Epilepsy Clinical Trial
Official title:
Management Strategies for Electrical Status Epilepticus During Sleep
Electrical status epilepticus during slow wave sleep is a condition in which the epileptic patient starts to develop neurocognitive deterioration, any type of seizures and continuous electrical activity in the EEG during non rapid eye movement sleep. It is an age related condition and will resolve spontaneously at around the age of puberty. However if left untreated or treatment is delayed, it may lead to permanent neurocognitive deterioration. Thus early diagnosis and treatment is essential in these children to preserve neurocognitive function.
Electrical status epilepticus in sleep (ESES), also known as continuous spikes and waves
during slow sleep (CSWS), is an age related, self - limiting disorder characterized by:
1. epilepsy with different seizure types,
2. neuropsychological regression, and
3. typical EEG pattern of continuous epileptiform activity during non-rapid eye movement
sleep (NREM).
During wakefulness, the EEG shows focal/multifocal spikes that increase in frequency during
the acute stage. During sleep, ESES appears and is characterized by (1) marked potentiation
of epileptiform discharges during non-REM sleep, leading to (2) a (near)-continuous,
bilateral, or occasionally lateralized slow spikes and waves, (3) and these spikes and waves
occur "during a significant proportion" of the non-REM sleep with a threshold ranging from
25% to 85%
The exact incidence of CSWS is not known. In pediatric neurology clinics, a reported
frequency of 0.2% of childhood epilepsies most probably underestimates the incidence of CSWS.
CSWS is a devastating syndrome, which requires prompt treatment, the efficacy of which is
checked with regular intervals, e.g. three or six months. Although epilepsy resolves with
time in most cases, many children are left with significant cognitive or language impairment.
Longer duration of ESES appears to be the major predictor of poor outcome.
So, early recognition and effective therapy are necessary to improve long-term prognosis in
this condition.
The goal of treatment is not only to control clinical seizures but also to improve
neuropsychological functions and prevent potential cognitive deterioration. There was no
agreement on best treatment, but potential candidates included high-dose benzodiazepines,
valproate, levetiracetam, and corticosteroids.
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