Lennox-Gastaut syndrome is a usually difficult-to-treat form of epilepsy that generally begins in children between two and six years of age, is associated with frequent and diverse seizure types, and results from an etiologically heterogeneous brain injury occurring prenatally, perinatally, or postnatally.
Epidemiology
LGS accounts for approximately 1–2 % of all epilepsies and up to 10 % of childhood epilepsies. Onset usually occurs before the age of 8 years and is rarely later. Boys are affected slightly more often than girls. Due to the low remission rate, the syndrome frequently persists into adulthood[^1].
Pathophysiology
LGS is understood as a secondary network epilepsy. The epileptic phenomena result less from a single focus than from disrupted cortico-subcortical network dynamics. EEG-fMRI imaging studies demonstrate involvement of the association cortex, Thalamus, Basal ganglia, and Brainstem, with persistent disruption of cognitive networks, even independently of acute seizures[^1].