NOONAN SYNDROME AND MOYAMOYA

We report a patient with Noonan syndrome and asymptomatic cardiac dise ase (supravalvular aortic stenosis and pulmonary valvular stenosis) wh o had frequent transient ischemic attacks, Bilateral moyamoya was evid ent; in addition, he manifested activated protein C resistance and was heterozygous for the factor V Leiden mutation, Anticoagulation abolis hed his episodes and, despite extensive cerebrovascular disease, he ha s no permanent neurologic deficits, The association between Noonan syn drome and moyamoya has not previously been described, Disruption of va scular development in prenatal life mag have resulted in both cardiac and cerebrovascular disease in this child. (C) 1997 by Elsevier Scienc e Inc.