Sturge-Weber syndrome with no leptomeningeal enhancement on MRI

Neuroradiology. 1998 Mar;40(3):177-80. doi: 10.1007/s002340050563.

Abstract

Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome characterized by a facial nevus flammeus associated with seizures, developmental delay, and, often, with hemiparesis and hemianopia. On MRI, the most characteristic finding has been reported to be leptomeningeal enhancement, believed to represent leakage of contrast medium through the anomalous pial vessels that characterize the disease. We present a case of SWS with no evidence of leptomeningeal enhancement. This case illustrates that leptomeningeal enhancement need not be present in SWS, and the absence of this characteristic finding does not preclude the diagnosis.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Arachnoid / pathology*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Pia Mater / pathology*
  • Sturge-Weber Syndrome / pathology*