Performance of a new quantitative method for assessing dural ectasia in patients with FBN1 mutations and clinical features of Marfan syndrome

Neuroradiology. 2009 Jun;51(6):397-400. doi: 10.1007/s00234-009-0508-9. Epub 2009 Feb 19.

Abstract

Introduction: This study presents a comparison of established methods for measuring dural ectasia with a new quantitative method of assessing this clinical feature.

Methods: Seventeen patients with an identified mutation in FBN1 were examined for dural ectasia. The results were compared with 17 age- and sex-matched controls. Our images were also evaluated using the two methods of quantifying dural ectasia, namely those of Ahn et al. and of Oosterhof et al.

Results: With our method, 80% MFS1 patients and 7% controls fulfilled the criterion for dural ectasia. Using the method of Oosterhof et al., dural ectasia was found in 88% patients with MFS1 and in 47% controls. Using the method of Ahn et al. 76% patients with Marfan syndrome and 29% controls showed dural ectasia.

Conclusion: We present a novel quantitative method of evaluating MRT images for dural ectasia, which, in our own patient cohort, performed better than those previously described.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Dilatation, Pathologic / diagnosis
  • Dura Mater / pathology*
  • Female
  • Fibrillin-1
  • Fibrillins
  • Genetic Predisposition to Disease / genetics
  • Humans
  • Image Interpretation, Computer-Assisted / methods*
  • Magnetic Resonance Imaging / methods*
  • Male
  • Marfan Syndrome / diagnosis*
  • Marfan Syndrome / genetics*
  • Microfilament Proteins / genetics*
  • Middle Aged
  • Mutation
  • Reproducibility of Results
  • Sensitivity and Specificity
  • Young Adult

Substances

  • FBN1 protein, human
  • Fibrillin-1
  • Fibrillins
  • Microfilament Proteins