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. 2012:2012:403592.
doi: 10.1155/2012/403592. Epub 2012 Nov 20.

Epileptic encephalopathies: an overview

Sonia Khan  1 , Raidah Al Baradie
Affiliations

Epileptic encephalopathies: an overview

Sonia Khan et al. Epilepsy Res Treat. 2012.

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.

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Figures

Figure 1
Figure 1
EEG is characterized by a “burst-suppression” pattern with bursts of spikes, sharp waves, and slow waves (arrow), which are irregularly intermingled and separated by periods of electrical silence (arrow head).
Figure 2
Figure 2
Hypsarrhythmia, the typical interictal EEG finding, consists of a disorganized pattern with asynchronous, very high amplitude slowing and frequent multifocal spike and sharp wave discharges (arrows).
Figure 3
Figure 3
The ictal EEG in West syndrome typically reveals a generalized slow wave followed by diffuse voltage attenuation (electrodecrement), which may associate with a spasm or be only electrographic without clinical correlate (arrows).
Figure 4
Figure 4
EEG in Lennox-Gastaut syndrome with paroxysms of fast activity and generalized slow spike and wave discharges (1.5–2 Hz) (arrows).
Figure 5
Figure 5
Electrical status epilepticus during slow sleep (ESES), disappearance of spike waves with eye opening (arrows).
Figure 6
Figure 6
Electrical status epilepticus during slow sleep (ESES), continuous spike-wave discharges on the EEG during slow sleep (arrows).
See this image and copyright information in PMC

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