Sturge-Weber Syndrome
This syndrome is also known as Encephalotrigeminal Angiomatosis. This condition is characterized by the presence of a huge port wine nevus occupying the upper face associated with the following:
– focal or generalized seizures
– intracranial calcification ipsilateral to the skin lesion
– contralateral hemiparesis
– mental retardation
The seizures are seen in 90 % of all cases and usually appear early. These are generally intractable to treatment particularly with increasing age. Mental retardation and behavioral disorders are noted in about 80% of all patients.
In individuals with associated angioma of the eye, glaucoma and buphthalmos may be seen.
This condition can be diagnosed in children who showed the above manifestations. Skull radiographs usually reveal the typical “railroad tracks” calcification. Electroencephalogram is frequently abnormal , showing focal and epileptiform changes.
Sturge-Weber Syndrome is being managed clinically with the use of anticonvulsant medications; however, many of the patients continue to deteriorate thus surgical resection of the affected lobe or a complete hemispherectomy may be done as the ultimate treatment. And it is noted that postoperative results showed good control of seizures and behavior.