[Isolated arterial blood vessel dysplasia in Sturge-Weber-Syndrome (author's transl)].

An 18 year old patient is reported who suffered from cerebral convulsions, originating in the right hemisphere, beginning at age fourteen. Explorative trepanation of the scull was carried out after confirmation of (1) discreet neurologic disturbances on neurologic examination in the right hemisphere, (2) focal sign on the right side in the EEG (focal slowing and focal sharp wave), and (3) a right-parietal increase of radioactive activity in the scintigram. Cerebral angiographia, insufflation encephalographia, skull x-rays and the ophthalmologic examinations were without pathologic findings. During trepanation a macroscopically typical finding of Sturge-Weber-syndrome could be demonstrated (angioma capillare et venosum) covering almost the entire right posterior hemisphere.