Epilepsy Research and Treatment

Epileptic Encephalopathy


Publishing date
03 Aug 2012
Status
Published
Submission deadline
16 Mar 2012

Guest Editors

1Division of Neurology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy

2Leeds General Infirmary, Leeds LS1 3ES, UK

3Great Ormond Street Hospital, London WC1N 1LE, UK


Epileptic Encephalopathy

Description

The relationship between epilepsy and cognitive/behavioral impairments is complex and not completely understood. There is, however, a number of childhood onset epilepsy syndromes in which cognitive/behavioral impairments become apparent at the time that epileptic seizures begin, a phenomenon known as epileptic encephalopathy. Suggested mechanisms underlying epileptic encephalopathy include the effect of very frequent seizures, subclinical paroxysmal epileptic activity, the underlying disease, and other unknown processes or combinations of these. This assumes that epileptic encephalopathy is a pathophysiological process. However, the term has also been used to categorize conditions in which neurological deterioration, cognitive and/or behavioral impairment, and refractory epilepsy occur together. The ILAE Task Force on classification and terminology has therefore defined epileptic encephalopathies as clinical conditions in which the epileptiform EEG abnormalities may contribute to a progressive disturbance in cerebral functions. The following conditions have been included: 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.

However, epilepsies associated with malformation of cortical development (e.g., focal cortical dysplasia), benign tumors (e.g., DNETs), and genetic epilepsies (i.e., CDKL5, SCN1A, STX1B, PCDH19, and ring chromosome 20) may manifest with encephalopathy associated with epilepsy. Knowledge and understanding of these conditions have led to the development of animal models, successful therapies, and novel tools to better characterize the clinical course.

We therefore invite authors to contribute original research as well as review articles that will stimulate the efforts to understand the pathology underlying epileptic encephalopathy, the development of strategies for the treatment, and the evaluation of outcomes. We are interested in articles describing new modalities for clinical characterization of epileptic encephalopathies; advances in molecular genetics; new insights using animal models; current concepts in the treatment of epileptic encephalopathies. Potential topics include, but are not limited to:

  • Clinical characterization of new epileptic encephalopaties
  • Evidences for a revision of the current classification
  • EEG and other neurophysiological findings in humans and in animal models
  • Outcome and prognosis
  • Molecular genetics
  • New genetic conditions
  • Surgical treatment
  • New therapeutic approaches
  • Neuropsychological findings

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