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Sturge-Weber syndrome is characterized by a facial cutaneous nevus (port-wine stain) and a leptomeningeal angioma, often found ipsilateral to the facial lesion. Epileptic seizures, mental retardation, and focal neurological deficits are the primary neurologic abnormalities of Sturge-Weber syndrome. In a series of 52 adults with Sturge-Weber syndrome, 65% had neurologic deficits including stroke, hemiparesis, spasticity, and/or weakness. Neuroimaging, electroencephalography, and functional testing with Positron emission tomography (PET) and Single-photon emission computed tomography (SPECT) may also help to define the extent of the intracranial lesion for possible epilepsy surgery. Although gyral calcification is a classic feature of Sturge-Weber syndrome, this "tram track" appearance is not always present. Bilateral calcification is common. Calcification often becomes more apparent as the patient becomes older but is sometimes already present at birth. Daily aspirin has been tried in an effort to prevent recurrent vascular thrombosis that may cause neurologic deterioration.
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