A clinical and neurological study on the Sturge-Weber syndrome

The author studied the relationship between facial nevus and intracranial changes seen on cranial computed tomography (CCT) scannings in 12 children with typical Sturge-Weber syndrome. The evaluation of epileptic attacks, repeat EEG and cranial CT examinations with or without enhancement during the follow-up period of 8 years in average were analysed. In 7 cases (58.3 %), the dominant side of facial hemangioma was identical with that of calcification on CCT. Three cases of central facial nevus showed calcification in one hemisphere, on either side. One who had facial nevus on one side showed dominant calcification on the other side on CT. The area and side of the facial nevus did not always coincide with those of the intracranial lesion. In 4 of the 9 patients who were followed up by repeat CCT, we recognized increases in degree of brain atrophy with or without increases in the area of calcification. In the enhancement study, 6 patients (89 %) showed positive choroid plexus images with abnormal enhancement on the same side as the calcification. On EEG 5 cases showed epileptiform activity over the hemisphere with calcification, and 3 showed it on the intact side of the brain. (author)