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Genetic and Rare Diseases Information Center (GARD)

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Sturge-Weber syndrome

Other Names for this Disease
  • Encephalofacial angiomatosis
  • Encephalotrigeminal angiomatosis
  • Fourth phacomatosis
  • Leptomeningeal angiomatosis
  • Meningeal capillary angiomatosis
More Names
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Your Question

How does Sturge-Weber syndrome affect adults? I'm a 43-year-old person who has this disorder and had my birthmark removed at age 4.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What information is available on adults with Struge-Weber syndrome?

Limited information is available on adults with Sturge-Weber syndrome. In 1995, Sujansky and Conradi reviewed the outcome of 52 adults with this syndrome. The ages of affected individuals ranged from 18 to 63 years. Among these individuals, 83 percent had seizures. The age at which seizures appeared varied from birth to 23 years. With medication, 11 participants had full control of their seizures, 20 had decreased seizures, and 10 had no improvement in their seizures. The level of seizure control was not known for the remaining participants. In those people who had developed seizures, 43 percent had developmental delay and 71 percent required special education. Developmental delay and the need for special education was not reported in people without seizures. Emotional and behavioral problems were present in 85 percent of people with seizures and 58 percent of those without seizures.

Headache occurred in 28 of 45 (62%) patients, the onset ranging from early childhood to age 38 years. The headache frequency could be determined in 23 patients: headaches occured daily in 9 patients, 1-4 times per week in 4 people, 1-2 times per month in 6 patients, and were rare in 4 individuals. 

In this study, 60 percent of the participants had glaucoma. The age of onset of glaucoma varied from birth to 41 years. In addition, 65 percent had a neurologic deficit, including stroke, paralysis, spasticity, or weakness. Ten people had children, and of the 20 liveborn children; 17 were healthy, and tuberous sclerosis, a cafe-au-lait spot, and a "birthmark" were found in 1 child each.
Last updated: 4/13/2010