Sturge-Weber’s syndrome (SVS) is a congenital developmental defect of the cerebral portion of the neuroectoderm with a dermal angioma in the face and a pial angioma as a rule in an occipital ipsilateral position to the facial angioma. Clinically it is manifested by partial epileptic seizures, hemiparesis, hemianopsia, glaucoma and mental retardation. The authors describe the clinical picture, X-ray and EEG findings in 8 children, 5 boys and 3 girls aged 10 months to 14 years who suffered from SW S and were followed up for at least two years. The facial angioma was unilateral in the innervation area of the first branch of the Vth nerve, in two children it was bilateral. The objective finding was hemiparesis of different grades, in one instance hemianopsia and glaucoma. Mental retardation was found in all children except one. All children had epileptic seizures mostly focal motor seizures and attacks of weakness of the extremities without cramps with headache and vomiting. In the EEG tracing there was slow activity above the angioma, only in three children there were epileptic discharges. In 5 children there was a typical CT picture of hemiatrophy, focal atrophy and calcifications. In the diagnosis of SWS MR examination of the brain proved most useful revealing an angioma even when the CT was negative. Angiography has no diagnostic value. In the course of the follow up the epileptic seizures were compensated by treatment in 3 children. The diagnosis of SWS can be established on the basis of the clinical picture, CT and MR examination, which reveal accurately morphological changes. A useful supplement of the examination is examination by functional imaging methods (SPECT and PET), in particular in case of an adverse development and planned surgical resection.

        Key words: Sturge-Weber syndrome, clinical picture, epileptic seizures, neuroradiological findings, EEG