Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome.
OBJECTIVE
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.


CONCLUSIONS
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.
Liukkonen
Elina
E
Epilepsy Unit, Department of Pediatric Neurology, Helsinki University Central Hospital, HUS, Finland. elina.liukkonen@hus.fi
Kantola-Sorsa
Elisa
E
Paetau
Ritva
R
Gaily
Eija
E
Peltola
Maria
M
Granström
Marja-Liisa
ML
D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis / Q000188:drug therapy; D004359:Drug Therapy, Combination; D004569:Electroencephalography / Q000187:drug effects / Q000706:statistics & numerical data; D019305:Epilepsy, Rolandic / Q000175:diagnosis / Q000188:drug therapy*; D005013:Ethosuximide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis / Q000188:drug therapy*; D008137:Longitudinal Studies; D008297:Male; D011446:Prospective Studies; D012640:Seizures / Q000175:diagnosis / Q000188:drug therapy; D012893:Sleep Wake Disorders / Q000175:diagnosis / Q000188:drug therapy*; D013226:Status Epilepticus / Q000175:diagnosis / Q000188:drug therapy*; D013577:Syndrome; D016896:Treatment Outcome; D014635:Valproic Acid / Q000627:therapeutic use*
D000927:Anticonvulsants; D005013:Ethosuximide; D014635:Valproic Acid