Abstract
Encephalopathy associated with electrical status epilepticus of sleep (ESES) is an underdiagnosed epileptic encephalopathy syndrome. Though there is considerable controversy regarding the terminology of this condition, there is increasing acceptance of the term, ‘encephalopathy associated with electrical status epilepticus of sleep,’ as it implies a clinical component as well as EEG feature. Core EEG feature of sleep activation of epileptiform discharges temporarily associated with cognitive or behavioral regression in a child with epilepsy is sufficient to make a diagnosis of ESES. It is important to quantify the spike wave index (SWI) during awake state and sleep to assess for sleep activation. Since focal spikes are also included in the counting of SWI, some grading to assess the distribution of epileptiform discharges is suggested to obtain the complete sleep EEG picture. MRI of the brain does not show abnormality in a number of patients. But common structural lesions include perinatal vascular insults and cortical malformations. Response to treatment is usually variable, but highest success rate is reported with the steroids. Prognosis usually depends on the duration of ESES.
Similar content being viewed by others
References
Engel J. Report of the ILAE classification core group. Epilepsia. 2006;47:1558–68.
Berg AT, Berkovic SF, Brodie MJ, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE commission on classification and terminology, 2005-2009. Epilepsia. 2010;51:676–85.
Hirsch E, Caraballo R, Bernardina BD, Loddenkemper T, Zuberi SM. Encephalopathy related to status epilepticus during slow sleep: from concepts to terminology. Epileptic Disord. 2019;21:5–12.
Jansen FE, Nikanorova M, Peltola M. Current treatment options for encephalopathy related to status epilepticus during slow sleep. Epileptic Disord. 2019;21:76–81.
Patry G, Lyagoubi S, Tassinari CA. Subclinical “electrical status epilepticus” induced by sleep in children. A clinical and electroencephalographic study of six cases. Arch Neurol. 1971;24:242–52.
Landau WM, Kleffner FR. Syndrome of acquired aphasia with convulsive disorder in children. Neurology. 1957;7:523–30.
Proposal for classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1985;26:268–78.
Nickels K, Wirrell E. Electrical status epilepticus in sleep. Semin Pediatr Neurol. 2008;15:50–60.
Gardella E, Cantalupo G, Larsson PG, et al. EEG features in encephalopathy related to status epilepticus during slow sleep. Epileptic Disord. 2019;21:22–30.
Loddenkemper T, Fernandez IS, Peters JM. Continuous spike and waves during sleep and electrical status epilepticus in sleep. J Clin Neurophysiol. 2011;28:154–64.
Tassinari CA, Cantalupo G, Rios-Pohl L, Giustina ED, Rubboli G. Encephalopathy with status epilepticus during slow sleep: “the Penelope syndrome”. Epilepsia. 2009;50:4–8.
Hughes JR. A review of the relationships between Landau-Kleffner syndrome, electrical status epilepticus during sleep, and continuous spike-waves during sleep. Epilepsy Behav. 2011;20:247–53.
Arzimanoglou A, Cross HJ. Cognitive impairment and behavioral disorders in encephalopathy related to status epilepticus during slow sleep: diagnostic assessment and outcome. Epileptic Disord. 2019;21:71–5.
Tassinari CA, Rubboli G, Volpi L, et al. Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia. Clin Neurophysiol. 2000;111:S94–102.
Bureau M. Outstanding cases of CSWS and LKS: analysis of the data sheets provided by the participants. In: Beaumanoir A, Bureau M, Deonna T, editors. Continuous spikes and waves during slow sleep. Electrical status epilepticus during slow sleep: acquired epileptic aphasia and related conditions. London: John Libbey; 1995. p. 213–6.
Morikawa T, Seino M, Watanabe M. Long-term outcome of CSWS syndrome. In: Beaumanoir A, Bureau M, Deonna T, editors. Continuous spikes and waves during slow sleep. Electrical status epilepticus during slow sleep: acquired epileptic aphasia and related conditions. London: John Libbey; 1995. p. 27–36.
Atkins M, Nikanorova M. A prospective study of levetiracetam efficacy in epileptic syndromes with continuous spikes-waves during slow sleep. Seizure. 2011;20:635–9.
Liukkonen E, Kantola-Sorsa E, Paetau R, Gaily E, Peltola M, Granström ML. Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia. 2010;51:2023–32.
Peltola ME, Liukkonen E, Granström ML, et al. The effect of surgery in encephalopathy with electrical status epilepticus during sleep. Epilepsia. 2011;52:602–9.
Scheltens-de BM. Guidelines for EEG in encephalopathy related to ESES/CSWS in children. Epilepsia. 2009;50:13–7.
Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1989;30:389–99.
Binnie CD. Cognitive impairment during epileptiform discharges: is it ever justifiable to treat the EEG? Lancet Neurol. 2003;2:725–30.
Pavlidis E, Rubboli G, Nikanorova M, Kölmel MS, Gardella E. Encephalopathy with status epilepticus during sleep (ESES) induced by oxcarbazepine in idiopathic focal epilepsy in childhood. Funct Neurol. 2015;30:139–41.
Losito E, Battaglia D, Chieffo D, et al. Sleep-potentiated epileptiform activity in early thalamic injuries: study in a large series (60 cases). Epilepsy Res. 2015;109:90–9.
Lesca G, Møller RS, Rudolf G, Hirsch E, Hjalgrim H, Szepetowski P. Update on the genetics of the epilepsy-aphasia spectrum and role of GRIN2A mutations. Epileptic Disord. 2019;21:41–7.
Uliel-Sibony S, Kramer U. Benign childhood epilepsy with centro-temporal spikes (BCECTSs), electrical status epilepticus in sleep (ESES), and academic decline--how aggressive should we be? Epilepsy Behav. 2015;44:117–20.
Vinayan KP, Biji V, Thomas SV. Educational problems with underlying neuropsychological impairment are common in children with benign epilepsy of childhood with centrotemporal spikes (BECTS). Seizure. 2005;14:207–12.
Kramer U, Sagi L, Goldberg-Stern H, Zelnik N, Nissenkorn A, Bruria B-ZB. Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES). Epilepsia. 2009;50:1517–24.
Kelley SA, Kossoff EH. How effective is the ketogenic diet for electrical status epilepticus of sleep? Epilepsy Res. 2016;127:339–43.
van den Munckhof B, van Dee V, Sagi L, et al. Treatment of electrical status epilepticus in sleep: a pooled analysis of 575 cases. Epilepsia. 2015;56:1738–46.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of Interest
None.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
RamachandranNair, R. Encephalopathy Associated with Electrical Status Epilepticus of Sleep (ESES): A Practical Approach. Indian J Pediatr 87, 1057–1061 (2020). https://doi.org/10.1007/s12098-020-03422-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12098-020-03422-9