Highlights & Basics
- Seizures in children may occur as a standalone event or may recur (epilepsy).
- Etiology can be structural, genetic, infectious, metabolic, immune, or unknown.
- An attempt should be made to identify the type(s) of epilepsy and the epilepsy syndrome by recognizing a pattern of seizure types, clinical features, etiologies, and electroencephalogram characteristics.
- Detailed history is of paramount importance in the diagnosis, as key diagnostic factors lie in the history as opposed to ancillary investigations.
- Treatment will vary depending on the epilepsy syndrome. Options include anticonvulsant medication, ketogenic diets, vagus nerve stimulation, and surgery, as well as consideration of lifestyle factors.
Quick Reference
History & Exam
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Definition
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Citations
Scheffer IE, Berkovic S, Capovilla G, et al. ILAE classification of the epilepsies: position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):512-21.[Abstract][Full Text]
Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy: position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):522-30.[Abstract][Full Text]
Wirrell EC, Nabbout R, Scheffer IE, et al. Methodology for classification and definition of epilepsy syndromes with list of syndromes: report of the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022 Jun;63(6):1333-48.[Abstract][Full Text]
Hirsch E, French J, Scheffer IE, et al. ILAE definition of the idiopathic generalized epilepsy syndromes: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022 Jun;63(6):1475-99.[Abstract][Full Text]
Gaillard WD, Chiron C, Cross JH, et al; ILAE Committee for Neuroimaging, Subcommittee for Pediatric Neuroimaging. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia. 2009 Sep;50(9):2147-53.[Abstract][Full Text]
American College of Radiology. ACR appropriateness criteria: seizures and epilepsy. 2019 [internet publication].[Full Text]
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 Jun;63(6):1349-97.[Abstract][Full Text]
Specchio N, Wirrell EC, Scheffer IE, et al. 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 Jun;63(6):1398-442.[Abstract][Full Text]
Kossoff EH, Zupec-Kania BA, Auvin S, et al; Charlie Foundation; Matthew's Friends; Practice Committee of the Child Neurology Society. Optimal clinical management of children receiving dietary therapies for epilepsy: updated recommendations of the International Ketogenic Diet Study Group. Epilepsia Open. 2018 Jun;3(2):175-92.[Abstract][Full Text]
1. Scheffer IE, Berkovic S, Capovilla G, et al. ILAE classification of the epilepsies: position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):512-21.[Abstract][Full Text]
2. Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy: position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):522-30.[Abstract][Full Text]
3. Wirrell EC, Nabbout R, Scheffer IE, et al. Methodology for classification and definition of epilepsy syndromes with list of syndromes: report of the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022 Jun;63(6):1333-48.[Abstract][Full Text]
4. Camfield P, Camfield C. Incidence, prevalence and aetiology of seizures and epilepsy in children. Epileptic Disord. 2015 Jun;17(2):117-23.[Abstract]
5. Eriksson KJ, Koivikko MJ. Prevalence, classification, and severity of epilepsy and epileptic syndromes in children. Epilepsia. 1997 Dec;38(12):1275-82.[Abstract][Full Text]
6. Ellis CA, Petrovski S, Berkovic SF. Epilepsy genetics: clinical impacts and biological insights. Lancet Neurol. 2020 Jan;19(1):93-100.[Abstract]
7. Hirsch E, French J, Scheffer IE, et al. ILAE definition of the idiopathic generalized epilepsy syndromes: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022 Jun;63(6):1475-99.[Abstract][Full Text]
8. Kramer MA, Cash SS. Epilepsy as a disorder of cortical network organization. Neuroscientist. 2012 Aug;18(4):360-72.[Abstract][Full Text]
9. Destexhe A, McCormick DA, Sejnowski TJ. Thalamic and thalamocortical mechanisms underlying 3 Hz spike-and-wave discharges. Prog Brain Res. 1999;121:289-307.[Abstract]
10. Thurman DJ, Begley CE, Carpio A, et al. The primary prevention of epilepsy: a report of the Prevention Task Force of the International League Against Epilepsy. Epilepsia. 2018 May;59(5):905-14.[Abstract][Full Text]
11. Appleton RE, Demellweek C. Post-traumatic epilepsy in children requiring inpatient rehabilitation following head injury. J Neurol Neurosurg Psychiatry. 2002 May;72(5):669-72.[Abstract][Full Text]
12. Stafstrom CE, Staedtke V, Comi AM. Epilepsy mechanisms in neurocutaneous disorders: tuberous sclerosis complex, neurofibromatosis type 1, and Sturge-Weber syndrome. Front Neurol. 2017 Mar 17;8:87.[Abstract][Full Text]
13. Vestergaard M, Pedersen CB, Sidenius P, et al. The long-term risk of epilepsy after febrile seizures in susceptible subgroups. Am J Epidemiol. 2007 Apr 15;165(8):911-8.[Abstract][Full Text]
14. Berg AT, Shinnar S, Levy SR, et al. Childhood-onset epilepsy with and without preceding febrile seizures. Neurology. 1999 Nov 10;53(8):1742-8.[Abstract]
15. Waruiru C, Appleton R. Febrile seizures: an update. Arch Dis Child. 2004 Aug;89(8):751-6.[Abstract][Full Text]
16. Giovanardi Rossi P, Posar A, Parmeggiani A. Epilepsy in adolescents and young adults with autistic disorder. Brain Dev. 2000 Mar;22(2):102-6.[Abstract]
17. Steffenburg S, Steffenburg U, Gillberg C. Autism spectrum disorders in children with active epilepsy and learning disability: comorbidity, pre- and perinatal background, and seizure characteristics. Dev Med Child Neurol. 2003 Nov;45(11):724-30.[Abstract]
18. Koutroumanidis M, Arzimanoglou A, Caraballo R, et al. The role of EEG in the diagnosis and classification of the epilepsy syndromes: a tool for clinical practice by the ILAE Neurophysiology Task Force (Part 1). Epileptic Disord. 2017 Sep 1;19(3):233-98.[Abstract]
19. Koutroumanidis M, Arzimanoglou A, Caraballo R, et al. The role of EEG in the diagnosis and classification of the epilepsy syndromes: a tool for clinical practice by the ILAE Neurophysiology Task Force (Part 2). Epileptic Disord. 2017 Dec 1;19(4):385-437.[Abstract]
