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What Is Epilepsy?

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Textbook of Pediatric Neurosurgery

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Abstract

Epilepsy is a complex neurological disease, characterized by the propensity for ongoing unprovoked seizures as well by an array of neurocognitive, behavioral and social impairments. Seizures can be classified into those with generalized, focal or unknown onset. The etiology of epilepsy is variable ranging and includes underlying genetic, structural – metabolic and unknown causes. Over the last decades, The International League Against Epilepsy has played a pivotal role in reframing how we define and classify epilepsy. Throughout this chapter, we explore the definition of epilepsy and its etiologies as well as its underlying pathophysiology and briefly discuss management options. The underlying mechanisms for seizure development are discussed as well as the genetics of epilepsy. Importantly, we discuss several of the epilepsy syndromes throughout this chapter, which have their onset from the neonatal period to adolescence, and describe their key clinical features, treatment and prognosis.

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References

  • Arzimanoglou A, French J, Blume WT, Cross JH, Ernst JP, Feucht M et al (2009) Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol 8(1):82–93

    PubMed  Google Scholar 

  • Atakli D, Senadim S, Baslo SA, Guveli BT, Daryan MD, Sari H (2016) Misdiagnosis in JME: still a problem after 17 years? Seizure 36:27–30

    PubMed  Google Scholar 

  • Auvin S, Cilio MR, Vezzani A (2016) Current understanding and neurobiology of epileptic encephalopathies. Neurobiol Dis 92(Pt A):72–89

    CAS  PubMed  Google Scholar 

  • Beal JC, Cherian K, Moshe SL (2012) Early-onset epileptic encephalopathies: Ohtahara syndrome and early myoclonic encephalopathy. Pediatr Neurol 47(5):317–323

    PubMed  Google Scholar 

  • Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W et al (2010) Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia 51(4):676–685

    PubMed  Google Scholar 

  • Blumenfeld H (2005) Cellular and network mechanisms of spike-wave seizures. Epilepsia 46(Suppl 9):21–33

    CAS  PubMed  Google Scholar 

  • Brodie MJ (2016) Modern management of juvenile myoclonic epilepsy. Expert Rev Neurother 16(6):681–688

    CAS  PubMed  Google Scholar 

  • Broomfield E, Cavazos J, Sirven J (eds) (2006) An introduction to epilepsy [internet]. Chapter 1, Basic mechanisms underlying seizures and epilepsy. American Epilepsy Society, West Hartford

    Google Scholar 

  • Camfield PR (2011) Definition and natural history of Lennox-Gastaut syndrome. Epilepsia 52(Suppl 5):3–9

    PubMed  Google Scholar 

  • Camfield P, Camfield C (2015) Febrile seizures and genetic epilepsy with febrile seizures plus (GEFS+). Epileptic Disord 17(2):124–133

    PubMed  Google Scholar 

  • Camfield CS, Striano P, Camfield PR (2013) Epidemiology of juvenile myoclonic epilepsy. Epilepsy Behav 28(Suppl 1):S15–S17

    PubMed  Google Scholar 

  • Caraballo R, Koutroumanidis M, Panayiotopoulos CP, Fejerman N (2009) Idiopathic childhood occipital epilepsy of Gastaut: a review and differentiation from migraine and other epilepsies. J Child Neurol 24(12):1536–1542

    PubMed  Google Scholar 

  • Caraballo RH, Flesler S, Pasteris MC, Lopez Avaria MF, Fortini S, Vilte C (2013) Myoclonic epilepsy in infancy: an electroclinical study and long-term follow-up of 38 patients. Epilepsia 54(9):1605–1612

    CAS  PubMed  Google Scholar 

  • Carmant L, Whiting S (2012) Lennox-Gastaut syndrome: an update on treatment. Can J Neurol Sci 39(6):702–711

    PubMed  Google Scholar 

  • Connolly MB (2016) Dravet syndrome: diagnosis and long-term course. Can J Neurol Sci 43(Suppl 3):S3–S8

    PubMed  Google Scholar 

  • Donner EJ, Snead OC 3rd (2006) New generation anticonvulsants for the treatment of epilepsy in children. NeuroRx 3(2):170–180

    CAS  PubMed  PubMed Central  Google Scholar 

  • Dravet C (2011) The core Dravet syndrome phenotype. Epilepsia 52(Suppl 2):3–9

    PubMed  Google Scholar 

  • Ferrari-Marinho T, Macedo EF, Costa Neves RS, Costa LV, Tudesco IS, Carvalho KC et al (2013) Gastaut type idiopathic childhood occipital epilepsy. Epileptic Disord 15(1):80–83

