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November 1969

Sturge-Weber Syndrome

Author Affiliations

Durham, NC
From the Divisions of Neurosurgery and Neurology, Duke University Medical Center and the Durham Veterans Administration Hospital, Durham, NC.

Arch Neurol. 1969;21(5):554-555. doi:10.1001/archneur.1969.00480170126013

THE Sturge-Weber syndrome is usually easy to recognize. It consists of angiomatosis of the upper portion of the face and the cerebral leptomeninges, with progressive calcification in the underlying cerebral cortex.1 These abnormalities are usually unilateral. Atrophy of the involved cerebral hemisphere may be present, along with contralateral hemiparesis, focal seizures, and mental retardation. Buphthalmos or glaucoma may also occur.

Information about this unusual condition has been compiled slowly during the past century through individual case reports by many physicians. William Allen Sturge (1850 to 1919)2 and Frederick Parkes Weber (1863 to 1962 )3 were English clinicians whose separate reports, published 43 years apart, illustrated different facets of the syndrome.

In 1860, Schirmer4 published a brief description of a patient with a facial vascular nevus and buphthalmos, but he made no mention of any neurological abnormalities. Then in 1879, at a meeting of the Clinical Society

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