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Epileptic Encephalopathies

  • Thalia Valeta
Chapter

Abstract

Epileptic encephalopathies are severe diseases of the brain starting at an early age of childhood and more usually in infancy. They manifest with (1) frequent and various types of epileptic seizures which are resistant to treatment and (2) cognitive, behavioural and neurological deficits which often worsen with time (progressive). West, Lennox-Gastaut and Dravet syndrome are described in this chapter. In addition to addressing seizures, holistic care for patients with epileptic encephalopathies must involve a multidisciplinary team that includes specialists in psychotherapy, physical, occupational and speech therapy and social work. Families need significant and continuous multiple support.

Keywords

West syndrome Lennox-Gastaut syndrome Dravet syndrome Cognitive decline Psychological support Treatment SUDEP 

References

  1. 1.
    Guerrini R, Pellock JM. Age-related epileptic encephalopathies. Handb Clin Neurol. 2012;107:179–93.CrossRefGoogle Scholar
  2. 2.
    Covanis A. Epileptic encephalopathies (including severe epilepsy syndromes). Epilepsia. 2012;53(Suppl 4):114–26.CrossRefGoogle Scholar
  3. 3.
    Wirrell EC, Laux L, Donner E, Jette N, Knupp K, Meskis MA, et al. Optimizing the diagnosis and management of Dravet syndrome: recommendations from a North American Consensus Panel. Pediatr Neurol. 2017;68:18–34.CrossRefGoogle Scholar
  4. 4.
    Camfield P, Camfield C, Nolan K. Helping families cope with the severe stress of Dravet syndrome. Can J Neurol Sci. 2016;43(Suppl 3):S9–S12.CrossRefGoogle Scholar
  5. 5.
    McTague A, Howell KB, Cross JH, Kurian MA, Scheffer IE. The genetic landscape of the epileptic encephalopathies of infancy and childhood. Lancet Neurol. 2015;15:304–16.CrossRefGoogle Scholar
  6. 6.
    Cetica V, Chiari S, Mei D, Parrini E, Grisotto L, Marini C, et al. Clinical and genetic factors predicting Dravet syndrome in infants with SCN1A mutations. Neurology. 2017;88:1037–44.CrossRefGoogle Scholar
  7. 7.
    Vigevano F, Arzimanoglou A, Plouin P, Specchio N. Therapeutic approach to epileptic encephalopathies. Epilepsia. 2013;54(Suppl 8):45–50.CrossRefGoogle Scholar
  8. 8.
    Covanis A. Clinical management of epileptic encephalopathies of childhood and infancy. Expert Rev Neurother. 2014;14:687–701.CrossRefGoogle Scholar
  9. 9.
    Jehi L, Wyllie E, Devinsky O. Epileptic encephalopathies: optimizing seizure control and developmental outcome. Epilepsia. 2015;56:1486–9.CrossRefGoogle Scholar
  10. 10.
    Wirrell EC. Treatment of Dravet syndrome. Can J Neurol Sci. 2016;43(Suppl 3):S13–8.CrossRefGoogle Scholar
  11. 11.
    Coppola G, Toro A, Operto FF, Ferrarioli G, Pisano S, Viggiano A, et al. Mozart's music in children with drug-refractory epileptic encephalopathies. Epilepsy Behav. 2015;50:18–22.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  • Thalia Valeta
    • 1
    • 2
  1. 1.First Neurology and Psychiatry ClinicUniversity of Athens, Aeginition HospitalAthensGreece
  2. 2.Department of Clinical Neurophysiology and EpilepsiesSt. Thomas’ HospitalLondonUK

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