A lateral X-ray of the skull showed the typical gyriform calciﬁcation, “tram-track” calciﬁcation, and enlargement of the paranasal sinuses (Figure 1). A computed tomography (CT) scan subsequently revealed extensive gyriform calciﬁcation in the right parieto-occipital region with ipsilateral cerebral cortical atrophy. Calciﬁc foci were also noted in the left cerebellar hemisphere (Figures 2 and 3).
Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a sporadic phakomatosis of unknown etiology that may be characterized by1:
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.