Syndrome van Sturge en Weber


Sturge Weber Syndrome is a congenital condition characterized by an intracranial vascular anomaly, leptomeningeal angiomatosis, most often involving the occipital and parietal lobes. The anomaly can affect both cerebral hemispheres.
An ipsilateral facial cutaneous capillary vascular malformation usually affects the upper face in the V1 distribution of the trigeminal nerve.
Other findings include glaucoma, buphthalmos, enlargement of the choriod plexus and seizures. Hemiparesis, hemiatrophy, hemianopia and strokelike events may occur contralateral to the cortical abnormality.
Venous stasis results in ischemia underlying the leptomeningeal angiomatosis leading to calcification and laminar cortical necrosis.

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