Sturge-Weber syndrome

Case contributed by Dr Ian Bickle

Presentation

Female in 30's presents to A and E following a seizure.

Patient Data

Age: 30 years
CT

Gyriform calcification in the high left frontal lobe, with the characteristic 'tram-track' calcification.

Associated parenchyma volume loss.

Disproportionately large left frontal sinus compared to the right.

Choriod plexus symmeterical.

Case Discussion

Sturge Weber syndrome is one form of phakamatosis, characterized by a pial hemangioma in the brain with an associated facial hemangioma.

The leptomeningeal hemangioma results in a vascular steal affecting the subjacent cortex and white matter producing localized ischemia, hence the associated parenchymal volume loss.

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