Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology.
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.
Arzimanoglou
Alexis
A
Institute for Children and Adolescents with Epilepsy-IDEE, University Hospitals of Lyon and INSERM U821, Lyon, France. alexis.arzimanoglou@chu-lyon.fr
French
Jacqueline
J
Blume
Warren T
WT
Cross
J Helen
JH
Ernst
Jan-Peter
JP
Feucht
Martha
M
Genton
Pierre
P
Guerrini
Renzo
R
Kluger
Gerhard
G
Pellock
John M
JM
Perucca
Emilio
E
Wheless
James W
JW
D000328:Adult; D000927:Anticonvulsants / Q000009:adverse effects / Q000627:therapeutic use; D002648:Child; D003937:Diagnosis, Differential; D018450:Disease Progression; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000209:etiology; D004827:Epilepsy / Q000175:diagnosis / Q000628:therapy*; D004832:Epilepsy, Absence / Q000175:diagnosis; D004829:Epilepsy, Generalized / Q000175:diagnosis; D017408:Guidelines as Topic; D006801:Humans; D007859:Learning Disabilities / Q000209:etiology; D016032:Randomized Controlled Trials as Topic; D012107:Research Design; D013226:Status Epilepticus / Q000175:diagnosis; D013577:Syndrome; D009626:Terminology as Topic
D000927:Anticonvulsants