Sturge-Weber Syndrome

What is Sturge-Weber Syndrome?

Sturge-Weber syndrome is a neurological disorder indicated at birth by seizures accompanied by a large port-wine stain birthmark on the forehead and upper eyelid of one side of the face.  The birthmark can vary in color from light pink to deep purple and is caused by an overabundance of capillaries around the trigeminal nerve just beneath the surface of the face.

Symptoms of Sturge-Weber Syndrome

Some common symptoms of Sturge-Weber Syndrome are:

• Seizures
  • Seizures begin in infancy and may worsen with age.
  • Convulsions usually happen on the side of the body opposite the birthmark.
• White part of eye may be a reddish color.

What Causes Sturge-Weber Syndrome?

The exact cause of Sturge-Weber Syndrome is unknown.

Types of Sturge-Weber Syndrome

There are 4 main types of Sturge-Weber Syndrome

1. Type 1
   • Type 1 is the most common type.
   • Characterized by facial and brain angiomas and may involve glaucoma.
     • An angioma is a benign growth that consists of small blood vessels.
   • Seizures usually occur within the first year of life, as a result of the brain involvement.
   • The white portion of the eye may be a reddish color.
   • Mental and physical developmental delays.
2. Type 2
   • This type involves a facial angioma and the possibility of glaucoma.
3. Type 3
   • Leptomeningeal angioma (numerous abnormally dilated blood vessels on the surface of the brain).
   • No facial involvement and usually no development of glaucoma.
   • Identified through brain scans.
   • Commonly referred to as forme fruste.

Can Sturge-Weber Syndrome be Treated?

Yes. The aim of treatment is to relieve the symptoms. Laser treatment may be used to lighten or remove the birthmark.   Anticonvulsant medications may be used to control seizures.  Surgery may be performed on more serious cases of glaucoma. 

Complications of Sturge Weber-Syndrome

One of the main complications of Sturge-Weber syndrome is the loss of nerve cells and calcification of tissue in the cerebral cortex of the brain on the same side of the body as the birthmark. Other complications of Sturge-Weber syndrome include: developmental delays, mental retardation, headaches, and glaucoma.

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