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Surgical management of pediatric patients with encephalopathy due to electrical status epilepticus during sleep (ESES) Volume 22, numéro 1, February 2020

TEST YOURSELF

(1) What is ESES, and what is its clinical relevance in epilepsy and neuropsychology?

 

(2) What are potential treatment options for ESES?

 

(3) In children who undergo resective or disconnective surgery for ESES, what are the clinical variables leading to favorable post-operative outcome?

 

 

 

 

 

 

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Answers

(1) Electrical status epilepticus during sleep (ESES), also known as continuous spike-wave discharges during slow-wave sleep (CSWSS), is an age-dependent developmental epileptic disorder. The onset of ESES is associated with both seizures and a progressive encephalopathy characterized by developmental regression, neurobehavioral disorder and deficits in language, social skills, memory, global intellect and motor function.

 

(2) Anti-epileptic drugs (AEDs), benzodiazepines, and high-dose corticosteroids are considered the first-line treatments, although a high proportion of cases remain medically refractory. More aggressive approaches include intravenous gamma globulins and the ketogenic diet. Surgery is a promising therapeutic option for pharmaco-resistant ESES, but is infrequently employed due to potential spontaneous ESES regression, and concerns about surgical complications. Multiple subpial transections (MSTs) have been performed on patients with language-eloquent ESES in Landau Kleffner syndrome (LKS), but the results are inconsistent. Focal resection and hemispherectomy are performed more rarely and yield variable results. Since the natural history of ESES and the predictors of surgical success remain unclear, there is clinical equipoise on the optimal approach for children with this condition. This paper describes a cohort of 11 children with seizures and ESES, who underwent resective or disconnective surgical intervention, following failure of medical management and progressive neuropsychological decline.

 

(3) In the pediatric population who underwent resective or disconnective surgery (focal cortical resection or hemispherectomy) for ESES in our large tertiary center, children with mild or moderate neurodevelopmental delay, focal epileptogenic foci, and hemi-ESES electrographic findings displayed more favorable seizure and neuropsychological outcome. In this cohort, surgery is a safe and feasible management option for selective patients, yielding results that are more favorable than those reported in the literature for medical therapy.

 

 

 

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