Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder featuring a facial port-wine stain (capillary malformation), leptomeningeal angiomatosis (pial vascular malformation over the brain), and eye involvement such as glaucoma.

MRI with contrast is the best imaging test to evaluate brain involvement. Changes are usually on one side (same side as the facial stain) but can affect both sides in some cases.

Main MRI Features

• Leptomeningeal angiomatosis: Hallmark finding. After gadolinium contrast on T1 images, you see serpiginous or sheet-like enhancement along the brain surface, often over the parieto-occipital region. Enhancement may fade in older patients or advanced (“burnt-out”) disease.
• Brain atrophy: Shrinking of the affected hemisphere with larger sulci and ventricles, more obvious over time.
• Accelerated myelination: In infants, the white matter under the angioma appears darker on T2 due to faster myelination from altered blood flow.
• Calcifications: Gyriform “tram-track” pattern (parallel lines along the cortex). These show as dark signal loss on susceptibility-weighted imaging (SWI) or GRE sequences. CT shows them better early on.
• Venous abnormalities: Prominent deep veins, enlarged choroid plexus on the same side, and flow voids on T2. SWI is excellent for seeing these vascular changes.
• Other signs: Increased T2/FLAIR signal in white matter (from gliosis or congestion), possible cortical dysplasia, and restricted diffusion if there are stroke-like episodes.

In infants: Changes can be subtle at first — look for accelerated myelination and venous issues. Contrast is very important. In older patients: More atrophy, calcifications (dark on SWI), and less enhancement.

Visual Examples of Typical Findings

CT (left) showing gyriform calcifications and atrophy; MRI (right) for comparison:

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T2-weighted MRI showing hemispheric atrophy and white matter changes (A vs B examples):

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Multi-sequence MRI panels highlighting enhancement, vascular structures, and cortical changes:

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CT and MRI panel showing calcifications, atrophy, enhancement, and choroid plexus enlargement:

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MRI diagnosis should be combined with clinical exam (port-wine stain), eye check, and sometimes EEG. Early imaging helps guide management for seizures, glaucoma, and development. This is general educational information — always consult a neurologist or neuroradiologist for specific cases.