Abstract
Drs. Gorman and Snead bring up a purely semantic question: "What is the Sturge-Weber syndrome?" According to McKusick,1 Sturge-Weber syndrome, sometimes called the fourth phacomatosis, is characterized by nevus flammeus of the face and angioma of the meninges. Warkany2 states that it "consists of a facial port wine nevus, convulsions and other neurological manifestations." In fact, the latter author, in discussing reported cases, rules out as Sturge-Weber syndrome a case with the CNS changes but no skin involvement.
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