Generalized Chorea Due to Secondary Polycythemia Responding to Phlebotomy

Abstract

Dear Editor, Chorea can be seen in association with polycythemia rubra vera (PV) or primary polycythemia, especially in elderly women. However, chorea due to secondary polycythemia is rare, and we report a case in an adolescent boy due to congenital cyanotic heart disease and its rewarding response to phlebotomy.A 15-year-old boy, born of non-consanguineous parentage, with cyanotic spells since 6 months of age, was found to have ventricular septal defect and pulmonary atresia for which he underwent placement of a bidirectional Glenn shunt and left pulmonary artery plasty at 12 years of age. He presented to us with a 2-month history of abnormal fidgety movements and difficulties with feeding and holding objects. There was no previous history of rheumatic fever nor were there any recent infections or drug exposure. Family history was negative for chorea. Physical examination showed facial dysmorphism in the form of hypertelorism, a low hair line, low-set ears and webbed neck. He had central cyanosis and pandigital clubbing, with a resting oxygen saturation of 75%. He was noted to have generalized chorea of all four limbs (right > left), facial chorea, variable tone in upper and lower limbs (segment 1, Supplementary Video 1 in the online-only Data Supplement) and a choreic gait. Muscle power and reflexes were normal, and there were no cerebellar signs. Routine blood evaluation showed a hemoglobin level of 22 g/dL with a hematocrit of 70%. Serum calcium, phosphorus and intact parathyroid hormone levels were within normal limits. Results of serum antistreptolysin O titer, vascu-

Monochorea after acute contralateral pontine infarction: A case report

“…Chorea can be induced by diverse factors, [ 5 ] and stroke is the most common cause of non-genetic chorea. [ 1 ] Although chorea is a rare post-stroke complication, it is the most common post-stroke phenotype among abnormal movements.…”