title: Clinical features of Sturge-Weber syndrome without facial nevus: five novel cases.
abstract: Classic Sturge-Weber syndrome (SWS) is characterized by presence of flammeus nevus involving the first sensory branch of trigeminal nerve, ipsilateral leptomeningeal angiomatosis, and choroidal angioma. Sporadic cases of SWS without facial nevus (SWS type III) have been rarely reported. Here we report the clinical and neuroradiological findings of five patients with SWS type III and compare their findings with those described in the literature. This study confirmed that SWS type III should be considered in any child or young adult presenting with seizures or complicated migraine and intracranial unilateral calcification. The diagnosis must be confirmed with contrast-enhanced MRI images of the brain. Surgical therapy should be considered in patients with drug-resistant and persistent epileptic seizures.
authors: Neuropsichiatria Infantile Pediatria Ospedale San Paolo, Savona, Italy. laura.siri@libero.it
authors: Laura
authors: L
authors: Siri
authors: Lucio
authors: L
authors: Giordano
authors: Patrizia
authors: P
authors: Accorsi
authors: Massimo
authors: M
authors: Cossu
authors: Lorenzo
authors: L
authors: Pinelli
authors: Laura
authors: L
authors: Tassi
authors: Pasquale
authors: P
authors: Striano
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001927:Brain Diseases / Q000209:etiology / Q000473:pathology / Q000601:surgery; D002114:Calcinosis; D020787:Central Nervous System Venous Angioma / Q000209:etiology / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005145:Face; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008881:Migraine Disorders / Q000209:etiology; D019339:Port-Wine Stain; D012640:Seizures / Q000209:etiology; D013341:Sturge-Weber Syndrome / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D055815:Young Adult