Sturge-Weber syndrome

Case contributed by Zachary Jon Perbohner

Presentation

Seizures

Patient Data

Age: 50 years
Gender: Male
ct

Extensive gyral calcification in the right occipital lobe with mild atrophic changes in the right cerebral hemisphere. Atrophic changes are most prominent in the right occipital lobe.

mri

There is right occipital cerebral atrophy with ex vacuo dilation of the right lateral ventricle, associated with extensive gyriform calcifications and parietoccipital leptomeningeal contrast enhancement.  

The right occipital gyriform calcifications demonstrate high T1 signal intensity, reflects emulsion form of calcification. The calcifications are also noted in the head CT.

There is associated volume loss of the right hippocampal structures posteriorly.

Case Discussion

Sturge-Weber syndrome is a rare neurocutaneous syndrome which can lead to neurovascular complications resulting in anoxia.  On imaging, it classically presents as volume loss, gyriform calcifications, and leptomeningeal enhancement.

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