[Sturge-Weber syndrome. Findings in 22 infantile cases (author's transl)]

Twenty two infants, 13 females and nine males, with Sturge-Weber syndrome are reviewed. Facial "nevus flammeus" is found to be located unilaterally in 13 cases and bilaterally in nine cases. Twenty infants presented seizures with generalized type being most frequent. E.E.G. alterations appeared in 18 cases. The I.Q. was normal in five infants although somewhat lower than the inferior limits of a normal I.Q. range. The major radiological alterations seen were an increased thickness of the skull cap; intracranial calcification (11 cases), unilateral in eight and bilateral in three; cerebral hemiatrophy (10 cases), arterial and venous hypoplasia and tortuosity in the entire extension of the carotid artery. One of the infants died with no apparent cause. This death cannot be attributed to the orignal syndrome. Computerized tomography offers early adequate data concerning the intracranial calcifications and cerebral hemiatrophy. Based on this study there does not seem to be sufficient motives to separate the Sturge-Weber and the Klippel-Trenaunay syndromes into two different entities.