Targeted treatment of migrating partial seizures of infancy with quinidine

Ann Neurol. 2014 Sep;76(3):457-61. doi: 10.1002/ana.24229. Epub 2014 Jul 26.


Migrating partial seizures of infancy is an early onset epileptic encephalopathy syndrome that is typically resistant to treatment. The most common cause is a gain of function mutation in the potassium channel KCNT1. The antiarrhythmic drug quinidine is a partial antagonist of KCNT1 and hence may be a candidate drug for treatment of this condition. We report the case of a child with migrating partial seizures of infancy secondary to an activating mutation in KCNT1 treated with quinidine. Treatment with quinidine was correlated with a marked reduction in seizure frequency and improved psychomotor development.

Publication types

  • Case Reports

MeSH terms

  • Anti-Arrhythmia Agents / administration & dosage
  • Anti-Arrhythmia Agents / pharmacology*
  • Child, Preschool
  • Dose-Response Relationship, Drug
  • Electroencephalography
  • Epilepsies, Partial / drug therapy*
  • Epilepsies, Partial / genetics
  • Epilepsies, Partial / physiopathology
  • Exons / genetics
  • Female
  • Humans
  • Mutation, Missense / genetics
  • Nerve Tissue Proteins / antagonists & inhibitors*
  • Nerve Tissue Proteins / genetics
  • Potassium Channels / genetics
  • Potassium Channels, Sodium-Activated
  • Quinidine / administration & dosage
  • Quinidine / pharmacology*
  • Treatment Outcome


  • Anti-Arrhythmia Agents
  • KCNT1 protein, human
  • Nerve Tissue Proteins
  • Potassium Channels
  • Potassium Channels, Sodium-Activated
  • Quinidine