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Eur J Paediatr Neurol. 2018 Jan;22(1):64-71. doi: 10.1016/j.ejpn.2017.08.006. Epub 2017 Oct 26.

Treatment of electrical status epilepticus in sleep: Clinical and EEG characteristics and response to 147 treatments in 47 patients.

Author information

1
Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands. Electronic address: B.vandenMunckhof@umcutrecht.nl.
2
Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.
3
Department of Pediatric Neuropsychology, University Medical Center Utrecht, Utrecht, The Netherlands.
4
Department of Biostatistics and Research Support, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands.

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.

KEYWORDS:

CSWS; EEG; ESES; Electrical status epilepticus in sleep; LKS; Landau–Kleffner syndrome

PMID:
29128194
DOI:
10.1016/j.ejpn.2017.08.006
[Indexed for MEDLINE]

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