Effectiveness of lamotrigine in children with paroxysmal kinesigenic choreoathetosis

Paroxysmal kinesigenic choreoathetosis (PKC) is a rare movement disorder, characterized by recurrent, brief involuntary dystonic attacks that are provoked by sudden movements. Pathophysiology is uncertain, but a channelopathy is discussed. Treatment recommendations favour antiepileptic drugs (AEDs) acting on voltage-gated neuronal ion channels. This report summarizes the history of three children (6, 8, and 10 years of age) with idiopathic PKC successfully treated with low doses of lamotrigine, an AED acting primarily via neuronal voltage-sensitive sodium channels.