Sturge-Weber syndrome

History: 33-year-old woman presents with postural headache.

Findings: A midline sagittal T1-weighted MR image demonstrates a well-defined homogeneously hyperintense mass located at the foremen of Monro. A coronal T1-weighted MR image after gadolinium administration reveals that the mass in unchanged in signal intensity. Enlargement of the ventricles is present.

In patients with Sturge-Weber syndrome, X-ray findings in the skull usually show a tram-track pattern of calcification that is caused by calcification in opposing gyri on either side of an intervening dilated sulcus. On CT, curvilinear calcifications in a gyral pattern are often seen, primarily in the occipital and posterior parietal lobes ipsilateral to the facial angioma. Progressive cortical atrophy, ipsilateral calvarial thickening, and enlargement of the paranasal sinuses and mastoid may also be seen. On contrast-enhanced CT, very prominent medullary and subependymal veins may be seen in some cases. Magnetic resonance imaging and angiography can provide detailed information about vascular anomalies.[2,3]

Sturge-Weber syndrome is sporadic neurocutaneous syndrome. Its imaging findings in patients presenting with facial angioma and epilepsy are classic.