20. Panayiotopoulos CP. A clinical guide to epileptic syndromes and their treatment. 2nd ed. London, UK: Springer; 2007.
21. Wirrell EC, Hood V, Knupp KG, et al. International consensus on diagnosis and management of Dravet syndrome. Epilepsia. 2022 Jul;63(7):1761-77.[Abstract][Full Text]
22. Lu Y, Waltz S, Stenzel K, et al. Photosensitivity in epileptic syndromes of childhood and adolescence. Epileptic Disord. 2008 Jun;10(2):136-43.[Abstract]
23. Gaillard WD, Chiron C, Cross JH, et al; ILAE Committee for Neuroimaging, Subcommittee for Pediatric Neuroimaging. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia. 2009 Sep;50(9):2147-53.[Abstract][Full Text]
24. Pressler R, Binnie CD, Cooper R, et al, eds. Neonatal and paediatric clinical neurophysiology. Edinburgh, UK: Churchill Livingstone; 2007.
25. Berg AT. Risk of recurrence after a first unprovoked seizure. Epilepsia. 2008;49(suppl 1):13-8.[Abstract]
26. Winckler MI, Rotta NT. Clinical and electroencephalographic follow-up after a first unprovoked seizure. Pediatr Neurol. 2004 Mar;30(3):201-6.[Abstract]
27. National Institute for Health and Care Excellence. Epilepsies in children, young people and adults. Apr 2022 [internet publication].[Full Text]
28. MacCormick JM, McAlister H, Crawford J, et al. Misdiagnosis of long QT syndrome as epilepsy at first presentation. Ann Emerg Med. 2009 Jul;54(1):26-32.[Abstract]
29. American College of Radiology. ACR appropriateness criteria: seizures and epilepsy. 2019 [internet publication].[Full Text]
30. Szaflarski JP, Gloss D, Binder JR, et al. Practice guideline summary: use of fMRI in the presurgical evaluation of patients with epilepsy. Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017 Jan 24;88(4):395-402.[Abstract][Full Text]
31. 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 Jun;63(6):1349-97.[Abstract][Full Text]
32. Specchio N, Wirrell EC, Scheffer IE, et al. 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 Jun;63(6):1398-442.[Abstract][Full Text]
33. Wirrell EC, Shellhaas RA, Joshi C, et al; Pediatric Epilepsy Research Consortium (PERC). How should children with West syndrome be efficiently and accurately investigated? Results from the National Infantile Spasms Consortium. Epilepsia. 2015 Apr;56(4):617-25.[Abstract][Full Text]
34. Stephani U. The natural history of myoclonic astatic epilepsy (Doose syndrome) and Lennox-Gastaut syndrome. Epilepsia. 2006;47(suppl 2):53-5.[Abstract][Full Text]
35. Avbersek A, Sisodiya S. Does the primary literature provide support for clinical signs used to distinguish psychogenic nonepileptic seizures from epileptic seizures? J Neurol Neurosurg Psychiatry. 2010 Jul;81(7):719-25.[Abstract]
36. Stone J, Burton C, Carson A. Recognising and explaining functional neurological disorder. BMJ. 2020 Oct 21;371:m3745.[Abstract]
37. Brown RJ, Reuber M. Psychological and psychiatric aspects of psychogenic non-epileptic seizures (PNES): a systematic review. Clin Psychol Rev. 2016 Apr;45:157-82.[Abstract]
38. Burgunder JM, Finsterer J, Szolnoki Z, et al. EFNS guidelines on the molecular diagnosis of channelopathies, epilepsies, migraine, stroke, and dementias. Eur J Neurol. 2010 May;17(5):641-8.[Abstract][Full Text]
39. Dwivedi R, Ramanujam B, Chandra PS, et al. Surgery for drug-resistant epilepsy in children. N Engl J Med. 2017 Oct 26;377(17):1639-47.[Abstract][Full Text]
40. Perucca E, Perucca P, White HS, et al. Drug resistance in epilepsy. Lancet Neurol. 2023 Aug;22(8):723-34.[Abstract]
41. Gidal B, Klein P, Hirsch LJ. Seizure clusters, rescue treatments, seizure action plans: unmet needs and emerging formulations. Epilepsy Behav. 2020 Nov;112:107391.[Abstract][Full Text]
42. Mesraoua B, Abou-Khalil B, Hosni Khodair R, et al. Seizure clusters. J Drug Assess. 2021 Aug 14;10(1):86-90.[Abstract][Full Text]
43. Jafarpour S, Hirsch LJ, Gaínza-Lein M, et al. Seizure cluster: definition, prevalence, consequences, and management. Seizure. 2019 May;68:9-15.[Abstract][Full Text]
44. Leone MA, Giussani G, Nevitt SJ, et al. Immediate antiepileptic drug treatment, versus placebo, deferred, or no treatment for first unprovoked seizure. Cochrane Database Syst Rev. 2021 May 4;(5):CD007144.[Abstract][Full Text]
45. Hirtz D, Berg A, Bettis D, et al. Practice parameter: treatment of the child with a first unprovoked seizure. Neurology. 2003 Jan 28;60(2):166-75.[Abstract][Full Text]
46. Guerrini R, Zaccara G, la Marca G, et al. Safety and tolerability of antiepileptic drug treatment in children with epilepsy. Drug Saf. 2012 Jul 1;35(7):519-33.[Abstract]
