Lennox-Gastaut syndrome

a consensus approach on diagnosis, assessment, management, and trial methodology

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 Wheless

Research output: Contribution to journalReview article

237 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)82-93
Number of pages12
JournalThe Lancet Neurology
Volume8
Issue number1
DOIs
StatePublished - Jan 1 2009

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Consensus
Seizures
Brain Diseases
Cognition
Comorbidity
Lennox Gastaut Syndrome
Therapeutics
Pharmaceutical Preparations

All Science Journal Classification (ASJC) codes

  • Clinical Neurology

Cite this

Lennox-Gastaut syndrome : a consensus approach on diagnosis, assessment, management, and trial methodology. / Arzimanoglou, Alexis; French, Jacqueline; Blume, Warren T.; Cross, J. Helen; Ernst, Jan Peter; Feucht, Martha; Genton, Pierre; Guerrini, Renzo; Kluger, Gerhard; Pellock, John M.; Perucca, Emilio; Wheless, James.

In: The Lancet Neurology, Vol. 8, No. 1, 01.01.2009, p. 82-93.

Research output: Contribution to journalReview article

Arzimanoglou, A, French, J, Blume, WT, Cross, JH, Ernst, JP, Feucht, M, Genton, P, Guerrini, R, Kluger, G, Pellock, JM, Perucca, E & Wheless, J 2009, 'Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology', The Lancet Neurology, vol. 8, no. 1, pp. 82-93. https://doi.org/10.1016/S1474-4422(08)70292-8
Arzimanoglou, Alexis ; French, Jacqueline ; Blume, Warren T. ; Cross, J. Helen ; Ernst, Jan Peter ; Feucht, Martha ; Genton, Pierre ; Guerrini, Renzo ; Kluger, Gerhard ; Pellock, John M. ; Perucca, Emilio ; Wheless, James. / Lennox-Gastaut syndrome : a consensus approach on diagnosis, assessment, management, and trial methodology. In: The Lancet Neurology. 2009 ; Vol. 8, No. 1. pp. 82-93.
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