Document Detail

Epileptic encephalopathies in infants and children.
MedLine Citation:
PMID:  23027099     Owner:  NLM     Status:  In-Data-Review    
SUMMARY:: The term epileptic encephalopathy is used to describe diffuse brain dysfunction that is caused, at least in part, by some aspect of epilepsy. Early-infantile epileptic encephalopathy (EIEE), West syndrome, late infantile epileptic encephalopathy, and Lennox-Gastaut are four epilepsy syndromes. These epilepsies are also among the most severe with dire consequences including intractable seizures and severe cognitive dysfunction. These epilepsies share several important characteristics: diverse causes; severe and frequent seizures; diffusely abnormal background activity on electroencephalograms that is often profound; medical intractability; and severe consequences for a normal development. Ohtahara proposed that these epilepsies exist on an electroclinical spectrum and that the clinical and electroencephalogram features are dependent on the maturation of the nervous system. One can now add Late Infantile Epileptogenic Encephalopathy (LIEE) or epilepsy with late-onset of epileptic spasms. Recently, similar gene mutations have been found in several different epilepsy syndromes, reinforcing the notion that these epilepsies are not likely to be distinguished based on cause alone. Recognition and accurate classification of these severe epilepsies is important as the first step toward improving treatment and outcomes.
Douglas R Nordli
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society     Volume:  29     ISSN:  1537-1603     ISO Abbreviation:  J Clin Neurophysiol     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-02     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8506708     Medline TA:  J Clin Neurophysiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  420-4     Citation Subset:  IM    
*Departments of Pediatrics †Neurology, Children's Memorial Hospital, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, U.S.A.
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