BACKGROUND: The Lennox-Gastaut syndrome is an age-specific disorder, 
characterised by epileptic seizures, a characteristic electroencephalogram 
(EEG), psychomotor delay and behaviour disorders. It occurs more frequently in 
males and onset is usually before the age of eight, with a peak between three 
and five years. Late cases occurring in adolescence and early adulthood have 
rarely been reported. Language is frequently affected, with both slowness in 
ideation and expression in addition to difficulties of motor dysfunction. Severe 
behavioural disorders (for example hyperactivity, aggressiveness and autistic 
tendencies) and personality disorders are nearly always present. There is also a 
tendency for psychosis to develop with time. The long-term prognosis is poor; 
although the epilepsy often improves, complete seizure freedom is rare and 
conversely the mental and psychiatric disorders tend to worsen with time.
OBJECTIVES: To compare the effects of pharmaceutical therapies used to treat 
Lennox-Gastaut syndrome in terms of control of seizures and adverse effects. 
Many people who suffer from this syndrome will already be receiving other 
antiepileptic medications at the time of their entry into a trial. However, for 
the purpose of this review we will only consider the effect of the single 
therapeutic agent being trialed (often as add-on therapy).
SEARCH STRATEGY: We searched the Cochrane Epilepsy Group's Specialized Register 
(February 2009), the Cochrane Central Register of Controlled Trials (CENTRAL) 
(The Cochrane Library Issue 1, 2009), and MEDLINE (1950 to January 2009). We 
also searched EMBASE (1980 to March 2003). We imposed no language restrictions. 
We searched the ISRCTN register for ongoing trials and in addition, we contacted 
pharmaceutical companies and colleagues in the field to seek any unpublished or 
ongoing studies.
SELECTION CRITERIA: All randomised controlled trials (RCTs) of the 
administration of drug therapy to patients with Lennox-Gastaut syndrome.
DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data. 
Analysis included assessing study quality, as well as statistical analysis of 
the effects on overall seizure rates and effects on specific seizure types (e.g. 
drop attacks), adverse effects and mortality.
MAIN RESULTS: We found seven RCTs, but were unable to perform any meta-analysis, 
because each trial looked at different populations, different therapies and 
considered different outcomes.
AUTHORS' CONCLUSIONS: The optimum treatment for Lennox-Gastaut syndrome remains 
uncertain and no study to date has shown any one drug to be highly efficacious; 
rufinamide, lamotrigine, topiramate and felbamate may be helpful as add-on 
therapy. Until further research has been undertaken, clinicians will need to 
continue to consider each patient individually, taking into account the 
potential benefit of each therapy weighed against the risk of adverse effects.