Brain imaging of a patient with Sturge-Weber syndrome and bilateral leptomeningeal capillary-venous malformations

A 64-year-old patient with SWS, epilepsy, and severe intellectual disability. Noncontrast bone window CT (A, B) shows extensive, bilateral gyriform calcifications in a curvilinear pattern often called the "tram track" sign. FLAIR MRI (C, D) shows extensive atrophy of left frontal and parietal lobes. Postcontrast MRI in transverse axial (E, F) and coronal planes (G) shows leptomeningeal enhancement consistent with leptomeningeal capillary venous malformations (LCVMs) in both cerebral hemispheres. In addition, choroid enlargement is also noted (D, F, G) and correlates with the extent of the LCVMs. Cognitive function is poorest in patients with bilateral LCVMs.