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This is a CT brain study, showing confluent, extensive calcification in a cortical / gyriform pattern with serpiginous appearance along the gyri – it involves all of the lobes on the left cerebral hemisphere, but only the frontal lobe on the right side in an asymmetrical pattern. The involved brain parenchyma is atrophied, causing expansion of the intracranial CSF spaces, prominent sulci and ex vacuo dilatation of the ventricles. These radiological features are characteristic of Sturge Weber syndrome. There is no acute haemorrhage.
There is also relative early thickening of the parietal bone on the left side, which may progress eventually to marked left sided calvarial thickening – termed Dyke Davidov Mason syndrome.
I note a prosthesis placed above the left globe, and would correlate with any past ophthalmic surgical history.
Question 1:
What is Sturge Weber syndrome?
This is a congenital neurocutaneous syndrome which causes leptomeningeal angiomatosis; this leads to an impairment of blood flow to the brain parenchyma, causing chronic ischemia. The brain changes observed are the effect of chronic ischemia.
Question 2:
What would you expect to see on contrast enhanced MRI?
The key finding would be leptomeningeal enhancement due to the presence of angiomatosis. The ipsilateral choroid plexus may also hypertrophied and may show relatively greater enhancement.
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