EPILEPSY AND ITS SPECIAL FORMS/EPILEPSY SYNDROMES: STURGE-WEBER SYNDROME
Sturge-Weber syndrome is not inherited. It is an abnormality of blood vessel formation occurring early in the development of the fetal face and brain. Children with this disease are born with a red birthmark (“port wine stain”) on the forehead and, at times, extending over the eye and lower face. Birthmarks that do not appear on the forehead are not associated with Sturge-Weber syndrome.
In this disease, blood vessel malformation causes atrophy (shrinking) of the underlying brain, leading to seizures and paralysis of the opposite side of the body. The seizures that accompany this shrinkage are usually one-sided and may be difficult to control with medication. Children with Sturge-Weber syndrome may be of normal intelligence but are often progressively handicapped by mental retardation, by the seizures, and by paralysis. Surgery to remove the affected portion of the brain is sometimes indicated and should be done early in treatment.
Laser surgery for the facial birthmark (hemangioma) shows promise of alleviating this disfiguring problem.
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