Sturge-Weber syndrome

Case contributed by Bahman Rasuli
Diagnosis almost certain

Presentation

Seizure and right-sided body weakness.

Patient Data

Age: 40 years
Gender: Female
mri
  • abnormal low T2 signal at the subcortical white matter on the left side
  • prominent abnormal vascular channels traversing the white matter of the left hemisphere, along with abnormally enlarged signal void deep venous drainage
  • mild volume loss of the left cerebrum
  • diffuse leptomeningeal enhancement of the left hemisphere
  • disproportionately large left frontal sinus compared to the right

Case Discussion

Findings are consistent with Sturge-Weber syndrome which is a rare congenital vascular disorder that leads to progressive unilateral brain ischemia and subsequent atrophy.

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