Document Detail


Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology.
MedLine Citation:
PMID:  19081517     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Lennox-Gastaut syndrome is one of the most severe epileptic encephalopathies of childhood onset. The cause of this syndrome can be symptomatic (ie, secondary to an underlying brain disorder) or cryptogenic (ie, has no known cause). Although Lennox-Gastaut syndrome is commonly characterised by a triad of signs, which include multiple seizure types, slow spike-wave complexes on electroencephalographic (EEG) recordings, and impairment of cognitive function, there is debate with regard to the precise limits, cause, and diagnosis of the syndrome. Tonic seizures, which are thought to be a characteristic sign of Lennox-Gastaut syndrome, are not present at onset and the EEG features are not pathognomonic of the disorder. There are few effective treatment options for the multiple seizures and comorbidities, and the long-term outlook is poor for most patients. Probably as a result of the complexity of the disorder, only a few randomised trials have studied Lennox-Gastaut syndrome, and thus many of the drugs that are more commonly used have little or no supporting evidence base from controlled trials. In this Review, we discuss the main issues with regard to the diagnosis and treatment options available. We also suggest key considerations for future trials and highlight the importance of a comprehensive approach to the assessment and management of this syndrome.
Authors:
Alexis Arzimanoglou; Jacqueline French; Warren T Blume; J Helen Cross; Jan-Peter Ernst; Martha Feucht; Pierre Genton; Renzo Guerrini; Gerhard Kluger; John M Pellock; Emilio Perucca; James W Wheless
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Lancet neurology     Volume:  8     ISSN:  1474-4422     ISO Abbreviation:  Lancet Neurol     Publication Date:  2009 Jan 
Date Detail:
Created Date:  2008-12-16     Completed Date:  2009-02-06     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101139309     Medline TA:  Lancet Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  82-93     Citation Subset:  IM    
Affiliation:
Institute for Children and Adolescents with Epilepsy-IDEE, University Hospitals of Lyon and INSERM U821, Lyon, France. alexis.arzimanoglou@chu-lyon.fr
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MeSH Terms
Descriptor/Qualifier:
Adult
Anticonvulsants / adverse effects,  therapeutic use
Child
Diagnosis, Differential
Disease Progression
Electroencephalography
Epilepsies, Myoclonic / etiology
Epilepsy / diagnosis,  therapy*
Epilepsy, Absence / diagnosis
Epilepsy, Generalized / diagnosis
Guidelines as Topic
Humans
Learning Disorders / etiology
Randomized Controlled Trials as Topic
Research Design
Status Epilepticus / diagnosis
Syndrome
Terminology as Topic
Chemical
Reg. No./Substance:
0/Anticonvulsants

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