS is an age-related condition, typically presenting in children around 5 years of age, with clinical seizures which progress within 2 years to a severe epileptic encephalopathy. The pathophysiology of CSWS is not completely understood, but the corticothalamic neuronal network involved in sleep patterns is thought to be involved. Genetic predisposition and injury in early development are thought to play etiological roles. Treatment strategies have involved traditional anticonvulsants, hormonal therapies, and other newer techniques. Outcomes are fair, and the thought is that earlier diagnosis and intervention preserve neurocognitive development, as in the case of other epileptic encephalopathies. Further understanding of the mechanisms of CSWS may lead to improved therapeutic options and thus outcomes of children with CSWS. 1. Introduction Electrical status epilepticus during sleep was first described in 1971 as EEG abnormalities that became continuous with sleep onset and ceased to be continuous upon arousal, occupying at least 85% of slow sleep tracing [1]. These children did not demonstrate overt clinical manifestations; thus, the condition was described as “subclinical” or “electrical.” In this initial study, the authors described this condition as a form of encephalopathy given cognitive impairment in all children [1], and as more patients were described with this condition, it became clear that neurocognitive deterioration occurred at the same time as the electrical status epilepticus during slow wave sleep developed [2]. Subsequently, the International League Against Epilepsy (ILAE) opted to term the condition “continuous spike and waves during sleep” (CSWS) as a specific epilepsy syndrome characterized by continuous diffuse spike-waves occurring during slow-wave sleep, seen after the onset of seizures, that has a potentially poor outcome despite a noncatastrophic onset of seizures, related to the development of neurocognitive deficits (Commission on Classification and Terminology of the International League Against Epilepsy, 1989). Electrical status epilepticus during sleep (ESES) is now the term typically used to describe solely the electrographic findings, while the CSWS
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