Premium
Targeted treatment of migrating partial seizures of infancy with quinidine
Author(s) -
Bearden David,
Strong Alanna,
Ehnot Jessica,
DiGiovine Marissa,
Dlugos Dennis,
Goldberg Ethan M.
Publication year - 2014
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.24229
Subject(s) - quinidine , partial seizures , psychomotor learning , medicine , antagonist , partial agonist , dravet syndrome , epilepsy , anesthesia , pediatrics , pharmacology , psychiatry , receptor , cognition
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. Ann Neurol 2014;76:457–461