Chapter 118. Progressive Muscle Dystrophies

  1. Robert P. Lisak MD, FAAN, FRCP Parker Webber Chair Professor Chair Neurologist-in-Chief Chief2,3,
  2. Daniel D. Truong MD, FAAN Head4,
  3. William M. Carroll MBBS, MD, FRACP, FRCP(E) Head5 and
  4. Roongroj Bhidayasiri MD, FRCP Director6,7
  1. Stephan Zierz MD Professor of Neurology Head

Published Online: 18 MAY 2010

DOI: 10.1002/9781444317008.ch118

International Neurology: A Clinical Approach

International Neurology: A Clinical Approach

How to Cite

Zierz, S. (2009) Progressive Muscle Dystrophies, in International Neurology: A Clinical Approach (eds R. P. Lisak, D. D. Truong, W. M. Carroll and R. Bhidayasiri), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444317008.ch118

Editor Information

  1. 2

    Wayne State University School of Medicine, Detroit, MI, USA

  2. 3

    Detroit Medical Center, Harper University Hospital, Detroit, MI, USA

  3. 4

    The Parkinson and Movement Disorder Institute, Memorial Neuroscience Institute, Orange Coast Memorial Medical Center, Fountain Valley, CA, USA

  4. 5

    Sir Charles Gairdner Hospital, Nedlands, Perth, Australia

  5. 6

    Chulalongkorn Comprehensive Movement Disorders Center, Chulalongkorn University Hospital, Bangkok, Thailand

  6. 7

    University of California at Los Angeles, School of Medicine, Los Angeles, CA, USA

Author Information

  1. Department of Neurology, University Halle-Wittenberg, Halle/Saale, Germany

Publication History

  1. Published Online: 18 MAY 2010
  2. Published Print: 11 SEP 2009

ISBN Information

Print ISBN: 9781405157384

Online ISBN: 9781444317008



  • progressive muscle dystrophies - clinically and etiologically heterogeneous group of myopathies;
  • clinical phenotypes of dystrophinopathies;
  • muscle dystrophy, by Wilhelm Erb - progressive neuromuscular disorder (“dystrophia muscularis progressiva”);
  • dystrophinopathies, transmitted by X-linked recessive inheritance;
  • Duchenne type (DMD);
  • dystrophinopathies, hereditary muscular dystrophies - with different phenotypes caused by different mutations;
  • kyphoscoliosis and respiratory muscle weakness - around 8 or 9 years;
  • limb-girdle muscle dystrophies (LGMD);
  • oculopharyngeal muscular dystrophy (OPMD);
  • myotonic dystrophies and nuclear envelopathies


This chapter contains sections titled:

  • Introduction

  • Dystrophinopathies

  • Limb-girdle muscle dystrophies (LGMD)

  • Facioscapulohumeral muscular dystrophy (FSHD)

  • Oculopharyngeal muscular dystrophy (OPMD)

  • Myotonic dystrophies

  • Nuclear envelopathies

  • Distal myopathies

  • Further reading