Treatment of Chorea in Childhood

Chorea is a movement disorder characterized by ongoing random-appearing sequences of discrete involuntary movements or movement fragments. Chorea results from dysfunction of the complex neuronal networks that interconnect the basal ganglia, thalamus, and related frontal lobe cortical areas. The complexity of basal ganglia circuitry and vulnerability of those circuits to injury explains why chorea results from a wide variety of conditions. Because etiology-specific treatments or effective symptomatic treatments are available for causes of chorea, defining the underlying disease is important. The treatment of chorea can be considered in three main categories: (1) terminating or modifying exposure to the causative agent, (2) symptomatic treatment of chorea, and (3) treatment targeting the underlying etiology. Symptomatic treatment decision of chorea should be based on the functional impact on the child caused by chorea itself. There have been no reported randomized, placebo-controlled trials of symptomatic treatment for chorea in childhood. Thus the recommendations are based on clinical experience, case reports, expert opinions, and small comparative studies. Better knowledge of mechanisms underlying childhood chorea will provide more etiology-based treatments in the future.