GENERALIZED CHOREA IN AN INFANT WITH SEMILOBAR HOLOPROSENCEPHALY

We report chorea in;an infant with holoprosencephaly. Congenital struc tural brain disease has not been a reported cause of chorea. Cranial m agnetic resonance imaging revealed small, fused frontal lobes with hyp oplastic caudates. Our patient responded to symptomatic treatment with dopamine-depleting agents, It is likely that the mechanism for the ch orea was a dysfunction of the striatum. The differential diagnosis of childhood chorea should include holoprosencephaly.