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.