Neurol. praxi. 2026;27(4):284-290 | DOI: 10.36290/neu.2026.055

Getting the diagnosis right in adults with childhood-onset epileptic encephalopathies: Lennox-Gastaut and Dravet syndromes-lessons from clinical practice

MUDr. Lucia Šmahovská, MUDr. Jitka Kočvarová, MUDr. Ondřej Strýček, Ph.D., MUDr. Klára Štillová, Ph.D., prof. MUDr. Milan Brázdil, Ph.D., doc. MUDr. Irena Doležalová, Ph.D.
Centrum pro epilepsie Brno, člen ERN-EpiCare, I. neurologická klinika, Fakultní nemocnice u sv. Anny a Lékařské fakulty, Masarykovy univerzity, Brno

Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) belong to the most severe developmental and epileptic encephalopathies, characterized by early onset, a lifelong disease course, and a high degree of drug-resistance. Both syndromes are associated with substantial cognitive, behavioral, and functional impairment, as well as an increased risk of serious complications. In recent years, syndrome-specific therapeutic options have expanded considerably. For both DS and LGS, fenfluramine and cannabidiol have become available as targeted treatment options, demonstrating remarkable efficacy in randomized clinical trials. The aim of this review is to summarize the key clinical characteristics of both syndromes and to briefly present our initial clinical experience with fenfluramine therapy.

Keywords: developmental and epileptic encephalopathies, Dravet syndrome, Lennox-Gastaut syndrome, fenfluramine, adult patients.

Received: July 8, 2026; Revised: September 7, 2026; Accepted: September 23, 2026; Published: October 2, 2026  Show citation

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Šmahovská L, Kočvarová J, Strýček O, Štillová K, Brázdil M, Doležalová I. Getting the diagnosis right in adults with childhood-onset epileptic encephalopathies: Lennox-Gastaut and Dravet syndromes-lessons from clinical practice. Neurol. praxi. 2026;27(4):284-290. doi: 10.36290/neu.2026.055.
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    References

