Sturge-Weber syndrome (encephalotrigeminal or leptomeningeal angiomatosis).

摘要

william Allen Sturge (fig 1) first described a syndrome in 1879 in a e'A-year-old girl."She started to have twitching on (the) left side of her body as a six months old baby. The attacks worsened but without loss of consciousness. Later, the twitching started to spread to the other side and she would lose consciousness. She benefited from potassium bromide. Of particular interest was that the child had what was described as (a) "mother's mark" on the right side of the head and face. The skin lesion was accurately demarcated in the midline and involved the upper lip, nose, forehead, scalp and back of the neck, extending a little beyond the midline on the chin and on the upper part of the sternum. It extended as low as the third or fourth dorsal vertebra. The lips, gums, tongue, roof of mouth, floor of mouth, uvula and pharynx were all similarly affected. The right eye was larger (buphthalmos, congenital glaucoma) and the sclera, choroid and retina were all affected by a vascular malformation. In addition, there was a patch about the size of the palm of the hand over the left eye, frontal and temporal regions. The mark was of a deep purple colour, the colour partially disappearing on firm pressure. The affected parts were distinctly larger than the corresponding parts on the other side".