(William Allen Sturge, 18501919, British physician, and Frederick Parkes Weber, 18631962, British physician) (also called encephalotrigeminal angiomatosis), a phakomatosis characterized by a "port-wine" vascular anomaly of the skin in the trigeminal nerve distribution (mainly in the region of the first division) associated with leptomeningeal venous angiomatosis, hypertrophy of the choroid plexus and anomalies of venous drainage of the brain.

Clinically patients may present with seizures, focal neurological deficits, dementia and glaucoma.

Pathologically atrophy of the underlying cortex and cortical calcifications are found.

All imaging modalities are useful in the diagnosis. Plain films may show the characteristic tramtrack calcifications (Fig.1), also well seen on CT, as well as skull asymmetries and calvarial thickening secondary to cortical and brain atrophy. Enhancement of pial angiomatosis is seen on both on CT (Fig.2) and MRI; associated ocular anomalies (buphthalmos and choroidal angiomas) as well as anomalies of the choroid plexus and of deep venous drainage are also seen and better demonstrated by angiography.