47. Mula M, Kanner AM, Schmitz B, et al. Antiepileptic drugs and suicidality: an expert consensus statement from the Task Force on Therapeutic Strategies of the ILAE Commission on Neuropsychobiology. Epilepsia. 2013 Jan;54(1):199-203.[Abstract][Full Text]
48. Kanner AM, Saporta AS, Kim DH, et al; Human Epilepsy Project. Mood and anxiety disorders and suicidality in patients with newly diagnosed focal epilepsy: an analysis of a complex comorbidity. Neurology. 2023 Mar 14;100(11):e1123-34.[Abstract][Full Text]
49. American College of Obstetricians and Gynecologists. Gynecologic management of adolescents and young women with seizure disorders: ACOG committee opinion, number 806. Obstet Gynecol. 2020 May;135(5):e213-20.[Abstract][Full Text]
50. European Medicines Agency. New measures to avoid valproate exposure in pregnancy endorsed. Mar 2018 [internet publication].[Full Text]
51. Medicines and Healthcare products Regulatory Agency. Antiepileptic drugs in pregnancy: updated advice following comprehensive safety review. Jan 2021 [internet publication].[Full Text]
52. Dreier JW, Bjørk MH, Alvestad S, et al. Prenatal exposure to antiseizure medication and incidence of childhood- and adolescence-onset psychiatric disorders. JAMA Neurol. 2023 Jun 1;80(6):568-77.[Abstract]
53. Athar F, Ehsan M, Farooq M, et al. Adverse fetal and neonatal outcomes following in-utero exposure to oxcarbazepine: a systematic review and meta-analysis. Br J Clin Pharmacol. 2022 Aug;88(8):3600-9.[Abstract][Full Text]
54. Bromley R, Adab N, Bluett-Duncan M, et al. Monotherapy treatment of epilepsy in pregnancy: congenital malformation outcomes in the child. Cochrane Database Syst Rev. 2023 Aug 29;(8):CD010224.[Abstract][Full Text]
55. Medicines and Healthcare products Regulatory Agency. Pregabalin (Lyrica): findings of safety study on risks during pregnancy. Apr 2022 [internet publication].[Full Text]
56. Bjørk MH, Zoega H, Leinonen MK, et al. Association of prenatal exposure to antiseizure medication with risk of autism and intellectual disability. JAMA Neurol. 2022 Jul 1;79(7):672-81.[Abstract][Full Text]
57. European Medicines Agency. New measures to avoid topiramate exposure in pregnancy. Oct 2023 [internet publication].[Full Text]
58. Medicines and Healthcare products Regulatory Agency. Topiramate (Topamax): start of safety review triggered by a study reporting an increased risk of neurodevelopmental disabilities in children with prenatal exposure. Jul 2022 [internet publication].[Full Text]
59. Robinson R. Vagal nerve stimulation is more effective than trials of further anti-epileptic drugs (AEDs) in children who have already tried >5 AEDs. Paper presented at: EPNS 2011 9th Congress of the European Paediatric Neurology Society. 11-14 May 2011. Dubrovnik, Croatia. Poster sessions: P14.3. Eur J Paediatr Neurol. 2011 May;15(suppl 1):89.
60. Englot DJ, Chang EF, Auguste KI. Vagus nerve stimulation for epilepsy: a meta-analysis of efficacy and predictors of response. J Neurosurg. 2011 Dec;115(6):1248-55.[Abstract]
61. Martin-McGill KJ, Bresnahan R, Levy RG, et al. Ketogenic diets for drug-resistant epilepsy. Cochrane Database Syst Rev. 2020 Jun 24;(6):CD001903.[Abstract][Full Text]
62. Kossoff EH, Zupec-Kania BA, Auvin S, et al; Charlie Foundation; Matthew's Friends; Practice Committee of the Child Neurology Society. Optimal clinical management of children receiving dietary therapies for epilepsy: updated recommendations of the International Ketogenic Diet Study Group. Epilepsia Open. 2018 Jun;3(2):175-92.[Abstract][Full Text]
63. Morris GL 3rd, Gloss D, Buchhalter J, et al. Evidence-based guideline update: vagus nerve stimulation for the treatment of epilepsy. Neurology. 2013 Oct 15;81(16):1453-9.[Abstract][Full Text]
64. Redgrave J, Day D, Leung H, et al. Safety and tolerability of transcutaneous vagus nerve stimulation in humans; a systematic review. Brain Stimul. 2018 Nov-Dec;11(6):1225-38.[Abstract]
65. Toffa DH, Touma L, El Meskine T, et al. Learnings from 30 years of reported efficacy and safety of vagus nerve stimulation (VNS) for epilepsy treatment: a critical review. Seizure. 2020 Dec;83:104-23.[Abstract][Full Text]
66. Sheng J, Liu S, Qin H, et al. Drug-resistant epilepsy and surgery. Curr Neuropharmacol. 2018;16(1):17-28.[Abstract][Full Text]
67. Bok LA, Maurits NM, Willemsen MA, et al. The EEG response to pyridoxine-IV neither identifies nor excludes pyridoxine-dependent epilepsy. Epilepsia. 2010 Dec;51(12):2406-11.[Abstract][Full Text]
68. Wilmshurst JM, Gaillard WD, Vinayan KP, et al. Summary of recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics. Epilepsia. 2015 Aug;56(8):1185-97.[Abstract][Full Text]
