Fatal status epilepticus in dravet syndrome

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

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review


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.
Lingua originaleEnglish
pagine (da-a)1-9
Numero di pagine9
RivistaBrain Sciences
Stato di pubblicazionePubblicato - 2020


  • Acute encephalopathy
  • Cause of death
  • Dravet syndrome
  • SCN1A
  • Status epilepticus


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