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The Epileptic Encephalopathies of Infancy and Childhood

Published online by Cambridge University Press:  02 December 2014

Elaine Wirrell*
Departments of Pediatrics and Clinical Neurosciences, University of Calgary, Calgary, AB, Canada
Kevin Farrell
Department of Pediatrics, University of British Columbia, Vancouver BC, Canada
Sharon Whiting
Department of Pediatrics, University of Ottawa, Ottawa, ON, Canada
Division of Pediatric Neurology, Alberta Children’s Hospital, 1820 Richmond Rd SW, Calgary, AB Canada T2T 5C7
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The epileptic encephalopathies comprise a group of devastating seizure syndromes which begin in infancy and early childhood and usually result in intractable epilepsy. While some syndromes are relatively easily diagnosed early in their course, others take time to evolve, hampering an early, confident diagnosis. Epileptic encephalopathies are associated with slowing of cognitive function and evolution of severe behavioral disorders, which are often more distressing to families than the epilepsy. While an underlying etiology may explain some of this co-morbidity, many children have no identifiable etiology found for their seizures. In these “idiopathic” cases, recurrent subtle seizures, frequent epileptiform discharge and non-convulsive status epilepticus probably all play a role in deterioration of cognitive function and evolution of behavior disorders. This paper will review the most common epileptic encephalopathy syndromes, discuss the cognitive and behavioral co-morbidities and review current therapeutic options.



Les encéphalopathies épileptiques incluent un groupe de syndromes épileptiques dévastateurs qui commencent dans la petite enfance et qui causent une épilepsie réfractaire au traitement. Bien que certains syndromes soient relativement faciles à diagnostiquer tôt au cours de la maladie, d’autres évoluent plus lentement ce qui retarde souvent le moment où un diagnostic ferme est posé. Les encéphalopathies épileptiques sont associées à un ralentissement des fonctions cognitives et au développement de troubles comportementaux sévères qui causent souvent plus de désarroi dans la famille que l’épilepsie. Bien qu’une pathologie sous-jacente peut expliquer en partie cette co-morbidité, on n’identifie chez plusieurs enfants aucune étiologie expliquant leurs crises. Chez ces cas « idiopathiques », des crises subtiles récurrentes, des décharges épileptiformes fréquentes et un état de mal épileptique non convulsif jouent probablement un rôle dans la détérioration de la fonction cognitive et l’évolution des troubles du comportement. Cet article revoit les syndromes encépahlopathiques épileptiques, discute les comorbidités cognitives et comportementales et revoit les options thérapeutiques actuelles.

Review Article
Copyright © The Canadian Journal of Neurological 2005


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The Epileptic Encephalopathies of Infancy and Childhood
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The Epileptic Encephalopathies of Infancy and Childhood
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