69. Ozawa H, Kawada Y, Noma S, et al. Oral high-dose phenobarbital therapy for early infantile epileptic encephalopathy. Pediatr Neurol. 2002 Mar;26(3):222-4.[Abstract]
70. Ohno M, Shimotsuji Y, Abe J, et al. Zonisamide treatment of early infantile epileptic encephalopathy. Pediatr Neurol. 2000 Oct;23(4):341-4.[Abstract]
71. Spoto G, Saia MC, Amore G, et al. Neonatal seizures: an overview of genetic causes and treatment options. Brain Sci. 2021 Sep 29;11(10):1295.[Abstract][Full Text]
72. Xu D, Chen S, Yang J, et al. Precision therapy with quinidine of KCNT1-related epileptic disorders: a systematic review. Br J Clin Pharmacol. 2022 Dec;88(12):5096-112.[Abstract][Full Text]
73. Malik SI, Galliani CA, Hernandez AW, et al. Epilepsy surgery for early infantile epileptic encephalopathy (Ohtahara syndrome). J Child Neurol. 2013 Dec;28(12):1607-17.[Abstract]
74. Nelson GR. Management of infantile spasms. Transl Pediatr. 2015 Oct;4(4):260-70.[Abstract][Full Text]
75. Hancock EC, Osborne JP, Edwards SW. Treatment of infantile spasms. Cochrane Database Syst Rev. 2013 Jun 5;(6):CD001770.[Abstract][Full Text]
76. O'Callaghan FJK, Edwards SW, Alber FD, et al; International Collaborative Infantile Spasms Study (ICISS) investigators. Vigabatrin with hormonal treatment versus hormonal treatment alone (ICISS) for infantile spasms: 18-month outcomes of an open-label, randomised controlled trial. Lancet Child Adolesc Health. 2018 Oct;2(10):715-25.[Abstract][Full Text]
77. Northrup H, Aronow ME, Bebin EM, et al; International Tuberous Sclerosis Complex Consensus Group. Updated international tuberous sclerosis complex diagnostic criteria and surveillance and management recommendations. Pediatr Neurol. 2021 Oct;123:50-66.[Abstract][Full Text]
78. Grinspan ZM, Knupp KG, Patel AD, et al. Comparative effectiveness of initial treatment for infantile spasms in a contemporary US cohort. Neurology. 2021 Jul 15;97(12):e1217-28.[Abstract][Full Text]
79. Demarest ST, Shellhaas RA, Gaillard WD, et al; Pediatric Epilepsy Research Consortium. The impact of hypsarrhythmia on infantile spasms treatment response: observational cohort study from the National Infantile Spasms Consortium. Epilepsia. 2017 Dec;58(12):2098-103.[Abstract][Full Text]
80. Knupp KG, Leister E, Coryell J, et al; Pediatric Epilepsy Research Consortium. Response to second treatment after initial failed treatment in a multicenter prospective infantile spasms cohort. Epilepsia. 2016 Nov;57(11):1834-42.[Abstract][Full Text]
81. O'Callaghan FJ, Edwards SW, Alber FD, et al. Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial. Lancet Neurol. 2017 Jan;16(1):33-42.[Abstract][Full Text]
82. Knupp KG, Coryell J, Nickels KC, et al; Pediatric Epilepsy Research Consortium. Response to treatment in a prospective national infantile spasms cohort. Ann Neurol. 2016 Mar;79(3):475-84.[Abstract][Full Text]
83. Riikonen R, Mankinen K, Gaily E. Long-term outcome in pyridoxine-responsive infantile epilepsy. Eur J Paediatr Neurol. 2015 Nov;19(6):647-51.[Abstract]
84. Sahu JK, Madaan P, Prakash K. The landscape of infantile epileptic spasms syndrome in South Asia: peculiarities, challenges, and way forward. Lancet Reg Health Southeast Asia. 2023 May;12:100170.[Abstract][Full Text]
85. Banerjee A, Sahu JK, Sankhyan N, et al. Randomized trial of high-dose pyridoxine in combination with standard hormonal therapy in West syndrome. Seizure. 2021 Oct;91:75-80.[Abstract][Full Text]
86. Auvin S, Pandit F, De Bellecize J, et al. Benign myoclonic epilepsy in infants: electroclinical features and long-term follow-up of 34 patients. Epilepsia. 2006 Feb;47(2):387-93.[Abstract][Full Text]
87. Caraballo RH, Flesler S, Pasteris MC, et al. Myoclonic epilepsy in infancy: an electroclinical study and long-term follow-up of 38 patients. Epilepsia. 2013 Sep;54(9):1605-12.[Abstract][Full Text]
88. Oguni H. Symptomatic epilepsies imitating idiopathic generalized epilepsies. Epilepsia. 2005 Nov;46(suppl 9):84-90.[Abstract][Full Text]
89. Lagae L. Dravet syndrome. Curr Opin Neurol. 2021 Apr 1;34(2):213-8.[Abstract]
90. Gao C, Pielas M, Jiao F, et al. Epilepsy in Dravet syndrome - current and future therapeutic opportunities. J Clin Med. 2023 Mar 27;12(7):2532.[Abstract][Full Text]
91. Brigo F, Igwe SC, Bragazzi NL. Antiepileptic drugs for the treatment of infants with severe myoclonic epilepsy. Cochrane Database Syst Rev. 2017 May 18;(5):CD010483.[Abstract][Full Text]
