Sturge-Weber syndrome

By Paresh Desai, MD, Arash Deep Singh, MD, and Ashutosh Dessai, DMRD, Resident Radiologists, Goa Medical College, Bambolim, Goa, India

Sturge-Weber syndrome


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:

  • “Port wine” vascular nevus flammeus in the trigeminal nerve distribution;
  • Leptomeningeal venous angiomatosis;
  • Seizures, dementia, hemiplegia;
  • Hemianopsia, bupthalmos, and glaucoma;
  • Possible associated cleral and choroidal angiomata;
  • Possible associated angio-osteohypertrophy of Klippel-Trenaunay syndrome; and/or
  • Intracranial calcification, a common radiologic manifestation.

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.


  1. Elster AD, Chen MY. MR imaging of Sturge-Weber syndrome: Role of gadopentetate dimeglumine and gradient-echo techniques. AJNR Am J Neuroradiol.1990;11:685-689.
  2. Benedikt RA, Brown DC, Walker R, et al. Sturge-Weber syndrome: Cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol. 1993;14:409-415.
  3. Martí-Bonmatí L, Menor F, Poyatos C, Cortina H. Diagnosis of Sturge-Weber syndrome: Comparison of the efficacy of CT and MR imaging in 14 cases. AJR Am J Roentgenol. 1992;158:867-871.
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Sturge-Weber syndrome.  Appl Radiol. 

June 23, 2011

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