Sturge-Weber syndrome

Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis, is a congenital thingy that is characterized by the triad:

1. Trigeminal distribution unilateral capillary malformations (flame nevus or port-wine stain).
2. Leptomeningeal vascular malformations (angiomatosis).

• • Results in intracranial calcifications.
  • Associated with neurologic signs.

1. Vascular malformations of the choroid plexus associated with glaucoma.

Associations:

• Intractable seizures.
• Mental retardation.
• Recurrent stroke-like episodes.

Prevalence

• 1 in 50 000 live births.

See also

• Neurocutaneous syndromes.