Abstract

FERNANDEZ JAEN, A et al. Sturge Weber syndrome with epilepsy and bilateral intracranial calcifications at the neonatal period. Arch. Neurocien. (Mex., D.F.) [online]. 2004, vol.9, n.4, pp.233-236. ISSN 1028-5938.

The Sturge Weber syndrome is an unfrequent neurocutaneous disorder characterized by the association of a venous angioma of the pia mater with a port-wine stain of the face. Bilateral facial nevi are not uncommon and have been seen in 30% of patients; 25% of these cases have bilateral leptomeningeal angiomatosis. Partial seizures are usually the first neurologic manifestation and frequently begin in the first months of life. CT scan can show the characteristic intracranial calcifications, although they are rarely present at birth. We report a rare case of Sturge Weber disease with an early onset of seizures, bilateral facial nevi and intracranial calcification at the neonatal periodo.

Keywords : Sturge Weber; leptomeningeal angiomatosis; intracranial calcification; facial nevi.