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Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome.
Epilepsia. 2010 Oct; 51(10):2023-32.E

Abstract

PURPOSE

To prospectively evaluate the efficacy of drug treatment and long-term cognitive outcome in children with encephalopathy with status epilepticus during sleep (ESESS).

METHODS

Thirty-two children were diagnosed and prospectively followed up for at least 3 years at our unit between 1991 and 2007. Twenty-seven children were included in the prospective treatment study with valproate (VPA) and 17 with VPA combined with ethosuximide (ESM). Treatment response of disappearance of electrical status epilepticus during sleep (SES) was documented with overnight EEG recordings. Neuropsychological follow up for at least 5 years was available in 18 patients.

RESULTS

Six children had atypical rolandic (AR) epilepsy, nine Landau-Kleffner syndrome (LKS), and 17 symptomatic epilepsy. Before ESESS, 20 children were cognitively normal. Prospective treatment with VPA and ESM was effective in 3 of the 17 children (18%) treated. Abolition of SES with drug treatment was observed in 16 patients. In all, 10 children (31%), 4 with AR (67%), 3 with LKS (33%), and 3 with symptomatic etiology (19%), including 9 with treatment response regained the pre-ESESS cognitive level. Unfavorable cognitive outcome was predicted by younger age at ESESS diagnosis, lower IQ at the time of the diagnosis, and no response to drug treatment when compared with those with favorable cognitive outcome. Eight of the 16 nonresponders underwent epilepsy surgery.

DISCUSSION

Treatment response with VPA combined with ESM was observed more often than with other drug combinations. Most children with ESESS experienced permanent cognitive impairment. Cognitive outcome depends on treatment response on electroencephalography (EEG) and seizures, and on underlying etiology.

Authors+Show Affiliations

Epilepsy Unit, Department of Pediatric Neurology, Helsinki University Central Hospital, HUS, Finland. elina.liukkonen@hus.fiNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

20477851

Citation

Liukkonen, Elina, et al. "Long-term Outcome of 32 Children With Encephalopathy With Status Epilepticus During Sleep, or ESES Syndrome." Epilepsia, vol. 51, no. 10, 2010, pp. 2023-32.
Liukkonen E, Kantola-Sorsa E, Paetau R, et al. Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia. 2010;51(10):2023-32.
Liukkonen, E., Kantola-Sorsa, E., Paetau, R., Gaily, E., Peltola, M., & Granström, M. L. (2010). Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia, 51(10), 2023-32. https://doi.org/10.1111/j.1528-1167.2010.02578.x
Liukkonen E, et al. Long-term Outcome of 32 Children With Encephalopathy With Status Epilepticus During Sleep, or ESES Syndrome. Epilepsia. 2010;51(10):2023-32. PubMed PMID: 20477851.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. AU - Liukkonen,Elina, AU - Kantola-Sorsa,Elisa, AU - Paetau,Ritva, AU - Gaily,Eija, AU - Peltola,Maria, AU - Granström,Marja-Liisa, PY - 2010/5/19/entrez PY - 2010/5/19/pubmed PY - 2010/12/22/medline SP - 2023 EP - 32 JF - Epilepsia JO - Epilepsia VL - 51 IS - 10 N2 - PURPOSE: To prospectively evaluate the efficacy of drug treatment and long-term cognitive outcome in children with encephalopathy with status epilepticus during sleep (ESESS). METHODS: Thirty-two children were diagnosed and prospectively followed up for at least 3 years at our unit between 1991 and 2007. Twenty-seven children were included in the prospective treatment study with valproate (VPA) and 17 with VPA combined with ethosuximide (ESM). Treatment response of disappearance of electrical status epilepticus during sleep (SES) was documented with overnight EEG recordings. Neuropsychological follow up for at least 5 years was available in 18 patients. RESULTS: Six children had atypical rolandic (AR) epilepsy, nine Landau-Kleffner syndrome (LKS), and 17 symptomatic epilepsy. Before ESESS, 20 children were cognitively normal. Prospective treatment with VPA and ESM was effective in 3 of the 17 children (18%) treated. Abolition of SES with drug treatment was observed in 16 patients. In all, 10 children (31%), 4 with AR (67%), 3 with LKS (33%), and 3 with symptomatic etiology (19%), including 9 with treatment response regained the pre-ESESS cognitive level. Unfavorable cognitive outcome was predicted by younger age at ESESS diagnosis, lower IQ at the time of the diagnosis, and no response to drug treatment when compared with those with favorable cognitive outcome. Eight of the 16 nonresponders underwent epilepsy surgery. DISCUSSION: Treatment response with VPA combined with ESM was observed more often than with other drug combinations. Most children with ESESS experienced permanent cognitive impairment. Cognitive outcome depends on treatment response on electroencephalography (EEG) and seizures, and on underlying etiology. SN - 1528-1167 UR - https://www.unboundmedicine.com/medline/citation/20477851/Long_term_outcome_of_32_children_with_encephalopathy_with_status_epilepticus_during_sleep_or_ESES_syndrome_ L2 - https://doi.org/10.1111/j.1528-1167.2010.02578.x DB - PRIME DP - Unbound Medicine ER -