American Journal of Neuroradiology, Vol 13, Issue 1 291-294, Copyright © 1992 by American Society of Neuroradiology
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Cranial CT and MR in the Klippel-Trenaunay-Weber syndrome
DW Williams 3d and AD Elster
Department of Radiology, Bowman Gray School of Medicine of Wake Forest University, Winston-Salem, NC 27157-1022.
This report describes the intracranial CT and MR findings in two cases of Klippel-Trenaunay-Weber Syndrome. The findings are 1) markedly enhancing choroid plexuses, 2) severe cerebral atrophy, 3) cerebral calcifications, and 4) angiomatous leptomeningeal enhancement. The findings may resemble those seen in cases of bilateral Sturge-Weber syndrome. The two diseases should be distinguishable by the external stigmata. The authors raise the question of a spectrum of involvement in the angiodysplasias of Klippel-Trenaunay-Weber syndrome and Sturge- Weber syndrome with considerable overlap.
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