IMPORTANT: We currently have a number of bugs related to image cropping and are actively trying to resolve them. In the meantime, we have disabled cropping. Apologies for any inconvenience. Stay informed: radiopaedia.org/chat

Sturge-Weber syndrome

Case contributed by Abdallah Al Khateeb
Diagnosis certain

Presentation

Refractory seizures. Left side upper face port wine stain.

Patient Data

Age: 1 year old
Gender: Male

NECT brain

ct

Left side parietal and occipital lobes flowing subcortical white matter calcifications.

There is no gross parenchymal volume loss.

Enhanced brain MRI

mri

There are subcortical areas of low T2 signal at the left parieto-temporo-occipital lobes. The subarachnoid spaces at the same areas are prominent due to mild parenchymal volume loss along with abnormal high signal obtained on FLAIR images.

Post-Gd administration, there is intense leptomeningeal enhancement at the same area.

Case Discussion

Sturge-Weber (SW) syndrome or encephalotrigeminal angiomatosis is caused by a leptomeningeal hemangioma that leads to progressive unilateral brain ischemia and subsequent atrophy.

As in this case, SW characteristically presents with an ipsilateral facial port-wine stain at the V1 trigeminal nerve distribution in a child with seizures. The diagnosis was achieved with those clinical and imaging features. 

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.