Concurrent Quinidine and Phenobarbital in the Treatment of a Patient with 2 KCNT1 Mutations

Camerin C Passey; John Erramouspe; Peter Castellanos; Elizabeth C O'Donnell; David M Denton

doi:10.1016/j.curtheres.2019.02.002

Concurrent Quinidine and Phenobarbital in the Treatment of a Patient with 2 KCNT1 Mutations

Curr Ther Res Clin Exp. 2019 Feb 28:90:106-108. doi: 10.1016/j.curtheres.2019.02.002. eCollection 2019.

Authors

Camerin C Passey¹, John Erramouspe¹, Peter Castellanos², Elizabeth C O'Donnell³, David M Denton⁴

Affiliations

¹ Idaho State University College of Pharmacy, Pocatello, Idaho.
² Grand Teton Child Neurology, Idaho Falls, Idaho.
³ Neonatal Intensive Care Unit, Portneuf Medical Center, Pocatello, Idaho.
⁴ Pocatello Children's Clinic, Pocatello, Idaho.

Abstract

Epilepsy of infancy with migrating focal seizures is a devastating pediatric neurologic disorder that often results in treatment-resistant seizure activity and developmental delay. The condition has been associated with mutations in the KCNT1 gene that cause a gain of function in neuronal sodium-activated potassium channels. Quinidine has been shown to reverse this gain of function and has recently been used to reduce seizure activity in patients with these mutations. We report the case of an infant with 2 KCNT1 mutations who experienced minor relief with quinidine and discuss the drug's important interaction with phenobarbital.

Keywords: KCNT1; Pediatrics; Phenobarbital; Quinidine; Seizures.