%0 Journal Article
%T Two South Indian Children with KCNT1-Related Malignant Migrating Focal Seizures of Infancy ¨C Clinical Characteristics and Outcome of Targeted Treatment with Quinidine
%A Abhijit Anil Patil
%A Arun Grace Roy
%A K. P. Vinayan
%J Archive of "Annals of Indian Academy of Neurology".
%D 2019
%R 10.4103/aian.AIAN_229_18
%X KCNT1 gene encodes a sodium-gated potassium channel subunit that plays an important role in regulating excitability in neurons. Quinidine is a partial antagonist of this channel. We report the clinical characteristics of two south Indian children with KCNT1-related epileptic encephalopathy. Both of them had very high seizure burden which were resistant to antiepileptic and dietary therapy. Pharmacological response to quinidine in these children is described. Case 1 had 30% reduction in seizure burden at 20 mg/kg/day and 80% reduction at 36 mg/kg/day; case 2 had 30% reduction at 20 mg/kg/day. Serial electrocardiography was used to monitor the cardiotoxicity. Serum quinidine levels were not measured due to nonavailability. A critical review on the current status of targeted treatment of KCNT1-related epileptic encephalopathies with quinidine is attempted
%K Epileptic encephalopathy
%K KCNT1
%K quinidine
%U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6613401/