Derby Ayudhia Utami Iskandar Putri, Riri Gusnita Sari


The definition and classification of epilepsy syndrome was first presented by the International League Against Epilepsy (ILAE) in 1985. According to the ILAE, epilepsy syndrome is an epileptic disorder characterized by a group of signs and symptoms, which can occur simultaneously. Symptoms depend on age and a certain set of comorbidities. The author aims to combine various current literature regarding epilepsy syndromes. Literature was taken using the Google Scholar, PubMed, Cochrane Library databases over a period of 4 (four) years, 2019-2023. The studies included in the literature that met the inclusion criteria encompass those that describe epilepsy syndrome and its characteristics. Classification of epilepsy syndromes was divided based on seizure type and age of onset. Each type has different characteristics and management. The most common epilepsy syndromes in childhood include, Generalized Tonic-Clonic Seizures Alone/ GTCSA, Childhood Absence Epilepsy/ CAE, Lennox-Gastaut Syndrome/ LGS, West syndrome/ WS, Self-Limited Childhood Epilepsy with Centrotemporal Spikes/ SLCECTS. It can be concluded that the initiation of antiepileptic treatment for epilepsy syndrome depends on age and the varying effectiveness of antiepileptic drugs.

Full text article

Generated from XML file


1. Zuberi SM, Wirrell E, Yozawitz E, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022;63(6):1349-1397. doi:10.1111/epi.17239
2. Yang Q, Hu D, Wang T, et al. Childhood epilepsy syndromes classification based on fused features of electroencephalogram and electrocardiogram. Cogn Comput Syst. 2022;4(1):1-10. doi:10.1049/ccs2.12035
3. Stödberg T, Tomson T, Barbaro M, et al. Epilepsy syndromes, etiologies, and the use of next-generation sequencing in epilepsy presenting in the first 2 years of life: A population-based study. Epilepsia. 2020;61(11):2486-2499. doi:10.1111/epi.16701
4. Howell KB, Freeman JL, Mackay MT, et al. The severe epilepsy syndromes of infancy: A population-based study. Epilepsia. 2021;62(2):358-370. doi:10.1111/epi.16810
5. Mohamed IN, Elseed MA, Mohamed S, et al. Classification and management of epilepsy and epileptic syndromes in a cohort of 202 school children- a 2 year follow up study- Sudan. BMC Neurol. 2019;19(1):1-6. doi:10.1186/s12883-019-1514-0
6. Kapoor D, Sharma S, Garg D, et al. Intravenous Methylprednisolone Versus Oral Prednisolone for West Syndrome: A Randomized Open-Label Trial. Indian J Pediatr. 2021;88(8):778-784. doi:10.1007/s12098-020-03630-3
7. Sagar P, Wawryk O, Vogrin S, et al. Efficacy and tolerability of adjuvant perampanel: an Australian multicenter real-world observational study in refractory focal and generalized epilepsy syndromes. Epilepsy Behav. 2021;119:107935. doi:10.1016/j.yebeh.2021.107935
8. Poudel P, Kafle SP, Pokharel R. Clinical profile and treatment outcome of epilepsy syndromes in children: A hospital-based study in Eastern Nepal. Epilepsia Open. 2021;6(1):206-215. doi:10.1002/epi4.12470
9. Zhao B, Liao S, Zhong X, et al. Effectiveness and Safety of Oxcarbazepine vs. Levetiracetam as Monotherapy for Infantile Focal Epilepsy: A Longitudinal Cohort Study. Front Neurol. 2022;13(June):1-9. doi:10.3389/fneur.2022.909191
10. Verma A, Kumar A. Classification Of Epilepsies And Epileptic Syndromes Using International League Against Epilepsy 2010: A Hospital Based Study From Rural Population Of North India. Int J Sci Res. 2019;90(1):60-62.
11. Wirrell E, Tinuper P, Perucca E, Moshé SL. Introduction to the epilepsy syndrome papers. Epilepsia. 2022;63(6):1330-1332. doi:10.1111/epi.17262
12. Specchio N, Pietrafusa N. New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. Dev Med Child Neurol. 2020;62(8):897-905. doi:10.1111/dmcn.14553
13. Clarkson BDS, LaFrance-Corey RG, Kahoud RJ, Farias-Moeller R, Payne ET, Howe CL. Functional deficiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome. Ann Neurol. 2019;85(4):526-537. doi:10.1002/ana.25439
14. Payne ET, Koh S, Wirrell EC. Extinguishing Febrile Infection-Related Epilepsy Syndrome: Pipe Dream or Reality? Semin Neurol. 2020;40(2):263-272. doi:10.1055/s-0040-1708503
15. Serino D, Santarone ME, Caputo D, Fusco L. Febrile infection-related epilepsy syndrome (FIRES): Prevalence, impact and management strategies. Neuropsychiatr Dis Treat. 2019;15:1897-1903. doi:10.2147/NDT.S177803
16. Katyayan A, Diaz-Medina G. Epilepsy: Epileptic Syndromes and Treatment. Neurol Clin. 2021;39(3):779-795. doi:10.1016/j.ncl.2021.04.002
17. Riney K, Bogacz A, Somerville E, et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset at a variable age: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022;63(6):1443-1474. doi:10.1111/epi.17240
18. Peng P, Peng J, Yin F, et al. Ketogenic diet as a treatment for super-refractory status epilepticus in febrile infection-related epilepsy syndrome. Front Neurol. 2019;10(APR). doi:10.3389/fneur.2019.00423
19. Koh S, Wirrell E, Vezzani A, et al. Proposal to optimize evaluation and treatment of Febrile infection-related epilepsy syndrome (FIRES): A Report from FIRES workshop. Epilepsia Open. 2021;6(1):62-72. doi:10.1002/epi4.12447
20. Mironov, M., Chebanenko, N., Burd, S., Rubleva, Y. V., Tikhonova, O., Krasilshchikova, T., Zykov, V., & Shamalov N. The effectiveness of antiepileptic therapy in patients with epileptic syndromes associated with myoclonic seizures. Zhurnal Nevrol i Psikhiatrii Im SS Korsakova. 2020;120(4):51-59.
21. Cervenka, M. C., Wood, S., Bagary, M., Balabanov, A., Bercovici, E., Brown, M.-G., Devinsky, O., Di Lorenzo, C., Doherty, C. P., & Felton E. International recommendations for the management of adults treated with ketogenic diet therapies. Neurol Clin Pract. 2021;11(5):385-397.
22. Obara, K., & Ono T. Ketogenic diet for a young adult patient with chronic-phase febrile infection-related epilepsy syndrome. Cureus. 14(2).


Derby Ayudhia Utami Iskandar Putri
ayudhiaderby@gmail.com (Primary Contact)
Riri Gusnita Sari

Article Details