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Pediatric super-refractory status epilepticus treated with allopregnanolone

Eileen Broomall1, JoAnne E Natale, Michele Grimason

  • 1Ruth D. and Ken M. Davee Pediatric Neurocritical Care Program, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL.

Annals of Neurology
|November 4, 2014
PubMed

Insights

Super-refractory status epilepticus (SRSE) is a severe condition. Allopregnanolone successfully treated SRSE in two pediatric patients, resolving seizures and allowing anesthetic weaning.

Area of Science:

  • Neuroscience
  • Pediatric Neurology
  • Pharmacology

Background:

  • Super-refractory status epilepticus (SRSE) is a critical neurological emergency.
  • Treatment resistance in SRSE is linked to synaptic GABAA receptor internalization.
  • GABAA receptor dysfunction contributes to persistent seizures.

Observation:

  • Two pediatric patients with SRSE were treated with the neurosteroid allopregnanolone.
  • Allopregnanolone modulates GABAA receptors, acting as a positive allosteric modulator.
  • This neurosteroid targets both synaptic and extrasynaptic GABAA receptors.

Findings:

  • Allopregnanolone administration led to the resolution of status epilepticus in both pediatric cases.
  • Patients were able to be weaned off general anesthetic infusions following treatment.
  • This represents the first documented use of allopregnanolone for pediatric SRSE.

Implications:

  • Allopregnanolone shows promise as a novel therapeutic agent for refractory epilepsy syndromes.
  • Targeting GABAA receptors with neurosteroids may offer a new strategy for managing severe seizures.
  • Further research is warranted to explore allopregnanolone's efficacy and safety in pediatric epilepsy.

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