Definition:
Weber Sturge Dimitri Syndrome otherwise known as the encephalotrigeminal angiomatosis is a rare congenital neurological and skin disorder. It is a phakomatoses and usually associated with port-wine stains on the face, glaucoma, seizures, ipsilateral leptomeningeal angioma and mental retardation.
Diagnosis:
Diagnosis includes physical and medical examination of the symptoms as they appear to the person affected with the condition.
Treatment:
Treatment of the medical condition varies and depends mainly on the symptoms. Usually it includes laser treatments to lighten or even to remove the birthmarks. Adminsitration of Anticonvulsant medications are used to control seizures. Frequent monitoring to the eye which is prone for glaucoma are also done and in serious cases, surgery may be performed. Physical therapy for the weak muscles are also used to treat the muscles. In some cases, doctors also advised brain surgery where the part of the brain that is affected by the disorder is removed to control seizures but nevertheless shows no guarantee that seizures will never manifest again.
Symptoms and Signs:
Weber Sturge syndrome includes varying birthmarks in infants, convulsions weakness of one side of the body which the birthmark which is usually a large port-wine stain. Delay in growth and development is also seen as well as mental retardation and galucoma due to the increase pressure within the eye which also can cause enlargement of the eyeball and bupthalmos or the bulging of the eye out of its socket. Seizures in infants are also noted.
Causes:
Weber Sturge syndrome is said to be caused by an arteriovenous malformation that usually happen in the cerebrum of the brain on the same side where the symptoms are seen to wit, the presence of the the birthmarks (W. A. Sturge. A case of partial epilepsy, apparently due to a lesion of one of the vasomotor centres of the brain. Transactions of the Clinical Society of London, 1879, 12: 162).
:
webersturgedimitrisyndrome
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