Classical Sturge-Weber Syndrome (SWS) is a neuroectodermal disease characterized by facial congenital unilateral port-wine nevus that affects the area innervated by the first sensory branch of trigeminal nerve, typical ipsilateral leptomeningeal angiomatosis and calcifications in the occipital region, focal seizures, intellectual impairment, hemiparesis and homonymous hemianopsia controlateral to the brain lesion. Clinical picture may be extremely variable with different subtypes described: Type I: with facial and leptomeningeal angiomas (classic SWS); Type II: with only facial angioma; Type III: with only leptomeningeal angioma. Here we describe 3 cases of SWS Type III: a 9 year-old girl (Case A), a 19 year-old boy (case B) and 11 year-old boy (Case C); data concerning electroencephalograms (EEG), CT and MRI with intravenous administration of gadolinium are provided. Our patients showed a SWS type III with no facial angioma, no mental retardation nor glaucoma or hemiparesis, differently than observed in classical SWS. All the 3 patients showed epilepsy, in 2 cases with drug resistant seizures; one of them (Case A) underwent neurosurgery for intractable epilepsy.
|Translated title of the contribution||Sturge-Weber syndrome without facial nevus: Description of 3 cases|
|Number of pages||3|
|Journal||Bollettino - Lega Italiana contro l'Epilessia|
|Publication status||Published - Apr 2010|
ASJC Scopus subject areas
- Clinical Neurology