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| Definition: |
A rare and sporadically occurring disease without a recognized hereditary component. It consists of multiple vascular lesions (angiomas) involving the skin of the face, mucus membranes, and covering of the brain (meninges).
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| Alternative Names: |
Encephalotrigeminal angiomatosis; Sturge-Weber-Dimitri syndrome
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| Causes, incidence, and risk factors: |
| The cause of Sturge-Weber is unknown. Because of its spotty appearance in the population, specific risk factors are not yet recognized. |
| Signs and tests: |
| A skull X-ray shows damage to the inner layer of the skull caused by the vascular lesion and intracranial calcifications. Increased pressure in the eyeball or glaucoma is found in up to 60% of patients. MRI or CT scans of the skull may show benign tumors of the lining of the brain (dural angiomas) or of the choroid plexus. |
| Treatment: |
Treatment is based on the findings:
- Seizures are treated with antiseizure medications such as phenobarbital, Dilantin, or carbamazepine. Occasionally, surgical excision is done in hopes of preventing seizures that are difficult to control with medications.
- Paralysis or weakness is treated with appropriate physical therapy.
- Learning disabilities require appropriate school intervention.
- Glaucoma requires early intraocular pressure testing and treatment to prevent blindness.
- Port-wine stain treatment varies. Laser therapy has proved effective at destroying the vascular lesion without damaging the normal skin.
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| Support Groups: |
| Sturge-Weber Foundation |
| Expectations (prognosis): |
| Most cases of Sturge-Weber are fairly mild. Intervention is indicated as noted in the treatment section. For mild cases life expectancy is normal. Surgical treatment of the intracranial lesion is sometimes successful in reducing symptoms. |
| Calling your health care provider: |
| All birthmarks, including a port-wine stain, should be evaluated by the health care provider. Convulsions, visual problems, paralysis, and change in alertness or mental state may indicate involvement of the coverings of the brain. These symptoms should be evaluated promptly. |
| Prevention: |
| There are no known preventive measures. |
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Review Date: 12/3/2001
Reviewed By: David G. Brooks, M.D., Ph.D., Division of Medical Genetics, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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