clinical features

Last reviewed 01/2018

Sturge-Weber syndrome is characterised by:

  • a capillary naevus or port wine stain which is unilateral and usually over the forehead and eyelid conforming to the 1st or the 1st and 2nd divisions of the trigeminal nerve
  • epilepsy occurs in 90%, usually presenting in infancy
  • developmental delay - particularly of speech - and behavioural disorders occur in 50%
  • hemiparesis and homonymous hemianopia occur in 30%
  • there may be glaucoma in the affected eye