EFFICACY AND SAFETY OF LEVETIRACETAM IN CHILDREN WITH ELECTRICAL STATUS EPILEPTICUS DURING SLOW-WAVE SLEEP (ESES)
https://doi.org/10.17749/2077-8333.2017.9.2.021-028
Abstract
Electrical status epilepticus during slow-wave sleep (ESES) is an EEG pattern of continuous (85-100%) diffuse epileptiform activity in the sleep EEG. The morphology of the 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 the phenomenon of continuous spike-waves during slow wave sleep. This group of epilepsies also includes Pseudo-Lennox syndrome, Landau-Kleffner syndrome, autistic epileptiform regression and some others. In most cases, the ESES pattern correlates with the severity of cognitive deficit in this population of epileptic children. The aim of this study was to evaluate the efficacy and safety of levetiracetam in children with the ESES pattern in the EEG. Materials and Methods. During the period of 2010-2016, 34 epileptic children with ESES patterns in the EEG (14 boys and 20 girls) treated with levetiracetam (33 in combined and 1 in monotherapy) were studied. Results. Twenty eight of the patients with ESES patterns in the sleep EEG were diagnosed with the ESES form of epilepsy. Among them, 7 idiopathic, 13 symptomatic, and 8 “symptomatic” cases of ESES (the latter resulted from “double pathology” of idiopathic + hypoxic-ischemic factors and these children had cerebral palsy). Among other ESES patients, 3 cases were of Pseudo-Lennox syndrome, 2 cases of Landau-Kleffner syndrome and one girl with autistic epileptiform regression. All 28 children received levetiracetam at therapeutic doses of 20-80 mg/kg/daily. Levetiracetam was highly effective in 67.6% of patients (n=23); of those, 2 children showed full clinical and electroencephalographic remission before reaching puberty, 9 children had clinical remission and 12 children had a significant decrease in seizures and epileptiform discharges. A low efficacy of levetiracetam was seen in 20.6% (n=7) of patients. Disease aggravation (seizures and epileptiform discharges) was found in 11.8% (n=4) of patients. Other negative effects were observed in only 4 (11.8%) children (3 cases of agitation and sleep disturbance and one case of allergic rash). In conclusion, levetiracetam is a highly effective medication (67.6% of cases) in combined antiepileptic therapy in children with ESES. However, the risk of exacerbation was as high as 11.8%. The most effective combinations of levetiracetam were those with valproates and ethosuximide.
About the Authors
A. A. KholinRussian Federation
MD, PhD, neurologist, neurophysiologist, Professor, the Department of Neurology, Neurosurgery and Medical Genetics, Faculty of Pediatrics, Russian National Research Medical University named after N.I. Pirogov
Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997. Tel.: +7(926)5368725
N. N. Zavadenko
Russian Federation
MD, PhD, neurologist, Professor, Head of the Department of Neurology, Neurosurgery and Medical Genetics, Faculty of Pediatrics, Russian National Research Medical University named after N.I. Pirogov
Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997. Tel.: +7(495)9369452
I. D. Fedonyuk
Russian Federation
MD, senior assistant, the Department of Neurology, Neurosurgery and Medical Genetics, Faculty of Pediatrics, Russian National Research Medical University named after N.I. Pirogov; neurologist in the Department of Psychoneurology № 2, Russian Children’s Clinical Hospital
Address: Leninskiy Prospect, 117, Moscow, Russia, 117997. Tel.: +7(916)3571876
E. S. Il`ina
Russian Federation
MD, senior assistant, the Department of Neurology, Neurosurgery and Medical Genetics, Faculty of Pediatrics, Russian National Research Medical University named after N.I. Pirogov; Head of the Department of Psychoneurology № 2, Russian Children’s Clinical Hospital
Address: Leninskiy Prospect, 117, Moscow, Russia, 117997. Тел: +7(903)1526751
References
1. Schwab R. S. A method of measuring consciousness in Petit Mal epilepsy. J. Nerv. Ment. Dis. 1939; 89: 690-691.
2. Landau W. M., Kleffner F. Syndrome of acquired aphasia with convulsive disorder in children. Neurology. 1957; 7: 523-530.
3. Patry G., Lyagoubi S., Tassinari C. A. Subclinical electrical status epilepticus induced by sleep in children. Arch. Neurol. 1971; 24: 242-252.
4. Zenkov L. R. Non-paroxysmal epileptic disorders [Neparoksizmal’nye epilepticheskie rasstroistva (in Russian)]. Moscow. 2007; 278 s.
5. Karlov V. A. Epilepsy in children and adult women and men. A guide for doctors [Epilepsiya u detei i vzroslykh zhenshchin i muzhchin. Rukovodstvo dlya vrachei (in Russian)]. Moscow. 2010; 719 s.
6. Mukhin K. Yu., Petrukhin A. S., Kholin A. A. Epileptic encephalopathies and similar syndromes in children [Epilepticheskie entsefalopatii i skhozhie sindromy u detei (in Russian)]. Moscow. 2011; 680 s.
7. 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.
8. 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.
9. Aicardi J., Chevrie J.-J. Atypical benign partial epilepsy of childhood. Dev. Med. Child. Neurol. 1982; 24: 281-292.
10. 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.
11. Martнnez Bermejo A., Pascual Castroviejo I., Lуpez Martнn V., Arcas J., Pйrez Higueras A. Acquired aphasia syndrome with epilepsy (Landau-Kleffner syndrome) secondary to cerebral arteritis. 4 cases. Neurologia. 1989; 4 (8): 296-299.
12. Deonna T. Acquired epileptiform aphasia in children (Landau – Kleffner syndrome). J. Clin. Neurophysiol. 1991; 8 (2): 288-298.
13. Beaumanoir A. The Landau – Kleffner syndrome. In: Eds.: J. Roger, M. Bureau, Ch. Dravet i soavt. Epileptic syndromes in infancy, childhood and adolescence. London: John Libbey. 1992; 231-244.
14. Mukhin K. Yu., Kholin A. A., Petrukhin A. S., Glukhova L. Yu., Zaitseva M. N. Zhurnal nevrologii i psikhiatrii im. S. S. Korsakova. 2003; 103 (9): 16-27.
15. Echenne B., Cheminal R., River F. Epileptic electroencephalographic abnormalities and developmental dysphasias: a study of 32 patients. Brain Dev. 1992; 14: 216-225.
16. Fejerman N., Di Blasi A. M. Status epilepticus of benign partial epilepsies in children: report of two cases. Epilepsia. 1987; 28: 351-355.
17. 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.
18. Tuchman R. F., Rapin I. Regression in pervasive developmental disorders: seizures and epileptiform electroencephalogram correlates. Pediatrics. 1997; 99: 560-566.
19. Nass R., Gross A., Devinsky O. Autism and autistic epileptiform regression with occipital spikes. Developmental Medicine & Child Neurology. 1998; 40: 453-458.
20. 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.
21. Kholin A. A. Epilepticheskie entsefalopatii s elektricheskim statusom medlennovolnovogo sna (ESES). Epilepsiya i paroksizmal’nye sostoyaniya. 2016; 8 (1): 62-65.
Review
For citations:
Kholin A.A., Zavadenko N.N., Fedonyuk I.D., Il`ina E.S. EFFICACY AND SAFETY OF LEVETIRACETAM IN CHILDREN WITH ELECTRICAL STATUS EPILEPTICUS DURING SLOW-WAVE SLEEP (ESES). Epilepsy and paroxysmal conditions. 2017;9(2):21-28. (In Russ.) https://doi.org/10.17749/2077-8333.2017.9.2.021-028

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