Spike-Wave Index Assessment and Electro-Clinical Correlation in Patients with Encephalopathy Associated with Epileptic State During Slow Sleep (ESES / CSWS); Single-Center Experience

•Status epilepticus during sleep (ESES/CSWS) syndrome is an epileptic encephalopathy affects the development and cognitive functioning of children.•ESES/CSWS, has a wide range of SWI, patients with SWI> 50% should also be followed up with neuropsychological evaluations.•Etiology seems to have the...

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Published in:Epilepsy research 2021-02, Vol.170, p.106549-106549, Article 106549
Main Authors: Öztoprak, Ülkühan, Yayici Köken, Özlem, Aksoy, Erhan, Yüksel, Deniz
Format: Article
Language:English
Subjects:
Brain Diseases
Child
Continuous spikes and waves during slowsleep (CSWS)
Electrical status epilepticus in sleep (ESES)
Electroclinical assessment
Epilepsy
Female
Humans
Infant
Male
Neurocognitive outcome
Retrospective Studies
Sleep
Spike-wave index
Status Epilepticus
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Yayici Köken, Özlem
Aksoy, Erhan
Yüksel, Deniz
description •Status epilepticus during sleep (ESES/CSWS) syndrome is an epileptic encephalopathy affects the development and cognitive functioning of children.•ESES/CSWS, has a wide range of SWI, patients with SWI> 50% should also be followed up with neuropsychological evaluations.•Etiology seems to have the major impact on long-term neurocognitive outcome.•Age of onset, ESES duration, and spike-wave index also appears to be the main determinants of neurocognitive outcome. This study aimed to describe the electroclinical spectrum and neurocognitive outcome in children with epileptic encephalopathy with status epilepticus during sleep (ESES) according to the EEG patterns. Records of 48 (19 males, 29 females) patients with ESES/CSWS syndrome were retrospectively evaluated for data on sleep and awake EEGs, psychometric tests, and brain MRI. Patients with a spike-wave index (SWI) of at least 50 % in the NREM sleep EEG were included in the study. Electrophysiologic findings were separated into two groups based on SWI: SWI>85-100 % (typical ESES) and SWI 
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This study aimed to describe the electroclinical spectrum and neurocognitive outcome in children with epileptic encephalopathy with status epilepticus during sleep (ESES) according to the EEG patterns. Records of 48 (19 males, 29 females) patients with ESES/CSWS syndrome were retrospectively evaluated for data on sleep and awake EEGs, psychometric tests, and brain MRI. Patients with a spike-wave index (SWI) of at least 50 % in the NREM sleep EEG were included in the study. Electrophysiologic findings were separated into two groups based on SWI: SWI&gt;85-100 % (typical ESES) and SWI &lt; 85 % (atypical ESES). The neurocognitive prognosis was also evaluated in two groups; favorable and unfavorable. The median age at the onset of ESES was 6 years and 5 months and ranged from 3 to 13 years. The median duration of follow-up after the ESES diagnosis was 57 months (range 24–150 months). Etiology was evaluated in three groups: symptomatic/structural, idiopathic, and unknown (cryptogenic). Twenty-seven (56.25 %) patients had atypical ESES patterns and 21 patients (43.75 %) had typical ESES patterns. Twenty-eight patients (58.3 %) had cognitive deterioration. Long term neurocognitive outcome was unfavorable in half of the patients. Symptomatic/structural etiology was more common in patients with unfavorable (p &lt; 0.001) outcomes. The median age at the diagnosis of ESES (p &lt; 0.001) was significantly earlier in the patients with unfavorable neurocognitive outcomes. The longer duration of ESES(p &lt; 0.001), and the longer time between the onset of epilepsy and ESES (p = 0.039) was significantly associated with unfavorable outcomes. We found that patients with typical ESES had a higher risk for poor neurocognitive outcomes than patients with atypical ESES (OR: 31.096 [1.565–617.696]). The long-term outcome of ESES is exceedingly variable. 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This study aimed to describe the electroclinical spectrum and neurocognitive outcome in children with epileptic encephalopathy with status epilepticus during sleep (ESES) according to the EEG patterns. Records of 48 (19 males, 29 females) patients with ESES/CSWS syndrome were retrospectively evaluated for data on sleep and awake EEGs, psychometric tests, and brain MRI. Patients with a spike-wave index (SWI) of at least 50 % in the NREM sleep EEG were included in the study. Electrophysiologic findings were separated into two groups based on SWI: SWI&gt;85-100 % (typical ESES) and SWI &lt; 85 % (atypical ESES). The neurocognitive prognosis was also evaluated in two groups; favorable and unfavorable. The median age at the onset of ESES was 6 years and 5 months and ranged from 3 to 13 years. The median duration of follow-up after the ESES diagnosis was 57 months (range 24–150 months). Etiology was evaluated in three groups: symptomatic/structural, idiopathic, and unknown (cryptogenic). Twenty-seven (56.25 %) patients had atypical ESES patterns and 21 patients (43.75 %) had typical ESES patterns. Twenty-eight patients (58.3 %) had cognitive deterioration. Long term neurocognitive outcome was unfavorable in half of the patients. Symptomatic/structural etiology was more common in patients with unfavorable (p &lt; 0.001) outcomes. The median age at the diagnosis of ESES (p &lt; 0.001) was significantly earlier in the patients with unfavorable neurocognitive outcomes. The longer duration of ESES(p &lt; 0.001), and the longer time between the onset of epilepsy and ESES (p = 0.039) was significantly associated with unfavorable outcomes. We found that patients with typical ESES had a higher risk for poor neurocognitive outcomes than patients with atypical ESES (OR: 31.096 [1.565–617.696]). The long-term outcome of ESES is exceedingly variable. 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source ScienceDirect Journals
subjects Brain Diseases
Child
Continuous spikes and waves during slowsleep (CSWS)
Electrical status epilepticus in sleep (ESES)
Electroclinical assessment
Epilepsy
Female
Humans
Infant
Male
Neurocognitive outcome
Retrospective Studies
Sleep
Spike-wave index
Status Epilepticus
title Spike-Wave Index Assessment and Electro-Clinical Correlation in Patients with Encephalopathy Associated with Epileptic State During Slow Sleep (ESES / CSWS); Single-Center Experience
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