Childhood epileptic encephalopathy with diffuse slow spike and waves
Lennox-Gastaut syndrome (LGS) is characterized as a severe epileptic encephalopathy with generalized seizures of multiple types that emerge in children between the ages of 1 and 8 years and occur with high frequency. EEG studies typically reveal an abnormal background with slow spike-and-wave activity that occurs at less than 2.5 Hz frequency. Prognosis is poor, and intellectual disability is traditionally considered a defining feature.
Approximately 1–4% of childhood epilepsy cases are believed to meet the criteria for LGS. This accounts for about 10% of epilepsy syndromes that begin during the first 5 years of life. The prevalence rate in Western countries ranges from 0.1 to 0.28 per 1000, and about 2 in every 100,000 children receive a diagnosis of LGS each year. Representation among children with intellectual disability is higher and is estimated to be about 7%. Among...
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