The Sturge-Weber Syndrome: Correlation Between the Clinical Status and Radiological CT and MRI Findings

Childs Nerv Syst. 1993 Apr;9(2):107-9. doi: 10.1007/BF00305319.

Abstract

In the Sturge-Weber syndrome facial venous and leptomeningeal angiomas are associated. We studied 14 consecutive cases with clinical and radiological evaluations [computed tomography (CT) and magnetic resonance imaging (MRI)]. Radiological studies demonstrated the extent and patency of the leptomeningeal angiomatous malformation, the degree of parenchymal atrophy, the presumed ischemic changes affecting the gray and white matter, the presence and extent of cortical calcifications, the prominence of the choroid plexus, the parenchymal venous anomalies, and the diploic prominence in the affected side. The white matter alterations, being greater, the extent of lobar involvement, and the degree of parenchymal atrophy all correlated with the patient's clinical status being poorer. MRI is more efficient in the detection of the radiological findings related to the clinical status: seizure control, degree of psychomotor development and hemiparesis. Therefore, MRI is the imaging modality of choice in the diagnosis of these patients.

MeSH terms

  • Adolescent
  • Adult
  • Brain Damage, Chronic / diagnosis
  • Brain Damage, Chronic / genetics
  • Brain Neoplasms / diagnosis*
  • Brain Neoplasms / genetics
  • Child
  • Child, Preschool
  • Facial Neoplasms / diagnosis*
  • Facial Neoplasms / genetics
  • Female
  • Follow-Up Studies
  • Hemangioma / diagnosis*
  • Hemangioma / genetics
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Neurologic Examination
  • Sturge-Weber Syndrome / diagnosis*
  • Sturge-Weber Syndrome / genetics
  • Tomography, X-Ray Computed*