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

  • Diagnostic Neuroradiology
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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.

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Acknowledgements

We would like to thank the German Marfan patient support group, MarfanHilfe Deutschland e.V., for financial and logistic support. We also would like to thank all patients and control subjects for their participation in this study.

Conflict of interest statement

We declare that we have no conflict of interest.

Author information

Author notes

  1. Kerstin Hinz

    Present address: Vivantes Klinikum Neukölln, Institut für Radiologie und Interventionelle Therapie, Rudower Str. 48, 12351, Berlin, Germany

Authors and Affiliations

  1. Institute of Human Genetics, Hannover Medical School, Carl-Neuberg-Straße 1, 30625, Hannover, Germany

    Bahar Söylen, Jörg Schmidtke & Mine Arslan-Kirchner

  2. Institute of Diagnostic and Interventional Neuroradiology, Hannover Medical School, Carl-Neuberg-Straße 1, 30625, Hannover, Germany

    Kerstin Hinz & Hartmut Becker

  3. Institute for Biometrics, Hannover Medical School, Carl-Neuberg-Straße 1, 30625, Hannover, Germany

    Jana Prokein

Authors
  1. Bahar Söylen
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  2. Kerstin Hinz
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  3. Jana Prokein
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  4. Hartmut Becker
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  6. Mine Arslan-Kirchner
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Corresponding author

Correspondence to Jörg Schmidtke.

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Söylen, B., Hinz, K., Prokein, J. et al. Performance of a new quantitative method for assessing dural ectasia in patients with FBN1 mutations and clinical features of Marfan syndrome. Neuroradiology 51, 397–400 (2009). https://doi.org/10.1007/s00234-009-0508-9

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  • DOI: https://doi.org/10.1007/s00234-009-0508-9

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