Lennox Gastaut syndrome. Case report and mini-review
DOI:
https://doi.org/10.56294/saludcyt2022170Keywords:
Lennox-Gastaut Syndrome, Epileptic encephalopathy, Antiepileptic therapy, Intellectual disabilityAbstract
Introduction: Lennox-Gastaut syndrome is a form of childhood epilepsy that has a dramatic negative impact on the physical and developmental health of the patient. Being an age-related disorder, it is characterized by seizures, characteristic electroencephalogram, psychomotor retardation and behavioral disturbances. It is more common in males and usually begins before the age of eight years and peaks between the ages of three and five years.
Case presentation: This was a 5-year-old patient who was born prematurely product of which presented sequelae of hypoxia, who around 4 months was diagnosed with epilepsy, for which he received double antiepileptic therapy based on levetiracetam and valproic acid with partial control of his seizures until before the age of 5 years, At this time it was necessary to include a third drug to the usual therapy called clobazam, for which with the adjustment of doses until the age of 8 years he achieved a better control, however the sequelae of infantile cerebral palsy are marked.
Conclusion: It is one of the most difficult syndromes to treat and is often resistant to commonly used antiepileptic drugs. The long-term prognosis is poor; although epilepsy usually improves, complete absence of seizures is rare and, instead, psychological and psychiatric disorders worsen over time
References
1. Cross JH, Auvin S, Falip M, Striano P, Arzimanoglou A. Expert Opinion on the Management of Lennox–Gastaut Syndrome: Treatment Algorithms and Practical Considerations. Frontiers in Neurology 2017;8.
2. Arzimanoglou A, French J, Blume WT, Cross JH, Ernst J-P, Feucht M, et al. Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. The Lancet Neurology 2009;8:82-93. https://doi.org/10.1016/S1474-4422(08)70292-8.
3. Asadi-Pooya AA. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci 2018;39:403-14. https://doi.org/10.1007/s10072-017-3188-y.
4. Brigo F, Jones K, Eltze C, Matricardi S. Anti‐seizure medications for Lennox‐Gastaut syndrome. Cochrane Database of Systematic Reviews 2021. https://doi.org/10.1002/14651858.CD003277.pub4.
5. Devinsky O, Patel AD, Cross JH, Villanueva V, Wirrell EC, Privitera M, et al. Effect of Cannabidiol on Drop Seizures in the Lennox–Gastaut Syndrome. New England Journal of Medicine 2018;378:1888-97. https://doi.org/10.1056/NEJMoa1714631.
6. Archer JS, Warren AEL, Stagnitti MR, Masterton RAJ, Abbott DF, Jackson GD. Lennox-Gastaut syndrome and phenotype: Secondary network epilepsies. Epilepsia 2014;55:1245-54. https://doi.org/10.1111/epi.12682.
7. Camfield PR. Definition and natural history of Lennox-Gastaut syndrome. Epilepsia 2011;52:3-9. https://doi.org/10.1111/j.1528-1167.2011.03177.x.
8. Lund C, Brodtkorb E, Øye A-M, Røsby O, Selmer KK. CHD2 mutations in Lennox–Gastaut syndrome. Epilepsy & Behavior 2014;33:18-21. https://doi.org/10.1016/j.yebeh.2014.02.005.
9. Mastrangelo M. Lennox–Gastaut Syndrome: A State of the Art Review. Neuropediatrics 2017;48:143-51. https://doi.org/10.1055/s-0037-1601324.
10. Blume WT. Pathogenesis of Lennox-Gastaut syndrome: Considerations and hypotheses. Epileptic Disorders 2002;3:183-96.
11. van Rijckevorsel K. Treatment of Lennox-Gastaut syndrome: overview and recent findings. Neuropsychiatr Dis Treat 2008;4:1001-19.
12. Abu Saleh T, Stephen L. Lennox gastaut syndrome, review of the literature and a case report. Head & Face Medicine 2008;4:9. https://doi.org/10.1186/1746-160X-4-9.
13. Bourgeois BFD, Douglass LM, Sankar R. Lennox-Gastaut syndrome: A consensus approach to differential diagnosis. Epilepsia 2014;55:4-9. https://doi.org/10.1111/epi.12567.
14. Markand ON. Lennox-Gastaut Syndrome (Childhood Epileptic Encephalopathy). Journal of Clinical Neurophysiology 2003;20:426.
15. Verrotti A, Striano P, Iapadre G, Zagaroli L, Bonanni P, Coppola G, et al. The pharmacological management of Lennox-Gastaut syndrome and critical literature review. Seizure 2018;63:17-25. https://doi.org/10.1016/j.seizure.2018.10.016.
16. Conry JA, Ng Y-T, Paolicchi JM, Kernitsky L, Mitchell WG, Ritter FJ, et al. Clobazam in the treatment of Lennox-Gastaut syndrome. Epilepsia 2009;50:1158-66. https://doi.org/10.1111/j.1528-1167.2008.01935.x.
17. Schmidt D, Bourgeois B. A Risk-Benefit Assessment of Therapies for Lennox-Gastaut Syndrome. Drug-Safety 2000;22:467-77. https://doi.org/10.2165/00002018-200022060-00005.
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Copyright (c) 2022 Marlon Andrés López García , Tatiana Elizabeth Lara Abril , Lissette Carolina López Lasinquiza , Gabriela del Carmen Vargas Caiza , María Belén Vargas Congo (Author)
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