Generalized chorea in an infant with semilobar holoprosencephaly.
We report chorea in an infant with holoprosencephaly. Congenital structural brain disease has not been a reported cause of chorea. Cranial magnetic resonance imaging revealed small, fused frontal lobes with hypoplastic caudates. Our patient responded to symptomatic treatment with dopamine-depleting agents. It is likely that the mechanism for the chorea was a dysfunction of the striatum. The differential diagnosis of childhood chorea should include holoprosencephaly.
Louis
E D
ED
Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York, USA.
Lynch
T
T
Cargan
A L
AL
Fahn
S
S
D002675:Child, Preschool; D002819:Chorea / Q000150:complications* / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D016142:Holoprosencephaly / Q000150:complications*; D006801:Humans; D008279:Magnetic Resonance Imaging