Pediatric super-refractory status epilepticus treated with allopregnanolone

• Published in: Annals of neurology Vol. 76; no. 6; pp. 911 - 915
• Main Authors: Broomall, Eileen, Natale, JoAnne E., Grimason, Michele, Goldstein, Joshua, Smith, Craig M., Chang, Celia, Kanes, Stephen, Rogawski, Michael A., Wainwright, Mark S.
• Format: Journal Article
• Language: English
• Published: United States Blackwell Publishing Ltd 01-12-2014; Wiley Subscription Services, Inc
• Subjects: Child; Child, Preschool; Drug therapy; Epilepsy; Female; Humans; Pregnanolone - blood; Pregnanolone - therapeutic use; Status Epilepticus - blood; Status Epilepticus - diagnosis; Status Epilepticus - drug therapy; Treatment Outcome
• ISSN: 0364-5134; 1531-8249
• DOI: 10.1002/ana.24295

Super‐refractory status epilepticus is a life‐threatening condition. Resistance to benzodiazepine and barbiturate treatment for this disorder is thought to be due to internalization of synaptic γ‐aminobutyric acid (GABA)A receptors, and withdrawal of benzodiazepines and barbiturates during treatment often triggers seizure recurrence. The neurosteroid allopregnanolone acts as a positive allosteric modulator of synaptic and extrasynaptic GABAA receptors. Here we describe the use of allopregnanolone in 2 pediatric patients with super‐refractory status epilepticus. This treatment allowed the general anesthetic infusions to be weaned with resolution of status epilepticus. This is the first report of allopregnanolone use to treat status epilepticus in children. Ann Neurol 2014;76:911–915