    1. Auvin S, Arzimanoglou A, Falip M, et al. Refining management strategies for Lennox-Gastaut syndrome: Updated algorithms and practical approaches. Epilepsia Open. 2025;10(1):85-106. doi:10.1002/epi4.13075. Go to original source... Go to PubMed...
    2. Asadi-Pooya AA. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci. 2018;39(3):403-14. doi:10.1007/s10072-017-3188-y. Go to original source... Go to PubMed...
    3. Bishop KI, Isquith PK, Gioia GA, et al. Improved everyday executive functioning following profound reduction in seizure frequency with fenfluramine: Analysis from a phase 3 long-term extension study in children and young adults with Dra-vet syndrome. Epilepsy & Behavior. 2021;121:108024. doi: 10.1016/j.yebeh.2021.108024. Go to original source... Go to PubMed...
    4. Bonn-Miller MO, Loflin MJE, Thomas BF, et al. Labeling Accuracy of Cannabidi-ol Extracts Sold Online. JAMA. 2017;318(17):1708. doi: 10.1001/jama.2017.11909. Go to original source...
    5. Cardenal-Muñoz E, Auvin S, Villanueva V, et al. Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe. Epilepsia Open. 2022;7(1):11-26. doi: 10.1002/epi4.12569. Go to original source... Go to PubMed...
    6. Darra F, Battaglia D, Dravet C, et al. Dravet syndrome: Early electroclinical fin-dings and long-term outcome in adolescents and adults. Epilepsia. 2019;60(S3). doi: 10.1111/epi.16297. Go to original source... Go to PubMed...
    7. Devinsky O, Cross JH, Laux L, et al. Trial of Cannabidiol for Drug-Resistant Sei-zures in the Dravet Syndrome. N Engl J Med. 2017;376(21):2011-20. doi: 10.1056/NEJMoa1611618. Go to original source... Go to PubMed...
    8. Doležalová I, Brázdil M. Dravetové syndrom v dospělosti. Neurol. praxi. 2024;25(3):216-23. doi: 10.36290/neu.2024.043. Go to original source...
    9. Doležalová I, Brázdil M. Lennoxův-Gastautův syndrom v dospělosti. Neurol. praxi. 2025;26(5):415-24. doi: 10.36290/neu.2025.069. Go to original source...
    10. Ferlazzo E, Nikaronova M, Italiano D, et al. Lennox-Gastaut syndrome in adult-hood: Clinical and EEG features. Epilepsy Research. 2010;89(2-3):271-7. doi: 10.1016/j.eplepsyres.2010.01.012. Go to original source...
    11. Guerrini R, Specchio N, Aledo-Serrano Á, et al. An examination of the efficacy and safety of fenfluramine in adults, children, and adolescents with Dravet syn-drome in a REAL-WORLD practice setting: A report from the Fenfluramine European Early Access Program. Epilepsia Open. 2022;7(4):578-87. doi: 10.1002/epi4.12624. Go to original source... Go to PubMed...
    12. Horák O. Fenfluramin. Neurol. praxi. 2024;25(4):315-20. doi: 10.36290/neu.2024.049. Go to original source...
    13. Knupp KG, Scheffer IE, Ceulemans B, et al. Fenfluramine provides clinically me-aningful reduction in frequency of drop seizures in patients with Lennox-Gastaut syndrome: Interim analysis of an open-label extension study. Epilepsia. 2023;64(1):139-51. doi: 10.1111/epi.17431. Go to original source...
    14. Lagae L, Brambilla I, Mingorance A, et al.Quality of life and comorbidities associ-ated with Dravet syndrome severity: a multinational cohort survey. Dev Med Child Neurol. 2018;60(1):63-72. doi: 10.1111/dmcn.13591. Go to original source... Go to PubMed...
    15. Lagae L, Sullivan J, Knupp K. Fenfluramine hydrochloride for the treatment of se-izures in Dravet syndrome: A randomised, double-blind, placebo-controlled trial. The Lancet. 2019;394(10216):2243-54. doi: 10.1016/S0140-6736(19)32500-0. Go to original source... Go to PubMed...
    16. Lattanzi S, Di Gennaro G, Bisulli F, et al. Adjunctive Fenfluramine in Adults with Lennox-Gastaut Syndrome: Effectiveness and Tolerability in Real-World Clinical Practice. CNS Drugs. 2026;40(9):1307-11. doi: 10.1007/s40263-026-01307-7. Go to original source... Go to PubMed...
    17. Martin P, Reeder T, Sourbron J, et al. An Emerging Role for Sigma-1 Receptors in the Treatment of Developmental and Epileptic Encephalopathies. Int J Mol Sci. 2021;22(16):8416. doi: 10.3390/ijms22168416. Go to original source... Go to PubMed...
    18. Nabbout R, Mistry A, Zuberi SM. Fenfluramine for treatment-resistant seizures in patients with Dravet syndrome receiving stiripentol-inclusive regimens: A rando-mized clinical trial. JAMA Neurology. 2020;77(3):300-8. doi: 10.1001/jamaneurol.2019.4113. Go to original source... Go to PubMed...
    19. Nizami FM, Trivedi S, Kalita J. A systematic review of electroencephalographic findings in Lennox-Gastaut syndrome. Epilepsy Research. 2024;205:107406. doi: 10.1016/j.eplepsyres.2024.107406. Go to original source... Go to PubMed...
    20. Scheffer IE, Nabbout R, Lagae L, et al. Long-term safety and effectiveness of fen-fluramine in children and adults with Dravet syndrome. Epilepsia. 2025;66(6):1919-32. doi: 10.1111/epi.18342. Go to original source...
    21. Selvarajah A, Sabo A, Gorodetsky C, et al. Dravet syndrome: From neurodevelo-pmental to neurodegenerative disease? Epilepsia. 2025;66(6):1975-87. doi: 10.1111/epi.18329. Go to original source... Go to PubMed...
    22. Selvarajah A, Zulfiqar-Ali Q, Marques P, et al. A systematic review of adults with Dravet syndrome. Seizure. 2021;87:39-45. doi: 10.1016/j.seizure.2021.02.025. Go to original source... Go to PubMed...

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