Epileptic encephalopathies: an overview.

Sonia Khan,Raidah Al Baradie

doi:10.1155/2012/403592

Abstract

Epileptic encephalopathies are an epileptic condition characterized by epileptiform abnormalities associated with progressive cerebral dysfunction. In the classification of the International League Against Epilepsy eight age-related epileptic encephalopathy syndromes are recognized. These syndromes include early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, West syndrome and Dravet syndrome in infancy, myoclonic status in nonprogressive encephalopathies, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sleep in childhood and adolescences. Other epileptic syndromes such as migrating partial seizures in infancy and severe epilepsy with multiple independent spike foci may be reasonably added. In this paper, we provide an overview of epileptic encephalopathies including clinical neurophysiological features, cognitive deterioration, and management options especially that these conditions are generally refractory to standard antiepileptic drugs.

Highlights

Epileptic encephalopathy is defined as a condition in which epileptiform abnormalities are believed to contribute to the progressive disturbance in cerebral function, but this definition may be ambiguous [1]
A common feature is that these disorders are usually refractory to standard antiepileptic drugs (AEDs) [3]
More aggressive use of AEDs considered effective in suppressing interictal epileptiform discharges, immunomodulatory therapies (e.g., corticosteroids, intravenous immunoglobulin (IVIG), and plasmapheresis), ketogenic diet, and surgical options is often considered [3]

Summary

Introduction

Epileptic encephalopathy is defined as a condition in which epileptiform abnormalities are believed to contribute to the progressive disturbance in cerebral function, but this definition may be ambiguous [1]. The report of the International League Against Epilepsy (ILAE) Task Force on classification and terminology includes 8 syndromes under epileptic encephalopathies: early myoclonic encephalopathy, Ohtahara syndrome, West syndrome, Dravet syndrome, myoclonic status in nonprogressive encephalopathies, Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow-wave sleep [1]. To these syndromes, the migrating partial seizures in infancy and severe epilepsy with multiple independent spike foci may be reasonably added [2]. Epileptic encephalopathies will be dealt with in the following concept: a particular group of usually agerelated and extremely intractable epilepsies with characteristic generalized minor seizures and massive epileptic EEG abnormalities, both of which cause stagnation or deterioration in mental and cognitive functions in addition to the preexisting developmental deficit due to organic brain damage [1,2,3]

Epileptic Encephalopathy Syndromes in Infancy

Epileptic Encephalopathy Syndromes in Childhood

Findings

Conclusions