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What’s hidden behind Lennox-Gastaut syndrome?

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A number of epileptologists often diagnose Lennox-Gastaut syndrome, while others almost never do. It is necessary to understand why this is happening, especially since Lennox-Gastaut syndrome, according to literature descriptions, is quite common.

Aim. To systematize available data on Lennox-Gastaut syndrome, which will help to improve the quality of diagnosis and management of patients.

Materials and methods. Qualitative scientific publications from the International Scientific Databases (ISDS), including peer-reviewed journals and monographs, were selected for the review. The plan of the review included a brief description of the syndrome; seizure types; EEG features; diagnosis criteria; treatment.

Results and discussion. None of the seizure types are pathognomonic for Lennox-Gastaut  syndrome. The syndrome is considered to be confirmed if typical seizures (predominantly tonic) with typical interictal patterns without atypical EEG characteristics are reported. A diagnosis of the syndrome is considered probable if there are typical EEG patterns of waking and sleeping, but no tonic seizures are detected. The first choice of the treatment is valproate. Possible alternatives are lamotrigine and topiramate. Rufinamide and zonisamide are available as the second line of antiepileptic drugs. Rufinamide should be used when there is no effect from valproate and lamotrigine. Rufinamide also has antiabsance activity.

Conclusion. “Classic” Lennox-Gasto syndrome is rare. Cases of “probable” Lennox-Gasto syndrome are much more frequent, and not all criteria of its diagnosis are observed. Taken into account the blurring of the diagnosis criteria for Lennox-Gasto syndrome and data on the efficacy of rufinamide in children with another epilepsies, it is possible to use this drug not only for Lennox-Gasto syndrome, but also for other epileptic syndromes, especially those with dropattacks, tonic seizures and atypical absences. 

About the Author

E. D. Belousova
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
Russian Federation

Elena D. Belousova  – MD, PhD (Med. Sciences), Professor, Head of the Department of Psychoneurology and  Epileptology

2 Taldomskaya Str., Moscow 125412


1. Lennox-Gastaut syndrome. In С. P. Panayiotopoulos. A Clinical Guide to Epileptic Syndromes and their Treatment. Revised second edition. Springer. 2010; 287–303.

2. Engel J Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia. 2001; 42: 796–803.

3. Panayiotopoulos C. Epileptic encephalopathies in infancy and early childhood in which the epileptiform abnormalities may contribute to progressive dysfunction. In Panayiotopoulos C. (Ed) The epilepsies: seizures, syndromes and management. Oxfordshire, UK: Bladon Medical Publishing. 2005; 137–206.

4. Mastrangelo M. Lennox-Gastaut Syndrome: A State of the Art Review. Neuropediatrics. 2017 Jun; 48 (3): 143–151. DOI: 10.1055/s-0037-1601324.

5. Autry A. R., Trevathan E., Van Naarden B. K., et al. Increased risk of death among children with Lennox-Gastaut syndrome and infantile spasms. J Child Neurol. 2010; 25: 441–447.

6. Arzimanoglou A., Resnick T. All children who experience epileptic falls do not necessarily have Lennox-Gastaut syndrome… but many do. Epileptic Disord. 2011; 13 (1): 3–13.

7. Riikonen R. Long-term outcome of patients with West syndrome. Brain Dev. 2001; 23: 683–687.

8. Van Rijckevorsel K. Treatment of Lennox-Gastaut syndrome: overview and recent findings. Neuropsychiatr Dis Treat. 2008; 4: 1001–1019.

9. Amrutkar C., Riel-Romero R. M. Lennox Gastaut Syndrome. StatPearls. Treasure Island (FL). 2018–2019 Jan 16.

10. Jahngir M. U., Ahmad M. Q., Jahangir M. Lennox-Gastaut Syndrome: In a Nutshell. Cureus. 2018; 10 (8): e3134. DOI:10.7759/cureus.3134.

11. Bourgeois B. F., Douglass L. M., Sankar R. Lennox-Gastaut syndrome: a consensus approach to differential diagnosis. Epilepsia. 2014 Sep; 55 (4): 4–9. DOI: 10.1111/epi.12567.

12. Dulac O. N’Guyen T the Lennox-Gastaut syndrome. Epilepsia. 1993; 34 (7): 7–17.

13. Camfield P. R. Definition and natural history of Lennox-Gastaut syndrome. Epilepsia. 2011; 52 (5): 3–9.

14. Asadi-Pooya A. A. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci. 2018 Mar; 39 (3): 403-414. DOI: 10.1007/s10072-017-3188-y.

15. Al-Banji M.H., Zahr D. K., Jan M. M. Lennox-Gastaut syndrome. Management update. Neurosciences (Riyadh). 2015 Jul; 20 (3): 207–12.

16. Asadi-Pooya A.A., Sharifzade M. Lennox-Gastaut syndrome in south Iran: electro-clinical manifestations. Seizure. 2012; 21 (10): 760–763.

17. Koutroumanidis M. et al. The role of EEG in the diagnosis and classification of the epilepsy syndromes: a tool for clinical practice by the ILAE Neurophysiology Task Force (Part 2). Epileptic Disorders. 2017; 19 (4): 385–437.

18. Asadi-Pooya A.A., Dlugos D., Skidmore C., Sperling M. R. Atlas of electroencephalography, 3rd edition. Epileptic Disord. 2017; 19 (3): 384.

19. Sagi V., Kim I., Bhatt A. B., Sonmezturk H., Abou-Khalil B.W., Arain A. M. Generalized paroxysmal fast activity in EEG: An unrecognized finding in genetic generalized epilepsy. Epilepsy Behav. 2017 Nov; 76: 101-104. DOI: 10.1016/j.yebeh.2017.08.019.

20. ILAE website [Electronic resource] URL: https://www. Accessed: 20.12.2019.

21. Verrotti A., Striano P., Iapadre G., et al. The pharmacological management of Lennox-Gastaut syndrome and critical literature review. Seizure. 2018 Dec; 63: 17-25. DOI: 10.1016/j.seizure.2018.10.016. Epub 2018 Oct 26.

22. Glauser T. et al. Rufinamide for generalized seizures associated with Lennox-Gastaut syndrome. Neurology. 2008; 70 (21): 1950–1958.


For citations:

Belousova E.D. What’s hidden behind Lennox-Gastaut syndrome? Epilepsy and paroxysmal conditions. 2020;12(1S):S13-S22. (In Russ.)

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