Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Lennox-Gastaut Syndrome

  • Jeffrey B. TitusEmail author
  • William A. Schraegle
  • Dave Clarke
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_1563

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

  1. 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.PubMedCrossRefGoogle Scholar
  2. Kim, H. J., Kim, H. D., Lee, J. S., Heo, K., Kim, D. S., & Kang, H. C. (2015). Long-term prognosis of patients with Lennox–Gastaut syndrome in recent decades. Epilepsy Research, 110, 10–19.PubMedCrossRefGoogle Scholar
  3. Lemmon, M. E., Terao, N. N., Ng, Y. T., Reisig, W., Rubenstein, J. E., & Kossoff, E. H. (2012). Efficacy of the ketogenic diet in Lennox-Gastaut syndrome: A retrospective review of one institution’s experience and summary of the literature. Developmental Medicine & Child Neurology, 54(5), 464–468.CrossRefGoogle Scholar
  4. 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.Google Scholar
  5. Oguni, H., Hayashi, K., & Osawa, M. (1996). Long-term prognosis of Lennox-Gastaut syndrome. Epilepsia, 37(Suppl. 3), 44–47.PubMedCrossRefGoogle Scholar
  6. Yagi, K. (1996). Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study. Epilepsia, 37(Suppl 3), 48–51.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Jeffrey B. Titus
    • 1
    • 2
    Email author
  • William A. Schraegle
    • 3
    • 4
  • Dave Clarke
    • 1
    • 5
  1. 1.Comprehensive Epilepsy ProgramDell Children’s Medical Center of Central TexasAustinUSA
  2. 2.Department of PsychologyThe University of Texas at AustinAustinUSA
  3. 3.Pediatric NeuropsychologyDell Children’s Medical Center of Central TexasAustinUSA
  4. 4.Department of Educational PsychologyThe University of Texas at AustinAustinUSA
  5. 5.Department of Pediatrics, Dell Medical SchoolThe University of Texas at AustinAustinUSA