Sturge-Weber syndrome

Case contributed by Dr Abdallah Khateeb

Presentation

Refractory seizures. Left side upper face port wine stain.

Patient Data

Age: 1 year old
Gender: Male
CT

NECT brain

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

There is no gross parenchymal volume loss.

MRI

Enhanced brain 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) 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. 

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Case information

rID: 43320
Case created: 4th Mar 2016
Last edited: 30th Jul 2017
Inclusion in quiz mode: Included

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