    PubMed  Google Scholar 

  • Fisher RS (2015) Redefining epilepsy. Curr Opin Neurol 28(2):130–135

    CAS  PubMed  Google Scholar 

  • Fisher RS, Acevedo C, Arzimanoglou A, Bogacz A, Cross JH, Elger CE et al (2014) ILAE official report: a practical clinical definition of epilepsy. Epilepsia 55(4):475–482

    PubMed  Google Scholar 

  • Genton P, Thomas P, Kasteleijn-Nolst Trenite DG, Medina MT, Salas-Puig J (2013) Clinical aspects of juvenile myoclonic epilepsy. Epilepsy Behav 28(Suppl 1):S8–14

    PubMed  Google Scholar 

  • Glauser TA, Loddenkemper T (2013) Management of childhood epilepsy. Continuum (Minneap Minn) 19(3 Epilepsy):656–681

    Google Scholar 

  • Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S et al (2012) Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 78(24):1974–1980

    CAS  PubMed  PubMed Central  Google Scholar 

  • Gobbi G, Boni A, Filippini M (2006) The spectrum of idiopathic Rolandic epilepsy syndromes and idiopathic occipital epilepsies: from the benign to the disabling. Epilepsia 47(Suppl 2):62–66

    PubMed  Google Scholar 

  • Guerrini R, Pellacani S (2012) Benign childhood focal epilepsies. Epilepsia 53(Suppl 4):9–18

    PubMed  Google Scholar 

  • Hart AR, Pilling EL, Alix JJ (2015) Neonatal seizures-part 2: aetiology of acute symptomatic seizures, treatments and the neonatal epilepsy syndromes. Arch Dis Child Educ Pract Ed 100(5):226–232

    PubMed  Google Scholar 

  • Hawrylycz MJ, Lein ES, Guillozet-Bongaarts AL, Shen EH, Ng L, Miller JA et al (2012) An anatomically comprehensive atlas of the adult human brain transcriptome. Nature 489(7416):391–399

    CAS  PubMed  PubMed Central  Google Scholar 

  • Kelley SA, Kossoff EH (2010) Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress. Dev Med Child Neurol 52(11):988–993

    PubMed  Google Scholar 

  • Khan S, Al Baradie R (2012) Epileptic encephalopathies: an overview. Epilepsy Res Treat 2012:403592

    PubMed  PubMed Central  Google Scholar 

  • Korff CM, Nordli DR Jr (2006) Epilepsy syndromes in infancy. Pediatr Neurol 34(4):253–263

    PubMed  Google Scholar 

  • Korff CM, Jallon P, Lascano A, Michel C, Seeck M, Haenggeli CA (2009) Is benign myoclonic epilepsy of infancy truly idiopathic and generalized? Epileptic Disord 11(2):132–135

    PubMed  Google Scholar 

  • Kossoff EH, Zupec-Kania BA, Rho JM (2009) Ketogenic diets: an update for child neurologists. J Child Neurol 24(8):979–988

    PubMed  Google Scholar 

  • Koutroumanidis M (2007) Panayiotopoulos syndrome: an important electroclinical example of benign childhood system epilepsy. Epilepsia 48(6):1044–1053

    PubMed  Google Scholar 

  • Kwan P, Schachter SC, Brodie MJ (2011) Drug-resistant epilepsy. N Engl J Med 365(10):919–926

    CAS  PubMed  Google Scholar 

  • Lewis EC, Duchowny M (2015) Long-term seizure and antiepileptic drug outcomes after epilepsy surgery in children. In: Malmgren K, Baxendale S, Cross H (eds) Long-term outcomes of epilepsy surgery in adults and children, 1st edn. Springer, Switzerland, p 43

    Google Scholar 

  • Matricardi S, Verrotti A, Chiarelli F, Cerminara C, Curatolo P (2014) Current advances in childhood absence epilepsy. Pediatr Neurol 50(3):205–212

    PubMed  Google Scholar 

  • McCormick DA, Contreras D (2001) On the cellular and network bases of epileptic seizures. Annu Rev Physiol 63:815–846

    CAS  PubMed  Google Scholar 

  • Montouris GD (2011) Rational approach to treatment options for Lennox-Gastaut syndrome. Epilepsia 52(Suppl 5):10–20

    PubMed  Google Scholar 

  • Muthugovindan D, Hartman AL (2010) Pediatric epilepsy syndromes. Neurologist 16(4):223–237

    PubMed  Google Scholar 

  • Nickels K (2015) Seizure and psychosocial outcomes of childhood and juvenile onset generalized epilepsies: wolf in sheep’s clothing, or well-dressed wolf? Epilepsy Curr 15(3):114–117

    PubMed  PubMed Central  Google Scholar 

  • Ohtahara S, Yamatogi Y (2003) Epileptic encephalopathies in early infancy with suppression-burst. J Clin Neurophysiol 20(6):398–407

