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

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Epilepsy of infancy with migrating focal seizures (EIMFS) (n=1). Intervention: Quinidine.

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

doi:10.1016/j.curtheres.2019.02.002

January 1, 2019Current Therapeutic ResearchOpen Access

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

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Key result

Quinidine partially reduces seizures after phenobarbital withdrawal reverses induced metabolism and restores drug levels.

n=1

Study Design

Type

Case Report (n=1)

Structured PICO

P

Population

A 1-year-old girl with epilepsy of infancy with migrating focal seizures and two KCNT1 mutations treated with quinidine and phenobarbital over 50 weeks.

I

Intervention

Quinidine (titrated up to 72 mg/kg/d in 4 divided doses) with and without concurrent phenobarbital (initially 9.4 mg/kg/d, later weaned and discontinued).

O

Outcome

Seizure frequency and serum quinidine levels

Concomitant use of the enzyme-inducer phenobarbital can lead to undetectable serum quinidine levels, complicating the treatment of KCNT1-positive epilepsy and requiring careful monitoring of drug interactions and QTc prolongation.

Limitations

Single case report
Exact phenobarbital concentrations over time were not available
Erratic and inconsistent seizure frequency made it difficult to correlate quinidine dose with seizure control
Difficult to quantify quinidine's exact effect on seizure frequency due to erratic baseline and concurrent medications

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Implication

May reduce seizures in KCNT1 epilepsy; leaves open need for randomized confirmation.

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.

Discussion

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Cite This Study

Passey et al. (2019) conducted a case report in Epilepsy of infancy with migrating focal seizures (EIMFS) (n=1). Quinidine was evaluated. In a 1-year-old girl with EIMFS and 2 KCNT1 mutations, concurrent phenobarbital induced quinidine metabolism leading to undetectable levels, but upon phenobarbital withdrawal, quinidine partially decreased seizure frequency.

synapsesocial.com/papers/6aa0c7d9c0e1046e90f7a1ac https://doi.org/10.1016/j.curtheres.2019.02.002

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

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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