STURGE-WEBER SYNDROME (ENCEPHALOTRIGEMINAL ANGIOMATOSIS)

THE CLASSIC clinical manifestations of the Sturge-Weber syndrome include a vascular nevus of the face, a hemiplegia contralateral to the cutaneous lesion, an intracerebral calcification found roentgenographically ipsilateral to the nevus flammeus, various abnormalities of the ipsilateral eyeball, convulsive seizures, and mental retardation. This condition seems to have received more attention in the European literature. Indeed, Nussey and Miller¹ and McCoy and Voris ² have gone so far as to suggest that the prevalence of this disorder may be greater in continental Europe. However, it would seem to us that there may well be many cases which really belong in the category of this malady but may be unrecognized because the entire clinical picture is not present.

We have had the opportunity to study five patients with the Sturge-Weber syndrome who presented signs and symptoms in combinations varying from the cardinal features mentioned above. It was felt that a