Pharmacological management of prolonged seizures in Dravet syndrome including intravenous phenytoin

Author:

Abi Tayeh Rana1,Dozières‐Puyravel Blandine12ORCID,Arnaud Lionel3,Titomanlio Luigi4,Dauger Stéphane5,Höhn Sophie1,Le Guern Eric3ORCID,Auvin Stéphane126ORCID

Affiliation:

1. Pediatric Neurology Department, Centre de Référence Maladies Rares Epilepsies Rares, EpiCARE member Assistance Publique ‐ Hôpitaux de Paris, Robert Debré University Hospital Paris France

2. Université Paris Cité, Institut national de la santé et de la recherche médicale NeuroDiderot Paris France

3. Département de Génétique Sorbonne Université, AP‐HP, Hôpital Universitaire Pitié‐Salpêtrière Paris France

4. Pediatric Emergency Department Assistance Publique ‐ Hôpitaux de Paris, Robert Debré University Hospital Paris France

5. Pediatric Intensive Care Unit Assistance Publique ‐ Hôpitaux de Paris, Robert Debré University Hospital Paris France

6. Institut Universitaire de France Paris France

Abstract

AbstractDravet syndrome (DS) is an infantile onset developmental and epileptic encephalopathy. Sodium channel blockers are known to exacerbate seizures in this syndrome. Due to its high incidence, the management of prolonged seizures is crucial for DS patients. There is still ambiguity regarding the use of intravenous phenytoin for prolonged seizure in DS patients mainly due to the lack of data, raising concern about the safety of it use. We conducted a retrospective study (from January 2009 to January 2020) aiming to assess the management of prolonged seizures in DS with a focus on the use of intravenous phenytoin. Data were collected for patients admitted to our hospital for seizures lasting >5 min. Among 52 identified patients in our database, 23 experienced 59 prolonged seizures managed in our hospital. Only four seizures ceased without rescue medication. Notably, the use of intravenous phenytoin was not associated with discernible adverse effects and was effective in stopping status epilepticus in 71% of cases. This study suggests the safety and efficacy of intravenous phenytoin for prolonged seizure in DS. There is a need for broader investigations of emergency treatments for evidence‐based recommendations for the emergency plan of each patient.

Publisher

Wiley

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