92. Zhang L, Li W, Wang C. Efficacy and safety of fenfluramine in patients with Dravet syndrome: a meta-analysis. Acta Neurol Scand. 2021 Apr;143(4):339-48.[Abstract]
93. Lattanzi S, Brigo F, Trinka E, et al. Adjunctive cannabidiol in patients with Dravet syndrome: a systematic review and meta-analysis of efficacy and safety. CNS Drugs. 2020 Mar;34(3):229-41.[Abstract]
94. American Epilepsy Society. AES position statement on cannabis as a treatment for patients with epileptic seizures. Sep 2022 [internet publication].[Full Text]
95. Gunning B, Mazurkiewicz-Bełdzińska M, Chin RFM, et al. Cannabidiol in conjunction with clobazam: analysis of four randomized controlled trials. Acta Neurol Scand. 2021 Feb;143(2):154-63.[Abstract][Full Text]
96. Lattanzi S, Trinka E, Striano P, et al. Cannabidiol efficacy and clobazam status: a systematic review and meta-analysis. Epilepsia. 2020 Jun;61(6):1090-8.[Abstract]
97. Nieto-Barrera M, Candau R, Nieto-Jimenez M, et al. Topiramate in the treatment of severe myoclonic epilepsy in infancy. Seizure. 2000 Dec;9(8):590-4.[Abstract]
98. Nickels K, Kossoff EH, Eschbach K, et al. Epilepsy with myoclonic-atonic seizures (Doose syndrome): clarification of diagnosis and treatment options through a large retrospective multicenter cohort. Epilepsia. 2021 Jan;62(1):120-7.[Abstract]
99. Nickels K, Thibert R, Rau S, et al; Pediatric Epilepsy Research Consortium. How do we diagnose and treat epilepsy with myoclonic-atonic seizures (Doose syndrome)? Results of the Pediatric Epilepsy Research Consortium survey. Epilepsy Res. 2018 Aug;144:14-9.[Abstract]
100. Joshi C, Nickels K, Demarest S, et al. Results of an international Delphi consensus in epilepsy with myoclonic atonic seizures/ Doose syndrome. Seizure. 2021 Feb;85:12-8.[Abstract][Full Text]
101. Brigo F, Jones K, Eltze C, et al. Anti-seizure medications for Lennox-Gastaut syndrome. Cochrane Database Syst Rev. 2021 Apr 7;(4):CD003277.[Abstract][Full Text]
102. Kanner AM, Ashman E, Gloss D, et al. Practice guideline update summary: efficacy and tolerability of the new antiepileptic drugs II: treatment-resistant epilepsy. Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society. Neurology. 2018 Jul 10;91(2):82-90.[Abstract]
103. Cross JH, Auvin S, Falip M, et al. Expert opinion on the management of Lennox-Gastaut syndrome: treatment algorithms and practical considerations. Front Neurol. 2017 Sep 29;8:505.[Abstract][Full Text]
104. Montouris G, Aboumatar S, Burdette D, et al. Expert opinion: proposed diagnostic and treatment algorithms for Lennox-Gastaut syndrome in adult patients. Epilepsy Behav. 2020 Sep;110:107146.[Abstract][Full Text]
105. Sharawat IK, Panda PK, Panda P, et al. Efficacy and safety of rufinamide as adjunctive therapy in patients with Lennox Gastaut syndrome: a systematic review and meta-analysis. Seizure. 2021 Oct;91:296-307.[Abstract][Full Text]
106. Thiele EA, Marsh ED, French JA, et al. Cannabidiol in patients with seizures associated with Lennox-Gastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet. 2018 Mar 17;391(10125):1085-96.[Abstract]
107. Devinsky O, Patel AD, Cross JH, et al. Effect of cannabidiol on drop seizures in the Lennox-Gastaut syndrome. N Engl J Med. 2018 May 17;378(20):1888-97.[Abstract][Full Text]
108. Fazlollahi A, Zahmatyar M, ZareDini M, et al. Adverse events of cannabidiol use in patients with epilepsy: a systematic review and meta-analysis. JAMA Netw Open. 2023 Apr 3;6(4):e239126.[Abstract][Full Text]
109. Knupp KG, Scheffer IE, Ceulemans B, et al. Efficacy and safety of fenfluramine for the treatment of seizures associated with Lennox-Gastaut syndrome: a randomized clinical trial. JAMA Neurol. 2022 Jun 1;79(6):554-64.[Abstract][Full Text]
110. Knupp KG, Scheffer IE, Ceulemans B, et al. Fenfluramine provides clinically meaningful reduction in frequency of drop seizures in patients with Lennox-Gastaut syndrome: interim analysis of an open-label extension study. Epilepsia. 2023 Jan;64(1):139-51.[Abstract][Full Text]
111. Agashe S, Worrell G, Britton J, et al. Cenobamate in generalized epilepsy and combined generalized and focal epilepsy. Neurol Clin Pract. 2023 Apr;13(2):e200133.[Abstract][Full Text]
112. Strzelczyk A, Schubert-Bast S. Expanding the treatment landscape for Lennox-Gastaut syndrome: current and future strategies. CNS Drugs. 2021 Jan;35(1):61-83.[Abstract][Full Text]
113. Brigo F, Igwe SC, Lattanzi S. Ethosuximide, sodium valproate or lamotrigine for absence seizures in children and adolescents. Cochrane Database Syst Rev. 2021 Jan 21;(1):CD003032.[Abstract][Full Text]
