Encephalopathy with continuous spike-waves during slow-wave sleep: evolution and prognosis

Roberto Caraballo, Elena Pavlidis, Marina Nikanorova, Tobias Loddenkemper

Research output: Contribution to journalJournal articleResearchpeer-review

Abstract

Encephalopathy with continuous spike-waves during slow-wave sleep (CSWS) evolves over time, and three stages can be recognized: before the onset of CSWS, during CSWS, and after the CSWS period. Clinical seizures tend to remit spontaneously around puberty. This pattern is independent of the etiological lesion. The CSWS also disappears in all cases. Focal abnormalities instead, may persist for some time after the disappearance of CSWS. The disappearance of the clinical seizures and CSWS may be simultaneous or seizures may disappear before or after disappearance of the CSWS pattern on the EEG. Electroclinical parameters in the pre-CSWS period that have been proposed to predict a poor outcome are early-onset seizures, appearance of new seizures, and a significant increase in seizure frequency. From the electrical point of view, an increase in the frequency of the interictal EEG paroxysms while awake and during sleep and bilateral spike-and-wave paroxysms may also be predictive of a poor evolution in CSWS. When CSWS disappears, neurocognitive and behavioral status improve, but in most patients, residual moderate to severe neurocognitive impairments remain. In non-lesional epilepsy, cognitive recovery after cessation of the CSWS depends on the severity and duration of the initial regression. The duration of the CSWS seems to be the most important predictor of cognitive outcome. Early recognition and effective therapy to reduce the seizures and resolve the CSWS may be crucial to improve long-term prognosis. Cognitive recovery is observed in patients who respond well to AED treatment and outcome depends on the etiology.

Original languageEnglish
JournalEpileptic disorders : international epilepsy journal with videotape
Volume21
Issue numberS1
Pages (from-to)S15-S21
ISSN1294-9361
DOIs
Publication statusPublished - Jun 2019

Keywords

  • continuous spike-wave
  • electrical status epilepticus
  • encephalopathy related to status epilepticus during slow sleep
  • evolution
  • outcome
  • slow sleep

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