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What is Lennox Gastaut syndrome
Lennox Gastaut syndrome usually begins before age 5 years and is characterized by generalized tonic–axial, atonic, and atypical absence seizures.
Many patients also have myoclonic, partial, and tonic–clonic seizures.
The EEG is characterized by a slow (2 to 2.5 Hz) frontocentral dominant spike and wave pattern, and patients have developmental delay.
The seizures are usually refractory to medication, and status epilepticus is common.
About 60% of patients have a clear underlying cause of encephalopathy (symptomatic or structural/metabolic).
Sources
Arzimanoglou A, French J, Blume WT, et al.: Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol 8:82-93, 2009.