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Sturge-Weber Syndrome Variant With Atypical Intracranial Findings

Case Report

Department of Neurosciences Medical University of South Carolina Charleston, South Carolina

Joel K. Cure, MD

Department of Radiology Neuroradiology Section University of Alabama Medical Center Birmingham, Alabama

Kenton R. Holden, MD

Department of Neurosciences Medical University of South Carolina Charleston, South Carolina Greenwood Genetic Center Greenwood, South Carolina, kholden{at}ggc.org

Sturge-Weber syndrome is characterized by a facial port-wine nevus, leptomeningeal angiomatosis, and glaucoma; it is commonly complicated by epilepsy and hemiparesis. We present a patient with a head and neck port-wine nevus, glaucoma, abnormalities of the intracranial deep veins, and untreated communicating hydrocephalus. The patient lacks any radiologic or clinical evidence of cerebral leptomeningeal angiomatosis. Considering that intracranial venous anomalies also are likely compatible with the embryologic explanation of Sturge-Weber syndrome, this child can serve as an unusual example of Sturge-Weber syndrome type II. ( J Child Neurol 2006;21:155—157; DOI 10.2310/7010.2006.00026).

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