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Lennox-Gastaut Syndrome

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Synonyms

Childhood epileptic encephalopathy with diffuse slow spike and waves

Definition

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.

Epidemiology

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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References

  • Autry, A. R., Trevathan, E., Van Naarden Braun, K., & Yeargin-Allsopp, N. (2010). Increased risk of death among children with Lennox-Gastaut syndrome and infantile spasms. Journal of Child Neurology, 25, 441–447.

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  • Morita, D. A., & Glauser, T. A. (2017). Lennox-Gastaut syndrome. In J. M. Pellock, D. R. Nordli Jr., R. Sankar, & J. W. Wheless (Eds.), Pellock’s pediatric epilepsy: Diagnosis and therapy (4th ed., pp. 451–466). New York: Demos Medical.

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  • Oguni, H., Hayashi, K., & Osawa, M. (1996). Long-term prognosis of Lennox-Gastaut syndrome. Epilepsia, 37(Suppl. 3), 44–47.

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  • Yagi, K. (1996). Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study. Epilepsia, 37(Suppl 3), 48–51.

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Correspondence to Jeffrey B. Titus .

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Titus, J.B., Schraegle, W.A., Clarke, D. (2018). Lennox-Gastaut Syndrome. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_1563

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