Summary: A 10-year-old mentally disabled boy presented to the emergency room
after a fall. Lateral X-rays of the skull were obtained (Figure 1). Upon
questioning the parents, his history of long-standing epilepsy was
revealed. The child also had a “port-wine stain” on the right side of
his face along the distribution of the trigeminal nerve.
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.
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
- 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.
- 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.
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