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Electrical status epilepticus of slow sleep (ESES) is a sleep EEG pattern with a continuous (85-100%) diffuse epileptiform activity. The morphology of these epileptiform complexes is identical to benign epileptiform discharges of childhood (BEDC). Epilepsy with ESES (or Penelope Syndrome) is a form of age-dependent epileptic encephalopathies with continuous spike-wave activity during slow wave sleep. This group of epilepsies also includes Pseudo-Lennox syndrome, Landau-Kleffner syndrome, autistic epileptiform regression and some others. Usually, the ESES pattern correlates with the severity of cognitive deficit in epileptic children. The list of anti-epileptic drugs (AEDs) used in pharmacotherapy of epileptic encephalopathies includes valproates, levetiracetam, ethosuximide, sulthiame, topiramate, zonisamide and benzodiazepines; those are given in monotherapy or rationalized combined therapy. Levetiracetam was highly effective in 67.6% of patients with ESES (n=23); among them, two cases of clinical and EEG remission during the pre-puberty period, nine cases of clinical remission and 12 cases with a significant decrease in seizures and epileptiform discharges. In 20.6% of patients (n=7), the pharmacotherapy showed low efficacy. In 11.8% of patients (n=4), aggravation of the disease was noted. Combinations of levetiracetam with valproates and levetiracetam with ethosuximide were found the most effective AEDs. In the cases of epileptogenic structural defect that underlines drug-resistant epilepsy with ESES, neurosurgical intervention is recommended.

About the Author

A. A. Kholin
N. I. Pirogov Russian National Research Medical University, Ministry of Health of Russia; Russian Children Clinical Hospital, Ministry of Health of Russia
Russian Federation

MD, PhD, Neurologist, Neurophysiologist, Professor at the Department of Neurology, Neurosurgery and Medical Genetics, Faculty of Pediatrics,

1 Ostrovityanova Str., Moscow 117997;

117 Leninskii prospekt, Moscow 117513


1. Engel J.R., International League Against Epilepsy (ILAE). A Proposed Diagnostic Schema for People with Epileptic Seizures and with Epilepsy. Epilepsia. 2001; 42 (6): 796-803.

2. Scheffer I. E., Berkovic S., Capovilla G., Connolly M.B., French J., Guilhoto L., Hirsch E., Jain S., Mathern G.W., Moshé S. L., Nordli D.R., Perucca E., Tomson T., Wiebe S., Zhang Y.H., Zuberi S.M. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017; 58 (4): 512-521. DOI: 10.1111/epi.13709.

3. Fisher R.S., Cross J.H., French J.A., Higurashi N., Hirsch E., Jansen F. E., Lagae L., Moshé S. L., Peltola J., Roulet Perez E., Scheffer I. E., Zuberi S.M. Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017; 58 (4): 522-530. DOI: 10.1111/epi.13670.

4. Tassinari C.A., Cantalupo G., Rios-Pohl L., Giustina E.D., Rubboli G. Encephalopathy with status epilepticus during slow sleep: “the Penelope syndrome”. Epilepsia. 2009; 50 (7): 4-8.

5. Muhin K. Ju., Petruhin A.S., Kholin A.A. Epileptic encephalopathies and similar syndromes in children [Jepilepticheskie jencefalopatii i shozhie sindromy u detej (in Russian)]. Moscow. 2011; 680 s.

6. Schwab R.S. A method of measuring consciousness in Petit Mal epilepsy. J. Nerv. Ment. Dis. 1939; 89: 690-691.

7. Landau W.M., Kleffner F. Syndrome of acquired aphasia with convulsive disorder in children. Neurology. 1957; 7: 523-530.

8. Patry G., Lyagoubi S., Tassinari C.A. Subclinical electrical status epilepticus induced by sleep in children. Arch. Neurol. 1971; 24: 242-252.

9. Zenkov L.R. Non-paroxysmal epileptic disorders [Neparoksizmal’nye jepilepticheskie rasstrojstva (in Russian)]. Moscow. 2007; 278 s.

10. Karlov V.A. Epilepsy in children and adult women and men. A guide for doctors [Jepilepsija u detej i vzroslyh zhenshhin i muzhchin. Rukovodstvo dlja vrachej (in Russian)]. Moscow. 2010; 719 s.

11. Doose H., Baier W.K. Benign partial epilepsy and related conditions: multifactorial pathogenesis with hereditary impairment of brain maturation. Eur. J. Pediatr. 1989; 149:152-158.

