Sturge-Weber syndrome (SWS) is a sporadic phakomatoses and is characterized by angiomatosis of skin, eye and meninges. . Patients present with epilepsy and developmental delay since childhood. Clinical examination reveals reddish discoloration of face (referred to as port wine stain), sclera and limbs, usually on one side of body.

Imaging findings of SWS are typical. Our patient was a 30-year old lady who presented with recurrent seizures since childhood. Her developmental milestones and intelligence were within normal limits. MRI brain and MR Angiogram were done, which are shown below.

T1W MRI showing right cerebral hemiatrophy (yellow) and calvarial thickening on the same side (red)
FLAIR MRI Brain showing right cerebral hemiatrophy (yellow) and prominent (enlarged) choroid plexus on the right side (red)
T2W MRI showing subcortical calcification on the right side(tram track appearance)
T2W MRI showing subcortical calcifications (red) and calvarial thickening (yellow arrows) on the right side
MR Angiogram showing narrowing of right internal carotid and middle cerebral arteries

Above MRI images have shown the following findings

  1. Hemiatrophy on right side mainly involving right parietal and occipital lobes,
  2. Thickening of calvarium on the right side
  3. Enlargement of choroid plexus in the right lateral ventricle
  4. Subcortical calcifications in right parietal and occipital regions (tram track appearance)
  5. Moderate stenosis of right internal carotid and middle cerebral arteries.

The patient was started on anti-epileptic drugs, with which her seizures were adequately controlled.

Published by Dr Sudhir Kumar MD (Medicine) DM (Neurology)

I am a doctor with 25 years experience. I have worked as a neurologist for about 20 years. Educating public and healthcare professionals is very dear to me. This is possible due to my interactions with thousands of patients and their caregivers. I salute the patients who suffer and it is our duty to minimise suffering by preventing diseases and ensuring prompt diagnosis and treatment of those already affected.

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