Spectrum of epileptic syndromes with electrical status epilepticus during sleep in children

There has been much debate about the nosologic forms of electrical status epilepticus during sleep (ESES) that can occur in a number of syndromes. The pathogenesis of ESES is unknown, and the natural course is variable. It is debatable whether these age-specific epileptic syndromes belong to the same spectrum of disorders with different severity but a common denominator of sleep-related hypersynchronization of generalized paroxysmal epileptic discharges. This report describes 18 children with medically refractory seizures, gradual deterioration in language skills, fine-motor incoordination, behavioral changes, psychologic and intellectual regression of different degrees, and the ESES phenomenon. Most exhibited clinical and electroencephalographic responses to intravenous or oral benzodiazepines, especially if initiated within the first 2 years of seizure onset. Seizure remission was nearly complete with cessation of seizures and marked improvement in language and fine-motor skills, behavior, and intellectual function in those with an idiopathic etiology. Therapeutic trials with benzodiazepines should be given to all children with the ESES phenomenon. Sleep electroencephalographic monitoring is recommended in all young children with epilepsy and language or psychologic deterioration so that the brain dysfunction can be reversed at a critical and vulnerable period of early life.