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

Abstract

Summary Purpose: 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. Discussion: 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.

Encephalopathy with Electrical Status Epilepticus in Slow Wave Sleep – a review with an emphasis on regional (perisylvian) aspects

“…This convention behind the seemingly almost identical acronyms is accepted practice by several authors (Sánchez Fernán-dez et al, 2012, Liukkonen et al, 2010. The same survey showed that appart from nomenclature, oppinions about the course, outcome and diagnostic criteria were also very heterogenous.…”

“…Similarly, Tassinari et al (2012) relies on the secondary bilateral synchrony mechanism which was assumed to work classically (Tükel and Jasper, 1952) by involving secondarily thalamic and thalamo-cortical circuitry. However, asymmetric, unilateral, or more focal/regional discharges were frequently reported (Bureau, 1995;Saltik et al, 2005;Van Hirtum-Das et al, 2006;Kramer et al, 2009;Liukkonen et al, 2010;Sánches Fernández et al, 2012). The sleep discharges in LKS were also found almost always asymmetric, with a dominant hemispheric and regional distribution congruently to the language disturbances.…”

“…Only a few patiens had been followed longitudinally with concurrent description of the evolution in the key EEG and clinical features (Morikawa et al, 1995;Praline et al, 2006;Liukkonen et al, 2010;Sán-chez Fernández, 2012b;Hegyi et al, 2014). Presently we have mainly only cross-sectional data.…”

Landau kleffner syndrome, electrical status epilepticus in sleep and autistic regression: an overview of literature / Landau kleffner sendromu, uykuda elektriksel status epileptikus ve otistik regresyon: Literatüre bir bakış

“…Of these patients, 8 had surgery; five patients had callosotomy, one patient hemispherotomy, one parietooccipital resection, and one multiple subpial transection. The authors of this study suggested that surgical treatment options should be considered early in drug-resistant symptomatic ESES cases (58).…”

“…Among the other conventional AEDs, the single or combined uses of valproic acid and ethosuximide have been reported to be beneficial in some studies. (33,58,59) However, some authors hold the view that the conventional AEDs play only a minimal role and the response of patients is incomplete and transitory (10,54). Response to treatment by short-term, high dose diazepam cycles was reported (32).…”