Pediatric Radiology > Neurological > Neurocutaneous Syndromes > Sturge-Weber Syndrome

Sturge-Weber Syndrome

Sturge-Weber syndrome is also called encephalotrigeminal angiomatosis and is characterized by low flow vascular malformations in the trigeminal nerve distribution. On the skin, the port wine nevus is the abnormality. Intracranially, the low flow state in the leptomeninges results in chronic ischemic injury leading to atrophy of the involved gyri. Clinical manifestations include seizures, mental retardation, and hemiparesis. Serpiginous calcifications are seen overlying the involved segment of parenchyma.


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