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Treatment of electrical status epilepticus in sleep: Clinical and EEG characteristics and response to 147 treatments in 47 patients.
Eur J Paediatr Neurol. 2018 Jan; 22(1):64-71.EJ

Abstract

OBJECTIVE

Electrical status epilepticus in sleep (ESES) syndrome is characterized by near-continuous sleep-induced epileptiform activity and acquired cognitive deficits. Treatment is assumed mandatory to improve cognitive outcome. We aimed to compare EEG characteristics, subjective evaluation and objective neuropsychological assessment as measures to evaluate treatment efficacy, and to analyze possible predictors.

METHODS

We retrospectively included patients with ESES syndrome treated in our center. Treatment effect was analyzed on sleep EEG spike wave index (SWI) and cognitive functioning.

RESULTS

47 patients had 147 (43 steroid and 104 non-steroid) treatments. Cognitive improvement was reported after 36% of treatments at first follow-up and 45% of treatments at last follow-up. The median SWI change for treatments resulting in subjective cognitive improvement was -44%, and 0% for those not resulting in subjective cognitive improvement at first follow-up (p = 0.008) and -50% vs. +5% at last follow-up (p = 0.002). No clear association between subjective cognitive improvement and IQ change, and between SWI and IQ change was found. By means of logistic regression we found that steroid treatment, as compared to non-steroid treatment, was associated with cognitive improvement at first follow-up (multivariate OR after multiple imputation 2.5, 95% CI 1.1-5.7), while at last follow-up, higher age at diagnosis was related to cognitive improvement only in univariate analysis (OR 1.02, 95% CI 1.01-1.04).

CONCLUSIONS

We found that in children with ESES, cognitive improvement after treatment was strongly associated with SWI decrease, while it was not reflected by a significant IQ increase. Steroid treatment was most successful in improving cognitive performance.

Authors+Show Affiliations

Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands. Electronic address: B.vandenMunckhof@umcutrecht.nl.Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Pediatric Neuropsychology, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Pediatric Neuropsychology, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Biostatistics and Research Support, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29128194

Citation

van den Munckhof, Bart, et al. "Treatment of Electrical Status Epilepticus in Sleep: Clinical and EEG Characteristics and Response to 147 Treatments in 47 Patients." European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society, vol. 22, no. 1, 2018, pp. 64-71.
van den Munckhof B, Alderweireld C, Davelaar S, et al. Treatment of electrical status epilepticus in sleep: Clinical and EEG characteristics and response to 147 treatments in 47 patients. Eur J Paediatr Neurol. 2018;22(1):64-71.
van den Munckhof, B., Alderweireld, C., Davelaar, S., van Teeseling, H. C., Nikolakopoulos, S., Braun, K. P. J., & Jansen, F. E. (2018). Treatment of electrical status epilepticus in sleep: Clinical and EEG characteristics and response to 147 treatments in 47 patients. European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society, 22(1), 64-71. https://doi.org/10.1016/j.ejpn.2017.08.006
van den Munckhof B, et al. Treatment of Electrical Status Epilepticus in Sleep: Clinical and EEG Characteristics and Response to 147 Treatments in 47 Patients. Eur J Paediatr Neurol. 2018;22(1):64-71. PubMed PMID: 29128194.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Treatment of electrical status epilepticus in sleep: Clinical and EEG characteristics and response to 147 treatments in 47 patients. AU - van den Munckhof,Bart, AU - Alderweireld,Christian, AU - Davelaar,Susanne, AU - van Teeseling,Heleen C, AU - Nikolakopoulos,Stavros, AU - Braun,Kees P J, AU - Jansen,Floor E, Y1 - 2017/10/26/ PY - 2016/11/17/received PY - 2017/08/11/revised PY - 2017/08/26/accepted PY - 2017/11/13/pubmed PY - 2018/6/29/medline PY - 2017/11/13/entrez KW - CSWS KW - EEG KW - ESES KW - Electrical status epilepticus in sleep KW - LKS KW - Landau–Kleffner syndrome SP - 64 EP - 71 JF - European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society JO - Eur. J. Paediatr. Neurol. VL - 22 IS - 1 N2 - OBJECTIVE: Electrical status epilepticus in sleep (ESES) syndrome is characterized by near-continuous sleep-induced epileptiform activity and acquired cognitive deficits. Treatment is assumed mandatory to improve cognitive outcome. We aimed to compare EEG characteristics, subjective evaluation and objective neuropsychological assessment as measures to evaluate treatment efficacy, and to analyze possible predictors. METHODS: We retrospectively included patients with ESES syndrome treated in our center. Treatment effect was analyzed on sleep EEG spike wave index (SWI) and cognitive functioning. RESULTS: 47 patients had 147 (43 steroid and 104 non-steroid) treatments. Cognitive improvement was reported after 36% of treatments at first follow-up and 45% of treatments at last follow-up. The median SWI change for treatments resulting in subjective cognitive improvement was -44%, and 0% for those not resulting in subjective cognitive improvement at first follow-up (p = 0.008) and -50% vs. +5% at last follow-up (p = 0.002). No clear association between subjective cognitive improvement and IQ change, and between SWI and IQ change was found. By means of logistic regression we found that steroid treatment, as compared to non-steroid treatment, was associated with cognitive improvement at first follow-up (multivariate OR after multiple imputation 2.5, 95% CI 1.1-5.7), while at last follow-up, higher age at diagnosis was related to cognitive improvement only in univariate analysis (OR 1.02, 95% CI 1.01-1.04). CONCLUSIONS: We found that in children with ESES, cognitive improvement after treatment was strongly associated with SWI decrease, while it was not reflected by a significant IQ increase. Steroid treatment was most successful in improving cognitive performance. SN - 1532-2130 UR - https://www.unboundmedicine.com/medline/citation/29128194/Treatment_of_electrical_status_epilepticus_in_sleep:_Clinical_and_EEG_characteristics_and_response_to_147_treatments_in_47_patients_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1090-3798(16)30223-9 DB - PRIME DP - Unbound Medicine ER -