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De Liso P, Pironi V, Mastrangelo M, et al. Fatal Status Epilepticus in Dravet Syndrome. Brain Sci. 2020;10(11)doi: 10.3390/brainsci10110889.
De Liso, P., Pironi, V., Mastrangelo, M., Battaglia, D., Craiu, D., Trivisano, M., Specchio, N., Nabbout, R., & Vigevano, F. (2020). Fatal Status Epilepticus in Dravet Syndrome. Brain sciences, 10(11), . https://doi.org/10.3390/brainsci10110889
De Liso, Paola, et al. "Fatal Status Epilepticus in Dravet Syndrome." Brain sciences vol. 10,11 (2020). doi: https://doi.org/10.3390/brainsci10110889
De Liso P, Pironi V, Mastrangelo M, Battaglia D, Craiu D, Trivisano M, Specchio N, Nabbout R, Vigevano F. Fatal Status Epilepticus in Dravet Syndrome. Brain Sci. 2020 Nov 23;10(11). doi: 10.3390/brainsci10110889. PMID: 33238377; PMCID: PMC7700506.
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Brain Sci. 2020 Nov 23;10(11). doi: 10.3390/brainsci10110889.

Fatal Status Epilepticus in Dravet Syndrome.

Brain sciences

Paola De Liso, Virginia Pironi, Massimo Mastrangelo, Domenica Battaglia, Dana Craiu, Marina Trivisano, Nicola Specchio, Rima Nabbout, Federico Vigevano

Affiliations

  1. Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, Full Member of European Reference Network EpiCARE, 00165 Rome, Italy.
  2. Center for Rare Diseases and Birth Defects, Department of Woman and Child Health, Institute of Pediatrics, Policlinico Universitario Gemelli Foundation, Catholic University of Rome, 00168 Rome, Italy.
  3. Paediatric Neurology Unit, V. Buzzi Hospital, A.O. ICP, 20019 Milan, Italy.
  4. Department of Child Neurology and Psychiatry, Policlinico Universitario Gemelli Foundation, Catholic University of Rome, 00153 Rome, Italy.
  5. Department of Neurology, Paediatric Neurology, Psychiatry, Neurosurgery, "Carol Davila" University of Medicine of Bucharest, Full Member of European Reference Network EpiCARE, 050474 Bucharest, Romania.
  6. Centre for Rare Epilepsies, Department of Paediatric Neurology, Necker-Enfants Malades Hospital, Imagine Institute, INSERMU1163, Paris Descartes University, Full Member of European Reference Network EpiCARE, 75006 Paris, France.

PMID: 33238377 PMCID: PMC7700506 DOI: 10.3390/brainsci10110889

Abstract

Dravet Syndrome (DS) is burdened by high epilepsy-related premature mortality due to status epilepticus (SE). We surveyed centres within Europe through the Dravet Italia Onlus and EpiCARE network (European Reference Network for Rare and Complex Epilepsies). We collated responses on seven DS SCN1A+ patients who died following refractory SE (mean age 6.9 year, range 1.3-23.4 year); six were on valproate, clobazam, and stiripentol. All patients had previous SE. Fatal SE was always triggered by fever: either respiratory infection or one case of hexavalent vaccination. SE lasted between 80 min and 9 h and all patients received IV benzodiazepines. Four patients died during or within hours of SE; in three patients, SE was followed by coma with death occurring after 13-60 days. Our survey supports the hypothesis that unresponsive fever is a core characteristic feature of acute encephalopathy. We highlight the need for management protocols for prolonged seizures and SE in DS.

Keywords: Dravet syndrome; SCN1A; acute encephalopathy; cause of death; status epilepticus

References

  1. Pediatrics. 2017 Apr;139(4): - PubMed
  2. Epilepsia. 2011 Apr;52 Suppl 2:50-4 - PubMed
  3. Epilepsy Behav Case Rep. 2017 Dec 24;9:29-32 - PubMed
  4. Epilepsia. 2018 Jul;59(7):1372-1380 - PubMed
  5. Epilepsia. 2012 Jan;53(1):79-86 - PubMed
  6. Epilepsy Res. 2019 Feb;150:66-69 - PubMed
  7. Dev Med Child Neurol. 2018 Jun;60(6):566-573 - PubMed
  8. Heart Rhythm. 2014 Jul;11(7):1202-9 - PubMed
  9. Neurochem Res. 2013 Nov;38(11):2276-86 - PubMed
  10. Lancet Neurol. 2006 Jun;5(6):488-92 - PubMed
  11. Neuropediatrics. 2011 Feb;42(2):78-81 - PubMed
  12. Brain. 2011 Oct;134(Pt 10):2982-3010 - PubMed
  13. Epilepsy Behav. 2019 Dec;101(Pt B):106539 - PubMed
  14. Pediatr Neurol. 2017 Mar;68:18-34.e3 - PubMed
  15. Seizure. 2020 May;78:49-52 - PubMed
  16. Epilepsy Res. 2016 Sep;125:42-6 - PubMed
  17. Epilepsia. 2011 Apr;52 Suppl 2:95-101 - PubMed
  18. Acta Neurol Scand Suppl. 2007;186:45-56 - PubMed
  19. Lancet Neurol. 2010 Jun;9(6):592-8 - PubMed
  20. Epilepsy Res. 2016 Dec;128:43-47 - PubMed
  21. Epilepsy Behav. 2016 Nov;64(Pt A):69-74 - PubMed
  22. Brain Dev. 2001 Nov;23(7):736-48 - PubMed
  23. Epilepsia. 2010 Jun;51(6):1043-52 - PubMed
  24. Seizure. 2010 Apr;19(3):190-4 - PubMed
  25. Epilepsia. 2011 Apr;52 Suppl 2:44-9 - PubMed

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