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Dystrophic Myopathies

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Clinical Neuroradiology

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Abstract

This chapter of clinical neuroradiology focuses on the use of skeletal muscle imaging in dystrophic myopathies. It will discuss the use of both conventional (computed tomography, magnetic resonance imaging, ultrasound) and advanced muscle radiological techniques (diffusion tensor imaging, MR spectroscopy) to visualize the extent and distribution of muscle tissue damage which can be seen in this group of disorders. Muscle imaging can help to differentiate between different dystrophic myopathies and can be used as an aid for guided muscle biopsy. Indications for imaging will be discussed, and recommendations on systematically visualizing and evaluating the skeletal muscles will be formulated. Standardization of imaging tools is required before they can be used as reliable surrogate biomarkers in natural history studies and in therapeutic trials.

This publication is endorsed by: European Society of Neuroradiology (www.esnr.org)

Parts of this chapter have been reproduced from ten Dam, L., van der Kooi, A. J., Verhamme, C., Wattjes, M. P. and de Visser, M. (2016), Muscle imaging in inherited and acquired muscle diseases. Eur J Neurol, 23: 688–703 with permission from Wiley publications.

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Abbreviations

BMD:

Becker muscular dystrophy

CMD:

Congenital muscular dystrophy

DM1:

Myotonic dystrophy type 1

DM2:

Myotonic dystrophy type 2

DMD:

Duchenne muscular dystrophy

FSHD:

Facioscapulohumeral dystrophy

LGMD:

Limb-girdle muscular dystrophy

MMD:

Miyoshi distal myopathy

OPMD:

Oculopharyngeal muscular dystrophy

TMD:

Tibial muscular dystrophy/Udd myopathy

UCMD:

Ullrich congenital muscular dystrophy

WMD:

Welander distal myopathy

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Acknowledgements

We would like to thank Prof. E. Aronica, Dr. W.C.G. Plandsoen, Dr. C. Verhamme and Dr. A.J. van der for providing figures 2, 6, 7 and 11.

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Authors and Affiliations

  1. Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands

    Leroy ten Dam & Marianne de Visser

Authors
  1. Leroy ten Dam
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  2. Marianne de Visser
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Correspondence to Leroy ten Dam .

Editor information

Editors and Affiliations

  1. Radiology and Nuclear Medicine, VU University Medical Center, Amsterdam, The Netherlands

    Frederik Barkhof

  2. National Hosp.f Neurology & Neurosurgery, UCL National Hosp.f Neurology & Neurosurgery, London, United Kingdom

    Rolf Jager

  3. Division of Neuroradiology, University Hospital Vienna Division of Neuroradiology, Wien, Austria

    Majda Thurnher

  4. Department of Neuroradiology, Hospital Vall d’Hebrón Department of Neuroradiology, Barcelona, Spain

    Alex Rovira Cañellas

Section Editor information

  1. VUMC, Amsterdam, The Netherlands

    Mike Wattjes

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ten Dam, L., de Visser, M. (2018). Dystrophic Myopathies. In: Barkhof, F., Jager, R., Thurnher, M., Rovira Cañellas, A. (eds) Clinical Neuroradiology. Springer, Cham. https://doi.org/10.1007/978-3-319-61423-6_3-1

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  • DOI: https://doi.org/10.1007/978-3-319-61423-6_3-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-61423-6

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