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(Stroke. 1996;27:143-146.)
© 1996 American Heart Association, Inc.

Articles

Stroke in Williams Syndrome

Jan B. Wollack, MD, PhD; Marie Kaifer, MD; Marian P. LaMonte, MD MSN Michael Rothman, MD

Correspondence to Jan B. Wollack, MD, PhD, Department of Pediatrics, University of Maryland School of Medicine, Room N5W51 UMH, 22 S Greene St, Baltimore, MD 21201-1595. E-mail jwollack@umabnet.ab.umd.edu.

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 vasculature.

Case Description A 19-year-old girl with Williams syndrome developed an acute-onset hemiparesis. MRI demonstrated an infarct involving the internal capsule and putamen. No stenotic areas were seen on angiography.

Conclusions Stroke should be considered as a possible consequence of Williams syndrome, even in the absence of stenoses of the cerebral vasculature. Comparison of this case with those previously reported in the literature emphasizes the multiplicity of features in Williams syndrome that can contribute to the risk of stroke.

Key Words: cerebral infarction • cerebral ischemia, transient • cerebrovascular disorders • children • genetics

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