Teaching NeuroImage: Sturge-Weber Syndrome in an Adult

We report a 19-year-old right-handed man with a history of Sturge-Weber syndrome (SWS) based on port-wine stain involving the first division of the trigeminal nerve in the right hemiface (Figure 1) and leptomeningeal capillary-venous malformations associated with calcification involving the ipsilateral occipital lobe (Figures 2, E–G) and resultant refractory epilepsy. He was referred to our center for a presurgical epilepsy evaluation. Video-EEG data showed electroclinical and electrographic seizures arising from the right frontotemporal region and right posterior quadrant, respectively. In addition, there were frequent right temporal interictal discharges. He also had evidence of right hippocampal sclerosis (Figures 2, C and D), suspected to be the result of longstanding refractory epilepsy (i.e., dual pathology). SWS is a neurocutaneous disorder characterized by ipsilesional facial and leptomeningeal capillary-venous malformations with regional atrophy, gyral calcification, focal leptomeningeal enhancement, and bony changes (Figures 2, A, B, E–H).1,2

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