Epileptic encephalopathies are severe brain disorders of early age that manifest with (1) electrographic EEG paroxysmal activity that is often aggressive, (2) seizures that are usually multiform and intractable, (3) cognitive, behavioral and neurological deficits that may be relentless, and (4) sometimes early death. The concept of “epileptic encephalopathies” is based on the assumption that aggressive ictal (seizure) and electrical (electrographic) epileptogenic activity during brain maturation is the main causative factor of progressive cognitive and neuropsychological deterioration or regression. Conversely, this deleterious epileptic activity is a specific age-related brain reaction of excessive neocortical excitability to different pathological conditions, which are focal or diffuse, of symptomatic or idiopathic cause. This age-related epileptogenic reaction is peculiar to the immature brain and varies significantly in accordance with the stage of brain maturity at the time that this occurs. Thus, EEG demonstrates primarily burst-suppression patterns in the neonatal period, hypsarrhythmia in infancy, and slow generalized spike-wave discharges (GSWDs) in early childhood. With advancing age, the seizure and electrographic epileptogenic features may evolve from one to another age-related stage that is from burst suppression to hypsarrhythmia and then to slow GSWD. All epileptic encephalopathies have a tendency to abate, discontinue, or even stop in adolescence but often with serious neurocognitive residuals.
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