Annals of Neurology 2014-12-01

Pediatric super-refractory status epilepticus treated with allopregnanolone.

Eileen Broomall, JoAnne E Natale, Michele Grimason, Joshua Goldstein, Craig M Smith, Celia Chang, Stephen Kanes, Michael A Rogawski, Mark S Wainwright

Index: Ann. Neurol. 76(6) , 911-5, (2014)

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Abstract

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.© 2014 American Neurological Association.