Sturge-Weber Syndrome

Question 1

Key Clinical features of Sturge-Weber syndrome (encephalotrigeminal angiomatosis)?

Answer 1

(1) Hallmark feature is a facial cutaneous venous malformation, known as nevus flammeus or port-wine stain (which is sometimes absent). It is unilateral and involves
at least the ophthalmic division of the trigeminal nerve.
(2) Defining feature is the cortical leptomeningeal angioma => Hemiplegia contralateral to the leptomeningeal
angioma due to progressive ischemia of the underlying cortex
(3) Epilepsy.
(4) Variable degrees of mental retardation.
(5) Choroidal vascular malformations, which are on the same side as the facial capillary malformation in 70% of patients with SWS.

Question 2

Imaging features of Sturge Weber Syndrome?

Answer 2

(1) Dystrophic gyriform calcifications related to
chronic ischemia results in the so-called tram-track
sign visible on CT or even radiographs
(2) Enhancing leptomeningeal angiomas (seen over the pial surface) and associated underlying brain volume loss in the posterior left cerebral hemisphere, overlying
calvarial expansion and asymmetric prominence of the
left frontal sinus.

Question 2

Imaging features of Sturge Weber Syndrome?

Answer 2

(1) Dystrophic gyriform calcifications related to
chronic ischemia results in the so-called tram-track
sign visible on CT or even radiographs
(2) Enhancing leptomeningeal angiomas (seen over the pial surface) and associated underlying brain volume loss in the posterior left cerebral hemisphere, overlying
calvarial expansion and asymmetric prominence of the
left frontal sinus.