Evolution of ohtahara syndrome to continuous spikes and waves during slow sleep in an infant

Rajesh P. Poothrikovil*, Roshan Lal Koul, Renjith Mani, Amna Al Futaisi

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)


Encephalopathy with electrical status epilepticus in sleep (ESES) is defined as an age-related and self-limited electroclinical syndrome whose etiology is unknown and characterized by continuous spikes and waves during slow sleep (CSWS). Typical CSWS starts at an age of 4 to 5 years and ends by an average of 11 years. We report on an unexpected finding of CSWS in an 18-month-old male whose previous EEC at age 45 days was grossly abnormal with the presence of a burst suppression pattern during wakefulness and sleep. The patient had clinical seizures beginning day 1 of life leading to the suspicion of Ohtahara syndrome (the earliest form of epileptic encephalopathy). Patients with ESES usually have normal EEGs and functional development prior to the onset of CSWS pattern. To our knowledge, this presentation of a neonate with a history of neonatal seizures and developmental delay with an evolution to CSWS has not been described. A sleep record is strongly advised in children with epilepsy (despite the recording of awake EEG abnormalities), especially in patients with behavior or cognitive regression, to rule out the presence of CSWS.

Original languageEnglish
Pages (from-to)261-274
Number of pages14
JournalNeurodiagnostic Journal
Issue number3
Publication statusPublished - Sept 1 2012


  • Burst suppression
  • Cognitive regression
  • Continuous spikes and waves in slow sleep (csws)
  • Eeg
  • Electrical status epilepticus in sleep (eses)
  • Neonatal seizures
  • Non-rapid eye movement (nrem) sleep

ASJC Scopus subject areas

  • Clinical Neurology
  • Medical Laboratory Technology


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