Sturge Weber Syndrome is a condition of unknown cause characterised by a port wine stain in the distribution of the ophthalmic division of the trigeminal nerve and an ipsilateral leptomeningeal venous malformation. The malformations are abnormal venous structures situated along the space between the pia mater and the arachnoid membrane. The abnormal venous drainage in the underlying cerebral cortex results in cerebral ischaemia. Cerebral atrophy and classical cortical “tramtrack” calcification then develops. The abnormal cerebral cortex acts as an epileptogenic focus.
Clinical features:
Recurrent seizures
Hemiparesis
Dementia
Glaucoma
Buphthalmos (large eyeball)
Radiological features:
Cortical atrophy on the affected side.
Tramtrack calcification of the cerebral cortex.
Overlying calvarial thickening and paranasal sinus enlargement.
Ocular anomalies eg choroidal angiomata.
Enlarged deep medullary and subependymal veins. These
probably result from slow flow or thrombosis in the superficial
venous system and the subsequent shunting of blood through
deep medullary veins.
Choroid plexus enlargement in the ipsilateral lateral ventricle.