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Pediatric Radiology

, Volume 35, Issue 1, pp 85–90 | Cite as

Early diagnosis of cerebral involvement in Sturge-Weber syndrome using high-resolution BOLD MR venography

  • Hans-J. MentzelEmail author
  • Andrea Dieckmann
  • Clemens Fitzek
  • Ulrich Brandl
  • Jürgen R. Reichenbach
  • Werner A. Kaiser
Case Report

Abstract

Sturge-Weber syndrome (SWS) is a congenital disorder characterized by a vascular birthmark and neurological abnormalities. Typical imaging findings using MRI or CT are superficial cerebral calcification, atrophy and leptomeningeal enhancement. We present a neonate diagnosed with SWS because of a port-wine stain. In the absence of neurological symptoms the first MRI was performed when he was 4 months old, and follow-up MRI studies were performed after his first seizure at the age of 12 months. MRI was performed using standard sequences before and after administration of IV gadolinium. A high-resolution T2*-weighted, rf-spoiled 3D gradient-echo sequence with first-order flow compensation in all three directions was used for additional venographic imaging [blood-oxygen-level-dependent (BOLD) venography]. The initial conventional MRI sequences did not demonstrate any abnormality, but BOLD venography identified leptomeningeal internal veins. Follow-up MRI after the first onset of seizures demonstrated strong leptomeningeal enhancement, while BOLD venography revealed pathological medullary and subependymal veins as well as deep venous structures. At this time there were the first signs of atrophy and CT showed marginal calcifications. This report demonstrates that high-resolution BOLD MR venography allows early diagnosis of venous anomalies in SWS, making early therapeutic intervention possible.

Keywords

Brain Congenital disorder Sturge-Weber syndrome MRI BOLD MR venography Child 

Notes

Acknowledgements

This study was funded by the Deutsche Forschungsgemeinschaft (DFG) (Grant No. RE 1123/7–1) and the Nycomed Amersham Research Fellowship Grant (to J.R.R.) of the ECR 2002 Research and Education Fund.

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Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Hans-J. Mentzel
    • 1
    Email author
  • Andrea Dieckmann
    • 2
  • Clemens Fitzek
    • 1
  • Ulrich Brandl
    • 2
  • Jürgen R. Reichenbach
    • 1
  • Werner A. Kaiser
    • 1
  1. 1.Institute of Diagnostic and Interventional Radiology, Department of Pediatric RadiologyFriedrich-Schiller-University JenaJenaGermany
  2. 2.Department of NeuropediatricsFriedrich-Schiller-University JenaJenaGermany

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