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Journal of Pediatric Epilepsy 2021; 10(03): 128-134
DOI: 10.1055/s-0041-1723951
Case Report

Managing Status Epilepticus in a Child with Dravet Syndrome: How Difficult It Could Be?

Reza Shervin Badv 
1   Department of Pediatric Neurology, Pediatric Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
,
Azin Ghamari 
2   Growth and Development Research Center, Tehran University of Medical Sciences, Tehran, Iran
,
Mahmoud Reza Ashrafi 
1   Department of Pediatric Neurology, Pediatric Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
,
Mahmoud Mohammadi 
1   Department of Pediatric Neurology, Pediatric Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
,
Reza Azizi Malamiri 
3   Department of Pediatric Neurology, Golestan Medical, Educational and Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
,
Morteza Heidari 
1   Department of Pediatric Neurology, Pediatric Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
2   Growth and Development Research Center, Tehran University of Medical Sciences, Tehran, Iran
4   Department of Pediatric Neurology, Vali-e-Asr Hospital, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran
› Author Affiliations Funding None.
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Abstract

Previously known as severe myoclonic epilepsy of infancy, Dravet syndrome is characterized by febrile or afebrile prolonged hemiconvulsive seizures or generalized status epilepticus in an infant with previously normal development. Immediate management of status epilepticus is critical in these patients. Early control of status epilepticus prevents further brain damage; however, there is no consensus regarding the management of status epilepticus in children with Dravet syndrome, as many conventional antiseizure medications that are recommended in the management of status epilepticus worsen the seizures in these patients. A 2.5-year-old girl child patient was referred due to status epilepticus which was refractory to antiseizure medications. Sodium valproate, nitrazepam, ketogenic diet, intravenous phenytoin, and midazolam continuous infusion were administered. After controlling status epilepticus, the probable diagnosis of Dravet syndrome was proposed and confirmed by a mutation in SCN1A. As previously stated in numerous case reports, phenytoin worsens seizures in patients with Dravet syndrome. Therefore, it seems logical that in every infant with status epilepticus and probable Dravet syndrome, the practicing physician considers administering intravenous valproate or even midazolam continuous infusion instead of intravenous phenytoin.

Keywords

status epilepticus - Dravet syndrome - SCN1A

Ethical Approval

Verbal and written informed consent for publishing the manuscript wad obtained from the parents of the patient.


Authors' Contributions

R.S.B. and M.M, managed the patient and did the necessary treatments. M.R.A. collected the data and R.A. interpreted it. M.H. did the literature review and A.G. wrote the final manuscript.




Publication History

Received: 30 March 2020

Accepted: 23 December 2020

Article published online:
19 February 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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