Lennox-Gastaut syndrome

The Lennox-Gastaut syndrome (LGS) affects 1% to 5% of children with epilepsy and is one of the severe age-related generalized epileptic syndromes. The seizures are among the most intractable of all of the childhood epilepsies. Although LGS has well-accepted clinical and electrocncephalographic (EEG) criteria,distinct-ive boundaries are not always precise, particularly in individual cases. Children with LGS often have a history of infantile spasms and differentiation of LGS from other childhood epilepsies can be difficult. Neurodiagnostic evaluation can be complex and expensive. Polytherapy with established antiepileptic drugs (AEDs) has been the mainstay of therapy until the release of felbamate in 1993. Since then, several newer AEDs have been introduced. These newer drugs, along with the recent resurgence of the ketogenic diet and refinements In epilepsy surgery, have expanded the options now available to child neurologists. Many drugs have been reported to be beneficial in these patients; however, there have been few placebo-controlled studies. Any consensus regarding management must acknowledge the serious methodological problems inherent in studying drug efficacy in this complex and heterogeneous group of patients.