12. Aicardi J., Chevrie J.-J. Atypical benign partial epilepsy of childhood. Dev. Med. Child. Neurol. 1982; 24: 281-292.

13. Deonna T., Ziegler H. L., Despland P.A. Combined myoclonic – astatic and “benign” focal epilepsy of childhood (“atypical benign partial epilepsy of childhood”). A separate syndrome. Neuropediatrics. 1986; 17: 144-151.

14. Fejerman N., Caraballo R., Tenembaum S. Atypical evolutions of benign localization – related epilepsies in children: are they predictable? Epilepsia. 2000; 41 (4): 380-390.

15. Martínez Bermejo A., Pascual Castroviejo I., López Martín V., Arcas J., Pérez Higueras A. Acquired aphasia syndrome with epilepsy (Landau-Kleffner syndrome) secondary to cerebral arteritis. 4 cases. Neurologia. 1989; 4 (8): 296-299.

16. Deonna T. Acquired epileptiform aphasia in children (Landau – Kleffner syndrome). J. Clin. Neurophysiol. 1991; 8 (2): 288-298.

17. Beaumanoir A. The Landau – Kleffner syndrome. In: Eds.: J. Roger, M. Bureau, Ch. Dravet i soavt. Epileptic syndromes in infancy, childhood and adolescence. London. 1992; 231-244.

18. Muhin K. Ju., Kholin A.A., Petruhin A.S., Gluhova L. Ju., Zajceva M.N. Zhurnal nevrologii i psihiatrii im. S.S. Korsakova (in Russian). 2003; 103 (9): 16-27.

19. Echenne B., Cheminal R., River F. Epileptic electroencephalographic abnormalities and developmental dysphasias: a study of 32 patients. Brain Dev. 1992; 14: 216-225.

20. Scheffer I., Jones L., Pozzebon M. i soavt. Autosomal dominant rolandic epilepsy and speech dyspraxia: a new syndrome with anticipation. Ann. Neurol. 1995; 38 (4): 633-642.

21. Tuchman R. F., Rapin I. Regression in pervasive developmental disorders: seizures and epileptiform electroencephalogram correlates. Pediatrics. 1997; 99: 560-566.

22. Nass R., Gross A., Devinsky O. Autism and autistic epileptiform regression with occipital spikes. Developmental Medicine & Child Neurology. 1998; 40: 453-458.

23. Roulet-Perez E.R., Davidoff V., Despland P.A., Deonna T. Mental and behavioral deterioration of children with epilepsy and CSWS: acquired epileptic frontal syndrome. Dev. Med. Child Neurol. 1993; 35: 661-674.

24. Guerrini R., Belmonte A., Genton P. Antiepileptic drugs-induced worsening of seizures in children. Epilepsia. 1998; 39 (3): 2-10.

25. Kholin A.A. Epilepsiya i paroksizmal’nye sostoyaniya / Epilepsy and paroxysmal conditions. (in Russian). 2016; 8 (1): 62-65. DOI: 10.17749/2077-8333.2016.8.1.062-065.

26. Kholin A.A., Zavadenko N.N., Il’ina E.S., Fedonjuk I.D., Kolpakchi L.M., Halilov V.S., Kosjakova E.S. Zhurnal nevrologii i psihiatrii im. S.S. Korsakova. 2013; 113 (4-2): 45-51.

27. Kholin A.A., Zavadenko N.N., Fedonjuk I.D., Il’ina E.S. Epilepsiya i paroksizmal’nye sostoyaniya / Epilepsy and paroxysmal conditions. (in Russian). 2017; 9 (2): 21-28. DOI: 10.17749/2077-8333.2017.9.2.021-028.

28. Kholin A.A., Zavadenko N.N., Il’ina E.S., Kolpakchi L.M., Fedonjuk I.D., Esipova E.S. Zhurnal nevrologii i psihiatrii im. S.S. Korsakova. 2017; 117 (9): 37-42. DOI: 10.17116/jnevro20171179137-42.

Supplementary files

For citation: Kholin A.A. EPILEPTIC ENCEPHALOPATHIES WITH ELECTRICAL STATUS EPILEPTICUS OF SLOW-WAVE SLEEP (ESES): DIAGNOSIS AND PHARMACOTHERAPY. Epilepsia and paroxyzmal conditions. 2018;10(1):63-71. https://doi.org/10.17749/2077-8333.2018.10.1.063-071

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