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 - 2012/9/1
Y1 - 2012/9/1
N2 - 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.
AB - 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.
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
U2 - 10.1080/21646821.2012.11079861
DO - 10.1080/21646821.2012.11079861
M3 - Article
C2 - 23019763
AN - SCOPUS:84867852199
SN - 2164-6821
VL - 52
SP - 261
EP - 274
JO - Neurodiagnostic Journal
JF - Neurodiagnostic Journal
IS - 3
ER -