    PubMed  Google Scholar 

  • Ohtahara S, Yamatogi Y (2006) Ohtahara syndrome: with special reference to its developmental aspects for differentiating from early myoclonic encephalopathy. Epilepsy Res 70(Suppl 1):S58–S67

    PubMed  Google Scholar 

  • Ottman R (1993) Autosomal dominant partial epilepsy with auditory features. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH et al (eds) GeneReviews®. University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved

    Google Scholar 

  • Panayiotopoulos CP, Michael M, Sanders S, Valeta T, Koutroumanidis M (2008) Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 131(Pt 9):2264–2286

    PubMed  Google Scholar 

  • Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, Dlugos DJ et al (2010) Infantile spasms: a U.S. Consensus report. Epilepsia 51(10):2175–2189

    PubMed  Google Scholar 

  • Ramey WL, Martirosyan NL, Lieu CM, Hasham HA, Lemole GM Jr, Weinand ME (2013) Current management and surgical outcomes of medically intractable epilepsy. Clin Neurol Neurosurg 115(12):2411–2418

    PubMed  Google Scholar 

  • Scharfman HE (2007) The neurobiology of epilepsy. Curr Neurol Neurosci Rep 7(4):348–354

    CAS  PubMed  PubMed Central  Google Scholar 

  • Scheffer IE (2012) Diagnosis and long-term course of Dravet syndrome. Eur J Paediatr Neurol 16(Suppl 1):S5–S8

    PubMed  Google Scholar 

  • Scheffer IE, Zhang YH, Jansen FE, Dibbens L (2009) Dravet syndrome or genetic (generalized) epilepsy with febrile seizures plus? Brain and Development 31(5):394–400

    PubMed  Google Scholar 

  • Scheffer IE, Berkovic SF, et al (2014) The organization of the epilepsies: report of the ILAE Commission on Classification and Terminology

    Google Scholar 

  • Specchio N, Vigevano F (2006) The spectrum of benign infantile seizures. Epilepsy Res 70(Suppl 1):S156–S167

    PubMed  Google Scholar 

  • Stephani U (2006) The natural history of myoclonic astatic epilepsy (Doose syndrome) and Lennox-Gastaut syndrome. Epilepsia 47(Suppl 2):53–55

    PubMed  Google Scholar 

  • Tenney JR, Glauser TA (2013) The current state of absence epilepsy: can we have your attention? Epilepsy Curr 13(3):135–140

    PubMed  PubMed Central  Google Scholar 

  • Tenney JR, Jain SV (2014) Absence epilepsy: older vs newer AEDs. Curr Treat Options Neurol 16(5):290. -014-0290-9

    PubMed  Google Scholar 

  • Turnbull J, Lohi H, Kearney JA, Rouleau GA, Delgado-Escueta AV, Meisler MH et al (2005) Sacred disease secrets revealed: the genetics of human epilepsy. Hum Mol Genet 14(2):2491–2500

    PubMed  Google Scholar 

  • Verrotti A, Loiacono G, Coppola G, Spalice A, Mohn A, Chiarelli F (2011) Pharmacotherapy for children and adolescents with epilepsy. Expert Opin Pharmacother 12(2):175–194

    CAS  PubMed  Google Scholar 

  • Widjaja E, Go C, McCoy B, Snead OC (2015) Neurodevelopmental outcome of infantile spasms: a systematic review and meta-analysis. Epilepsy Res 109:155–162

    PubMed  Google Scholar 

  • Wirrell E (2016) Infantile, childhood, and adolescent epilepsies. Continuum (Minneap Minn) 22(1 Epilepsy):60–93

    Google Scholar 

  • Yamamoto H, Okumura A, Fukuda M (2011) Epilepsies and epileptic syndromes starting in the neonatal period. Brain Dev 33(3):213–220

    PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. SickKids, Toronto, Canada

    Berge Minassian

  2. Neurology Centre of Toronto, Toronto, ON, Canada

    Evan Lewis

  3. Division of Paediatric Neurology, SickKids, Toronto, Canada

    Robyn Whitney

Authors
  1. Berge Minassian
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  2. Evan Lewis
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  3. Robyn Whitney
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Corresponding author

Correspondence to Berge Minassian .

Editor information

Editors and Affiliations

  1. Pediatric Neurosurgery, International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Trust, London, UK

    Dachling Pang

  3. Division of Neurosurgery, The Hospital for Sick Children, Toronto, ON, Canada

    James T. Rutka

Section Editor information

  1. The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, The University of Toronto, Toronto, ONT, USA

    James Thomas Rutka

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Minassian, B., Lewis, E., Whitney, R. (2020). What Is Epilepsy?. In: Di Rocco, C., Pang, D., Rutka, J. (eds) Textbook of Pediatric Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-72168-2_96

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  • DOI: https://doi.org/10.1007/978-3-319-72168-2_96

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-72167-5

  • Online ISBN: 978-3-319-72168-2

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