Effectiveness of lamotrigine in children with paroxysmal kinesigenic choreoathetosis

Paroxysmal kinesigenic choreoathetosis (PKC) is a rare movement disorder, c haracterized by recurrent, brief involuntary dystonic attacks that are prov oked 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 h istory of three children (6, 8, and 10 years of age) with idiopathic PKC su ccessfully treated with low doses of lamotrigine, an AED acting primarily v ia neuronal voltage-sensitive sodium channels.