Epileptic Disord. 2012 Mar;14(1):1-11. doi: 10.1684/epd.2012.0482.
Therapy of encephalopathy with status epilepticus during sleep (ESES/CSWS syndrome): an update.
Abstract
Electrical status epilepticus in sleep (ESES)/continuous spikes and waves during slow sleep (CSWS) is an age-related, self-limiting disorder characterised by epilepsy
with different seizure types, global or selective neuropsychological
regression, motor impairment, and a typical EEG pattern of continuous
epileptiform activity for more than 85% of non-rapid eye movement (NREM)
sleep. Although the first description of ESES/CSWS dates back to 1971, an agreement about the optimal treatment for this condition is still lacking. ESES/CSWS
is rare (incidence is 0.2-0.5% of all childhood epilepsies) and no
controlled clinical trials have been conducted to establish the efficacy
of different antiepileptic drugs; only uncontrolled studies and case
reports are reported in the literature. Treatment options for ESES/CSWS
include some antiepileptic drugs (valproic acid, ethosuximide,
levetiracetam, and benzodiazepines), steroids, immunoglobulins, the
ketogenic diet, and surgery (multiple subpial transections). In this
study, the comparative value of each of these treatments is reviewed and
a personal therapeutic approach is proposed.
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