Clinical features of Sturge-Weber syndrome without facial nevus: Five novel cases

Laura Siri, Lucio Giordano, Patrizia Accorsi, Massimo Cossu, Lorenzo Pinelli, Laura Tassi, Pasquale Striano

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)91-96
Number of pages6
JournalEuropean Journal of Paediatric Neurology
Issue number1
Publication statusPublished - Jan 2013


  • Cortical calcifications
  • Drug-resistant seizures
  • Pial angioma
  • Sturge-Weber
  • Surgical resection

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health


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