A risk-benefit assessment of therapies for Lennox-Gastaut syndrome

The treatment of Lennox-Gastaut syndrome has been improved for some patients by the introduction of adjunctive therapy with newer anticonvulsants such as lamotrigine and topiramate and the availability of vagal nerve stimulation and the re-emergence of the use of the ketogenic diet in recent years. The place of standard anticonvulsants and the role of callosotomy needs to be re-evaluated in view of the new developments. Although recommendations for the treatment of patients with Lennox-Gastaut syndrome are difficult to make in the absence of direct head-on comparative trials, the following suggested treatment recommendations are based on the best evidence available. Medical treatment should start with valproic acid (sodium valproate) and be followed by adjunctive therapy with either lamotrigine or topiramate; clobazam can be added if necessary for better seizure control while trying to reduce the dose of the other anticonvulsants. If standard treatment does not achieve sufficient seizure control or proves to be intolerable, vagal nerve stimulation, ketogenic diet, felbamate, benzodiazepines such as clonazepam, and phenobarbital (phenobarbitone) are recommended as third-line choices. Further considerations include ethosuximide, methsuximide, corticotropin (adrenocorticotropic hormone) or corticosteroids, pyridoxine (vitamin B6) and vigabatrin. If adequate drug treatment and vagal nerve stimulation provide insufficient seizure control, partial callosotomy may be an option for the treatment of frequent, intractable and disabling drop attacks. These suggestions are based on the best evidence available and do not in any way exclude the use of other treatments if compelling individual risk-benefit considerations apply.