| Idiopathic West Syndrome followed by childhood absence epilepsy. | |
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MedLine Citation:
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PMID: 20729098 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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West Syndrome (WS) is a severe epileptic encephalopathy occurring in the first year of life. According the ILAE classification of epileptic seizures and epilepsy the etiology could be symptomatic or cryptogenic. Some authors identified a small group of patients (5%) with a particular good outcome, a complete recovery from seizures and a normal cognitive development within the cryptogenic group that they suggested to be idiopathic. Between 1996 and 2007, at the Neurology Division of the Bambino Gesù Children's Hospital in Rome, we collected 241 patients with WS. Sixteen (6.6%) were considered with idiopathic aetiology. All clinical notes of these patients were reviewed in order to evaluate the prevalence of other epileptic syndrome after WS. Two of them had at the age of 8 and 3 months idiopathic WS, and at the age of 6 and 4 years respectively, they presented with childhood absence epilepsy (CAE) successfully treated with valproate. The favorable evolution of the WS and the later occurrence of an idiopathic form of epilepsy, such as CAE, confirm the possibility of an idiopathic aetiology for WS that, although rare, can represent one of the etiologies of otherwise severe syndrome. Even if a common physiophatogenetic role, probably related to a genetic predisposition, could be hypothesized and appears to be intriguing, no data are available and more studies are needed to confirm this hypothesis. |
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Authors:
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Nicola Specchio; Marina Trivisano; Federico Vigevano; Lucia Fusco |
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Publication Detail:
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Type: Case Reports; Journal Article Date: 2010-08-21 |
Journal Detail:
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Title: Seizure : the journal of the British Epilepsy Association Volume: 19 ISSN: 1532-2688 ISO Abbreviation: Seizure Publication Date: 2010 Nov |
Date Detail:
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Created Date: 2010-10-18 Completed Date: 2011-01-28 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9306979 Medline TA: Seizure Country: England |
Other Details:
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Languages: eng Pagination: 597-601 Citation Subset: IM |
Copyright Information:
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Copyright © 2010 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved. |
Affiliation:
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Division of Neurology, Bambino Gesù Children's Hospital IRCCS, Pza S Onofrio, 4 00165 Rome, Italy. nicola.specchio@opbg.net |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Age of Onset Brain / physiopathology Child Child, Preschool Electroencephalography Epilepsy, Absence / etiology*, physiopathology Female Humans Infant Male Spasms, Infantile / complications*, physiopathology |
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