Stroke after cardiac surgery in a patient with Smith-Magenis syndrome.

Patients with Smith-Magenis syndrome have a high incidence of congenital heart disease that requires open-heart surgery. These patients may have gene deletions that affect cholesterol homeostasis, although no previous association has been made with premature atherosclerosis. Herein, we report a case of such a patient, who experienced a stroke after cardiac surgery because of what we believe to be premature intracerebral atherosclerosis.