The Sturge-Weber Syndrome

The association of vascular nxvi of the face, predominantly unilateral in distribution, with homolateral changes in the cerebral cortex, has long been of interest to clinicians. The first case4 of this disorder was presented by W. Allen Sturge to the Clinical Society of London in 1879. Weber5 called attention to the characteristic roentgenological features of this disease in 1922, and in 1934. Krabbe2 published the first histological studies of an involved brain. Although the disorder has been known by a variety of names, those of Sturge and Weber appear most prominently in the recent literature and it seems fitting to call the syndrome the Sturge-Weber syndrome. No attempt will be made in this paper to summarize the literature, for at least one extensive review is already available1 and shorter papers, such as the one by Nussey and Miller,3 summarize less exhaustively the facts known about the syndrome. Although the reported cases of this clinical entity now number considerably more than 100, the disease is still an unfamiliar one to most physicians, and a typical case is a clinical curiosity even to the neurologist. It seems justifiable, therefore, to repart threet classical cases of this syndrome which have been observed recently in the Medical and Pediatric Services of the New Haven Hospital and Dispensary. Report of cases