114. Nevitt SJ, Sudell M, Cividini S, et al. Antiepileptic drug monotherapy for epilepsy: a network meta-analysis of individual participant data. Cochrane Database Syst Rev. 2022 Apr 1;(4):CD011412.[Abstract][Full Text]
115. Glauser TA, Cnaan A, Shinnar S, et al; Childhood Absence Epilepsy Study Group. Ethosuximide, valproic acid, and lamotrigine in childhood absence epilepsy. N Engl J Med. 2010 Mar 4;362(9):790-9.[Abstract][Full Text]
116. Coppola G, Auricchio G, Federico R, et al. Lamotrigine versus valproic acid as first-line monotherapy in newly diagnosed typical absence seizures: an open-label, randomized, parallel-group study. Epilepsia. 2004 Sep;45(9):1049-53.[Abstract][Full Text]
117. Rinaldi VE, Di Cara G, Mencaroni E, et al. Therapeutic options for childhood absence epilepsy. Pediatr Rep. 2021 Dec 16;13(4):658-67.[Abstract][Full Text]
118. Trinka E, Alsaadi T, Goji H, et al. Perampanel for the treatment of people with idiopathic generalized epilepsy in clinical practice. Epilepsia. 2023 Aug;64(8):2094-107.[Abstract][Full Text]
119. Operto FF, Orsini A, Sica G, et al. Perampanel and childhood absence epilepsy: a real life experience. Front Neurol. 2022 Aug 11;13:952900.[Abstract][Full Text]
120. Trudeau V, Myers S, LaMoreaux L, et al. Gabapentin in naive childhood absence epilepsy: results from two double-blind, placebo-controlled, multicenter studies. J Child Neurol. 1996 Nov;11(6):470-5.[Abstract]
121. Zanzmera P, Menon RN, Karkare K, et al. Epilepsy with myoclonic absences: electroclinical characteristics in a distinctive pediatric epilepsy phenotype. Epilepsy Behav. 2016 Nov;64(pt a):242-7.[Abstract]
122. Smith KM, Wirrell EC, Andrade DM, et al. Management of epilepsy with eyelid myoclonia: results of an international expert consensus panel. Epilepsia. 2023 Sep;64(9):2342-50.[Abstract][Full Text]
123. Zawar I, Knight EP. Epilepsy with eyelid myoclonia (Jeavons syndrome). Pediatr Neurol. 2021 Aug;121:75-80.[Abstract]
124. Smith KM, Youssef PE, Wirrell EC, et al. Jeavons syndrome: clinical features and response to treatment. Pediatr Neurol. 2018 Sep;86:46-51.[Abstract]
125. Petropoulos MC, Bonaiuto K, Currier J, et al. Practical aspects of childhood epilepsy. BMJ. 2019 Nov 11;367:l6096.[Abstract]
126. Bresnahan R, Panebianco M, Marson AG. Lamotrigine add-on therapy for drug-resistant generalised tonic-clonic seizures. Cochrane Database Syst Rev. 2020 Jul 1;(7):CD007783.[Abstract][Full Text]
127. Caraballo R, Silva S, Beltran L, et al. Childhood-only epilepsy with generalized tonic-clonic seizures: a well-defined epileptic syndrome. Epilepsy Res. 2019 Jul;153:28-33.[Abstract]
128. Ji ZY, Huang YQ, He WZ. Sodium valproate combined with topiramate vs. sodium valproate alone for refractory epilepsy: a systematic review and meta-analysis. Front Neurol. 2022 Jan 5:12:794856.[Abstract][Full Text]
129. Murphy K, Delanty N. Primary generalized epilepsies. Curr Treat Options Neurol. 2000 Nov;2(6):527-42.[Abstract]
130. Cross JH. Pitfalls in the diagnosis and differential diagnosis of epilepsy. Paediatr Child Health. 2009 May;19(5):199-202.[Full Text]
131. Kostov H, Larsson PG, Roste GK. Is vagus nerve stimulation a treatment option for patients with drug-resistant idiopathic generalized epilepsy? Acta Neurol Scand Suppl. 2007;187:55-8.[Abstract]
132. Trevathan E, Kerls SP, Hammer AE, et al. Lamotrigine adjunctive therapy among children and adolescents with primary generalized tonic-clonic seizures. Pediatrics. 2006 Aug;118(2):e371-8.[Abstract]
133. Sharpe DV, Patel AD, Abou-Khalil B, et al. Levetiracetam monotherapy in juvenile myoclonic epilepsy. Seizure. 2008 Jan;17(1):64-8.[Abstract]
134. Verrotti A, Cerminara C, Coppola G, et al. Levetiracetam in juvenile myoclonic epilepsy: long-term efficacy in newly diagnosed adolescents. Dev Med Child Neurol. 2008 Jan;50(1):29-32.[Abstract]
135. Serafini A, Gerard E, Genton P, et al. Treatment of juvenile myoclonic epilepsy in patients of child-bearing potential. CNS Drugs. 2019 Mar;33(3):195-208.[Abstract]
136. Tennis P, Eldridge RR; International Lamotrigine Pregnancy Registry Scientific Advisory Committee. Preliminary results on pregnancy outcomes in women using lamotrigine. Epilepsia. 2002 Oct;43(10):1161-7.[Abstract][Full Text]
137. Bourgeois BF. Chronic management of seizures in the syndromes of idiopathic generalized epilepsy. Epilepsia. 2003 Mar;44 Suppl 2:27-32.[Abstract][Full Text]
138. Kothare SV, Valencia I, Khurana DS, et al. Efficacy and tolerability of zonisamide in juvenile myoclonic epilepsy. Epileptic Disord. 2004 Dec;6(4):267-70.[Abstract][Full Text]
