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Sturge-Weber syndrome

Case contributed by Ian Bickle
Diagnosis almost certain

Presentation

Known epileptic. Headache. Sensory symptoms.

Patient Data

Age: 45 years
Gender: Female
ct

Tram-track sulcal calcification in the left high frontal lobe. Small amount of associated parenchymal volume loss.

Mild enlargement of the left frontal sinus.

Normal ventricular system.

mri

Tram-track subcortical calcification with signal drop-out on SWI in the high left frontal convexity. Adjacent parenchymal volume loss in the left frontal lobe.

No other intracranial abnormality.

Mild enlargement of the left frontal sinus.
 

Case Discussion

Sturge-Weber syndrome if one of the well recognized phakamatoses clinically characterized by facial port wine stains and epilepsy.

The intracranial appearances are distinct in particular tram-track calcification on CT and MRI.

Other features include:

  • regional paranasal sinus enlargement
  • focal atrophic change
  • choroid plexus hypertrophy

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