ABSTRACT
Defined by Gastaut in 1966, but first recognized in 1939 by Lennox, LennoxGastaut syndrome (LGS) has attracted considerable interest in the last half century. Responsible for less than 5% of childhood epilepsies, this catastrophic epilepsy consists of a typical triad of intractable seizures (tonic axial, atonic, myoclonic, and atypical absence), characteristic EEG abnormalities (bursts of slow spike and wave during the awake state and 10Hz-fast rhythms with bursts of slow polyspikes during sleep state), and cognitive delay. Its disabling course and refractory nature give it an importance out of proportion to its incidence. Its often-cryptogenic nature is a continuing enigma.