139. Kessler SK, McGinnis E. A practical guide to treatment of childhood absence epilepsy. Paediatr Drugs. 2019 Feb;21(1):15-24.[Abstract][Full Text]
140. French JA, Krauss GL, Wechsler RT, et al. Perampanel for tonic-clonic seizures in idiopathic generalized epilepsy: a randomized trial. Neurology. 2015 Sep 15;85(11):950-7.[Abstract][Full Text]
141. Marson AG, Al-Kharusi AM, Alwaidh M, et al; SANAD Study group. The SANAD study of effectiveness of valproate, lamotrigine, or topiramate for generalised and unclassifiable epilepsy: an unblinded randomised controlled trial. Lancet. 2007 Mar 24;369(9566):1016-26.[Abstract][Full Text]
142. Wheless JW. Use of topiramate in childhood generalized seizure disorders. J Child Neurol. 2000;15(suppl 1):S7-13.[Abstract]
143. Wheless JW, Neto W, Wang S; EPMN-105 Study Group. Topiramate, carbamazepine, and valproate monotherapy: double-blind comparison in children with newly diagnosed epilepsy. J Child Neurol. 2004 Feb;19(2):135-41.[Abstract]
144. Boon P, Ferrao Santos S, Jansen AC, et al. Recommendations for the treatment of epilepsy in adult and pediatric patients in Belgium: 2020 update. Acta Neurol Belg. 2021 Feb;121(1):241-57.[Abstract][Full Text]
145. Gloss D, Pargeon K, Pack A, et al. Antiseizure medication withdrawal in seizure-free patients: practice advisory update summary: report of the AAN guideline subcommittee. Neurology. 2021 Dec 7;97(23):1072-81.[Abstract][Full Text]
146. Ayuga Loro F, Gisbert Tijeras E, Brigo F. Rapid versus slow withdrawal of antiepileptic drugs. Cochrane Database Syst Rev. 2022 Jan 10;(1):CD005003.[Abstract][Full Text]
147. De SK. Ganaxolone: first FDA-approved medicine for the treatment of seizures associated with cyclin-dependent kinase-like 5 deficiency disorder. Curr Med Chem. 2024;31(4):388-92.[Abstract][Full Text]
148. Olson HE, Demarest ST, Pestana-Knight EM, et al. Cyclin-dependent kinase-like 5 deficiency disorder: clinical review. Pediatr Neurol. 2019 Aug;97:18-25.[Abstract][Full Text]
149. Hahn CD, Jiang Y, Villanueva V, et al. A phase 2, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of soticlestat as adjunctive therapy in pediatric patients with Dravet syndrome or Lennox-Gastaut syndrome (ELEKTRA). Epilepsia. 2022 Oct;63(10):2671-83.[Abstract][Full Text]
150. Tolete P, Knupp K, Karlovich M, et al. Lorcaserin therapy for severe epilepsy of childhood onset: a case series. Neurology. 2018 Oct 30;91(18):837-9.[Abstract][Full Text]
151. ClinicalTrials.gov. A study of lorcaserin as adjunctive treatment in participants with Dravet syndrome (MOMENTUM 1). NCT04572243. Jun 2023 [internet publication].[Full Text]
152. ClinicalTrials.gov. Carisbamate in adult and pediatric subjects with Lennox-Gastaut syndrome. NCT03731715. May 2023 [internet publication].[Full Text]
153. ClinicalTrials.gov. Carisbamate safety study in adult and pediatric subjects with Lennox-Gastaut syndrome. NCT04062981. May 2023 [internet publication].[Full Text]
154. ClinicalTrials.gov. Investigate efficacy and safety of carisbamate as adjunctive treatment for seizures associated with LGS in children and adults. NCT05219617. Nov 2023 [internet publication].[Full Text]
155. Bialer M, Johannessen SI, Koepp MJ, et al. Progress report on new antiepileptic drugs: a summary of the Sixteenth Eilat Conference on New Antiepileptic Drugs and Devices (EILAT XVI): I. Drugs in preclinical and early clinical development. Epilepsia. 2022 Nov;63(11):2865-82.[Abstract]
156. ClinicalTrials.gov. An open-label extension of the study XEN496 (Ezogabine) in Children With KCNQ2-DEE (EPIK-OLE). NCT04912856. Jun 2023 [internet publication].[Full Text]
157. He Z, Li Y, Zhao X, et al. Dravet syndrome: advances in etiology, clinical presentation, and treatment. Epilepsy Res. 2022 Dec;188:107041.[Abstract]
158. Nelson JA, Knupp KG. Lennox-Gastaut syndrome: current treatments, novel therapeutics, and future directions. Neurotherapeutics. 2023 Sep;20(5):1255-62.[Abstract]
159. ClinicalTrials.gov. An open-label study to investigate the safety of single and multiple ascending doses in children and adolescents with Dravet syndrome. NCT04442295. Nov 2023 [internet publication].[Full Text]
160. ClinicalTrials.gov. An open-label extension study of STK-001 for patients with Dravet syndrome. NCT04740476. Nov 2023 [internet publication].[Full Text]
161. Lundstrom BN, Osman GM, Starnes K, et al. Emerging approaches in neurostimulation for epilepsy. Curr Opin Neurol. 2023 Apr 1;36(2):69-76.[Abstract]
162. Haneef Z, Skrehot HC. Neurostimulation in generalized epilepsy: a systematic review and meta-analysis. Epilepsia. 2023 Apr;64(4):811-20.[Abstract]
