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Lennox-Gastaut Syndrome: Current Treatments, Novel Therapeutics, and Future Directions
Julie A Nelson1, Kelly G Knupp2
1Section of Child Neurology, Children's Hospital Colorado, Anschutz School of Medicine, University of Colorado, Aurora, CO, USA. Julie.nelson@childrenscolorado.org.
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.
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