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.