Lennox-Gastaut Syndrome: Current Treatments, Novel Therapeutics, and Future Directions
- PMID: 37353676
- PMCID: PMC10480363
- DOI: 10.1007/s13311-023-01397-x
Lennox-Gastaut Syndrome: Current Treatments, Novel Therapeutics, and Future Directions
Abstract
Lennox-Gastaut syndrome is a severe drug-resistant developmental and epileptic encephalopathy with slow spike and wave on EEG (DEE-SSW) composing about 1-2% of epilepsy patients. Seizures in DEE-SSW are caused by a variety of etiologies, and there is a large unmet treatment need as seizures are usually treatment-resistant and individuals are often unable to function independently. The updated definition by the International League Against Epilepsy has established formal diagnostic criteria allowing for more uniform diagnosis. This article provides a review of typical medication management and treatment strategies, including new and developing surgical approaches. Future directions in treatment include expanding genetic testing with the potential for gene therapy and continuously improving surgical options with the goal to prevent progression to DEE-SSW.
Keywords: Anti-seizure medication; Developmental and epileptic encephalopathy; Lennox-Gastaut syndrome; Novel therapeutics; Review.
© 2023. The American Society for Experimental Neurotherapeutics, Inc.
Conflict of interest statement
None.
References
-
- Lennox Gastaut Syndrome. Available at: https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=2382
-
- Gastaut H, Roger J, Soulayrol R, Tassinari CA, Régis H, Dravet C, Bernard R, Pinsard N, Saint-Jean M. Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise known as “petit mal variant”) or Lennox syndrome. Epilepsia. 1966;7(2):139–179. doi: 10.1111/j.1528-1167.1966.tb06263.x. - DOI - PubMed
-
- Specchio N, Wirrell EC, Scheffer IE, Nabbout R, Riney K, Samia P, Guerreiro M, Gwer S, Zuberi SM, Wilmshurst JM, Yozawitz E, Pressler R, Hirsch E, Wiebe S, Cross HJ, Perucca E, Moshé SL, Tinuper P, Auvin S. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022;63(6):1398–1442. doi: 10.1111/epi.17241. - DOI - PubMed
-
- Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1989 Jul-Aug;30(4):389–99. 10.1111/j.1528-1157.1989.tb05316.x. PMID: 2502382. - PubMed
-
- French J, Smith M, Faught E, Brown L. Practice advisory: the use of felbamate in the treatment of patients with intractable epilepsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the American Epilepsy Society. Neurology. 1999;52(8):1540–1545. doi: 10.1212/wnl.52.8.1540. - DOI - PubMed
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