CT and MR pattern of Sturge-Weber syndrome
Unlike other phakomatoses, Sturge-Weber syndrome has no genetic basis. The pathophysiological basis is hypothesised to relate to a developmental failure of the superficial cerebral veins, leading to stasis, hypoxia and subsequent formation of abnormal leptomeningeal vascular channels.
In addition to its cutaneous manifestations, Sturge-Weber syndrome clinically features buphtalmos and/or glaucoma, seizures, focal neurological failures and mental retardation. Neuroradiological findings include leptomeningeal enhancement, cortical atrophia with enlarged adjacent subarachnoid spaces, "tram-track" gyriform calcifications (68%), enlarged and calcified choroidal plexus, enlarged facial air spaces and mastoid cells, and choroidal angiomatosis (53%).
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