Treatment of electrical status epilepticus in sleep: A pooled analysis of 575 cases.
OBJECTIVE
Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is a pediatric epilepsy syndrome with sleep-induced epileptic discharges and acquired impairment of cognition or behavior. Treatment of ESES is assumed to improve cognitive outcome. The aim of this study is to create an overview of the current evidence for different treatment regimens in children with ESES syndrome.


METHODS
A literature search using PubMed and Embase was performed. Articles were selected that contain original treatment data of patients with ESES syndrome. Authors were contacted for additional information. Individual patient data were collected, coded, and analyzed using logistic regression analysis. The three predefined main outcome measures were improvement in cognitive function, electroencephalography (EEG) pattern, and any improvement (cognition or EEG).


RESULTS
The literature search yielded 1,766 articles. After applying inclusion and exclusion criteria, 112 articles and 950 treatments in 575 patients could be analyzed. Antiepileptic drugs (AEDs, n = 495) were associated with improvement (i.e., cognition or EEG) in 49% of patients, benzodiazepines (n = 171) in 68%, and steroids (n = 166) in 81%. Surgery (n = 62) resulted in improvement in 90% of patients. In a subgroup analysis of patients who were consecutively reported (585 treatments in 282 patients), we found improvement in a smaller proportion treated with AEDs (34%), benzodiazepines (59%), and steroids (75%), whereas the improvement percentage after surgery was preserved (93%). Possible predictors of improved outcome were treatment category, normal development before ESES onset, and the absence of structural abnormalities.


CONCLUSIONS
Although most included studies were small and retrospective and their heterogeneity allowed analysis of only qualitative outcome data, this pooled analysis suggests superior efficacy of steroids and surgery in encephalopathy with ESES.
Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.
Bart
B
van den Munckhof
Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.
Violet
V
van Dee
Pediatric Neurology Unit, Dana Children's Hospital, Tel Aviv Medical Center, Tel Aviv, Israel.
Liora
L
Sagi
Neurology Service, Pediatric Hospital "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
Roberto H
RH
Caraballo
Department of Brain and Behavioral Sciences, Child Neuropsychiatry Unit, Casimiro Mondino National Neurological Institute, University of Pavia, Pavia, Italy.
Pierangelo
P
Veggiotti
Epilepsy Unit, Department of Pediatric Neurology, Helsinki University Central Hospital, Helsinki, Finland.
Elina
E
Liukkonen
Epilepsy Center, Cleveland Clinic, Cleveland, Ohio, U.S.A.|Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts, U.S.A.
Tobias
T
Loddenkemper
Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts, U.S.A.
Iván
I
Sánchez Fernández
Department of Pediatric Neurology, Erasme Hospital, Université libre de Bruxelles, Brussels, Belgium.
Marga
M
Buzatu
Department of Pediatric Neurosurgery, Ophthalmological Foundation A. Rothschild, Paris, France.|Inserm U1129, Paris, France.|University Paris Descartes, Paris, France.|University of Sorbonne Paris City, Paris, France.|CEA, Gif sur Yvette, France.
Christine
C
Bulteau
Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.
Kees P J
KP
Braun
Department of Pediatric Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.
Floor E
FE
Jansen
D000927:Anticonvulsants / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D012890:Sleep / Q000187:drug effects; D012893:Sleep Wake Disorders / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology*; D013226:Status Epilepticus / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology*; D016896:Treatment Outcome
D000927:Anticonvulsants
Continuous spikes and waves during sleep; Epilepsy; Landau-Kleffner syndrome; Meta-analysis; Systematic review