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Epilepsy
Epilepsy refers to the occurrence of repeated seizures not triggered by a specific
acute stimulus such as fever or head trauma. Seizures eventually develop in
about 80% of patients with SWS, most commonly in the first year or two of life.
Glaucoma
Glaucoma is vision loss resulting usually
from increased pressure in the eye. Glaucoma occurs in 30-70% of patients with
SWS.
Leptomeningeal angioma
Leptomeningeal angioma is the presence of numerous
abnormally dilated blood vessels on the surface of the brain. These abnormally
formed blood vessels frequently result in abnormal blood flow (perfusion) to
the involved side of the brain. Impaired brain perfusion, in turn, can result
in brain injury, seizures, stroke-like episodes and developmental delays.
Port-wine Stain
Port-wine stains (PWS)
are the most common kind of abnormality of blood vessel development and involves
dilated capillaries in the skin. PWS may occur anywhere on the body, however
the risk of brain or eye involvement is very low if the PWS is located anywhere
other than the upper face.
Sturge-Weber
Syndrome
Sturge-Weber Syndrome (SWS) is a disorder involving the association
of a facial port-wine stain with glaucoma and abnormal blood vessels called
a leptomeningeal angioma on the surface of the brain. The facial port-wine stain
is present from birth and when it ocurrs on the forehead and/or upper eyelid
(ophthalmic distribution) is associated with an increased risk (10-20%) of the
full syndrome. SWS can involve one side of the head (unilateral) or both sides
(bilateral). Infants with Sturge-Weber Syndrome are at increased risk of developing,
epilepsy, developmental delays, mental retardation, weakness on a side of the
body (hemiparesis), and vision loss. |
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