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

Research output: Contribution to journalArticle

Abstract

Encephalopathy with electrical status epilepticus in sleep (ESES) is defi ned 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 fi nding of CSWS in an 18-month-old male whose previous EEG 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
Volume52
Issue number3
Publication statusPublished - Sep 2012

Fingerprint

Sleep
Electroencephalography
Status Epilepticus
Brain Diseases
Sleep-Wake Transition Disorders
Seizures
Wakefulness
Epilepsy
Newborn Infant

Keywords

  • 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
  • Medicine(all)

Cite this

Evolution of ohtahara syndrome to continuous spikes and waves during slow sleep in an infant. / Poothrikovil, Rajesh P.; Koul, Roshan Lal; Mani, Renjith; Futaisi, Amna Al.

In: Neurodiagnostic Journal, Vol. 52, No. 3, 09.2012, p. 261-274.

Research output: Contribution to journalArticle

@article{209604dc34d94cabbfcefbc77f14afc6,
title = "Evolution of ohtahara syndrome to continuous spikes and waves during slow sleep in an infant",
abstract = "Encephalopathy with electrical status epilepticus in sleep (ESES) is defi ned 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 fi nding of CSWS in an 18-month-old male whose previous EEG 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.",
keywords = "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",
author = "Poothrikovil, {Rajesh P.} and Koul, {Roshan Lal} and Renjith Mani and Futaisi, {Amna Al}",
year = "2012",
month = "9",
language = "English",
volume = "52",
pages = "261--274",
journal = "The Neurodiagnostic journal",
issn = "2164-6821",
publisher = "American Society of Electroneurodiagnostic Tech. Inc.",
number = "3",

}

TY - JOUR

T1 - Evolution of ohtahara syndrome to continuous spikes and waves during slow sleep in an infant

AU - Poothrikovil, Rajesh P.

AU - Koul, Roshan Lal

AU - Mani, Renjith

AU - Futaisi, Amna Al

PY - 2012/9

Y1 - 2012/9

N2 - Encephalopathy with electrical status epilepticus in sleep (ESES) is defi ned 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 fi nding of CSWS in an 18-month-old male whose previous EEG 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.

AB - Encephalopathy with electrical status epilepticus in sleep (ESES) is defi ned 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 fi nding of CSWS in an 18-month-old male whose previous EEG 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.

KW - Burst suppression

KW - Cognitive regression

KW - Continuous spikes and waves in slow sleep (CSWS)

KW - EEG

KW - Electrical status epilepticus in sleep (ESES)

KW - Neonatal seizures

KW - Non-rapid eye movement (NREM) sleep

UR - http://www.scopus.com/inward/record.url?scp=84867852199&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84867852199&partnerID=8YFLogxK

M3 - Article

VL - 52

SP - 261

EP - 274

JO - The Neurodiagnostic journal

JF - The Neurodiagnostic journal

SN - 2164-6821

IS - 3

ER -