STROKE IN WILLIAMS-SYNDROME

Background Williams syndrome is a genetic disorder characterized by a high incidence of heart disease, arterial stenosis, and hypertension. Despite these features, cerebrovascular accidents have been described only recently and only in association with stenoses of the cerebral va sculature. Case Description A 19-year-old girl with Williams syndrome developed an acute-onset hemiparesis. MRI demonstrated an infarct invo lving the internal capsule and putamen. No stenotic areas were seen on angiography. Conclusions Stroke should be considered as a possible co nsequence of Williams syndrome, even in the absence of stenoses of the cerebral vasculature. Comparison of this case with those previously r eported in the literature emphasizes the multiplicity of features in W illiams syndrome that can contribute to the risk of stroke.