Epileptic Encephalopathies of Infancy and Childhood

Abstract

The term “epileptic encephalopathies” has been recently revised, restricting the criterion of inclusion to the forms in which the epilepsy itself is responsible of brain dysfunctions associated with mental and neurologic decline. This chapter describes the clinical and EEG features of the main epileptic encephalopathies that begin in infancy and childhood (West syndrome, Dravet syndrome, Lennox-Gastaut syndrome, Doose syndrome, progressive myoclonic epilepsies, Landau-Kleffner syndrome, electrical status epilepticus during slow sleep). Incidence and prevalence of these epilepsies are generally low. The electro-clinical feature is influenced by the brain maturation; some syndromes are typically age-dependent and show a clear continuity with evolution from one type to another. The etiology is heterogeneous, often consisting of structural brain damage, although the role of genetic factors has recently been emphasized in some forms. The prognosis is variable, depending on clinical finding and underlying etiology, but it is generally unfavorable both for seizure outcome and neurocognitive development..