163. Gummadavelli A, Englot DJ, Schwalb JM, et al; American Society for Stereotactic and Functional Neurosurgeons. ASSFN position statement on deep brain stimulation for medication-refractory epilepsy. Neurosurgery. 2022 May 1;90(5):636-41.[Abstract][Full Text]
164. Walton D, Spencer DC, Nevitt SJ, et al. Transcranial magnetic stimulation for the treatment of epilepsy. Cochrane Database Syst Rev. 2021 Apr 15;(4):CD011025.[Abstract][Full Text]
165. Simula S, Daoud M, Ruffini G, et al. Transcranial current stimulation in epilepsy: a systematic review of the fundamental and clinical aspects. Front Neurosci. 2022 Aug 25;16:909421.[Abstract][Full Text]
166. Kanner AM, Ashman E, Gloss D, et al. Practice guideline update summary: efficacy and tolerability of the new antiepileptic drugs I: treatment of new-onset epilepsy: report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society. Neurology. 2018 Jul 10;91(2):74-81.[Abstract][Full Text]
167. Harden C, Tomson T, Gloss D, et al; American Academy of Neurology, American Epilepsy Society. Practice guideline summary: sudden unexpected death in epilepsy incidence rates and risk factors. Neurology. 2017 Apr 25;88(17):1674-80.[Abstract][Full Text]
168. Capovilla G, Kaufman KR, Perucca E, et al. Epilepsy, seizures, physical exercise, and sports: a report from the ILAE Task Force on Sports and Epilepsy. Epilepsia. 2016 Jan;57(1):6-12.[Abstract][Full Text]
169. Glauser T, Ben-Menachem E, Bourgeois B, et al; ILAE Subcommission on AED Guidelines. Updated ILAE evidence review of antiepileptic drug efficacy and effectiveness as initial monotherapy for epileptic seizures and syndromes. Epilepsia. 2013 Mar;54(3):551-63.[Abstract][Full Text]
170. Hauser E, Freilinger M, Seidl R, et al. Prognosis of childhood epilepsy in newly referred patients. J Child Neurol. 1996 May;11(3):201-4.[Abstract]
171. Ramos Lizana J, Cassinello Garcia E, Carrasco Marina LL, et al. Seizure recurrence after a first unprovoked seizure in childhood: a prospective study. Epilepsia. 2000 Aug;41(8):1005-13.[Abstract][Full Text]
172. Sillanpää M, Schmidt D. Natural history of treated childhood-onset epilepsy: prospective, long-term population-based study. Brain. 2006 Mar;129(Pt 3):617-24.[Abstract][Full Text]
173. Guery D, Rheims S. Clinical management of drug resistant epilepsy: a review on current strategies. Neuropsychiatr Dis Treat. 2021 Jul 12;17:2229-42.[Abstract][Full Text]
174. McIntyre J, Robertson S, Norris E, et al. Safety and efficacy of buccal midazolam versus rectal diazepam for emergency treatment of seizures in children: a randomised controlled trial. Lancet. 2005 Jul 16-22;366(9481):205-10.[Abstract]
175. Holsti M, Sill BL, Firth SD, et al. Prehospital intranasal midazolam for the treatment of pediatric seizures. Pediatr Emerg Care. 2007 Mar;23(3):148-53.[Abstract]
176. Pensel MC, Nass RD, Taubøll E, et al. Prevention of sudden unexpected death in epilepsy: current status and future perspectives. Expert Rev Neurother. 2020 May;20(5):497-508.[Abstract]
177. Wilson SJ, Baxendale S, Barr W, et al. Indications and expectations for neuropsychological assessment in routine epilepsy care: report of the ILAE Neuropsychology Task Force, Diagnostic Methods Commission, 2013-2017. Epilepsia. 2015 May;56(5):674-81.[Abstract][Full Text]
178. Keezer MR, Sisodiya SM, Sander JW. Comorbidities of epilepsy: current concepts and future perspectives. Lancet Neurol. 2016 Jan;15(1):106-15.[Abstract]
179. Hesdorffer DC, Ishihara L, Mynepalli L, et al. Epilepsy, suicidality, and psychiatric disorders: a bidirectional association. Ann Neurol. 2012 Aug;72(2):184-91.[Abstract]
180. Patel AD, Baca C, Franklin G, et al. Quality improvement in neurology: Epilepsy Quality Measurement Set 2017 update. Neurology. 2018 Oct 30;91(18):829-36.[Abstract][Full Text]
181. Maguire MJ, Marson AG, Nevitt SJ. Antidepressants for people with epilepsy and depression. Cochrane Database Syst Rev. 2021 Apr 16;(4):CD010682.[Abstract][Full Text]
182. Cheng LS, Prasad AN, Rieder MJ. Relationship between antiepileptic drugs and biological markers affecting long-term cardiovascular function in children and adolescents. Can J Clin Pharmacol. 2010 Winter;17(1):e5-46.[Abstract]
183. American Epilepsy Society. Substitution of different formulations of antiseizure medication for the treatment of epilepsy. Aug 2023 [internet publication].[Full Text]
184. Fleeman N, Bradley PM, Panebianco M, et al. Care delivery and self-management strategies for children with epilepsy. Cochrane Database Syst Rev. 2022 Apr 27;(4):CD006245.[Abstract][Full Text]
