Chapter 23. Limb Girdle Muscular Dystrophies

  1. Nils Erik Gilhus MD, PhD Professor6,
  2. Michael P. Barnes MD, FRCP Professor of Neurological Rehabilitation Medical Director7 and
  3. Michael Brainin MD Professor8
  1. F. Norwood1,2,
  2. M. de Visser3,
  3. B. Eymard4,
  4. H. Lochmüller5 and
  5. K. Bushby1

Published Online: 6 SEP 2010

DOI: 10.1002/9781444328394.ch23

European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition

European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition

How to Cite

Gilhus, N. E., Barnes, M. P. and Brainin, M. (2010) Limb Girdle Muscular Dystrophies, in European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition, Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444328394.ch23

Author Information

  1. 6

    Department of Clinical Medicine, University of Bergen and Department of Neurology, Haukeland University Hospital, Bergen, Norway

  2. 7

    University of Newcastle, Hunters Moor Neurorehabilitation Ltd, Newcastle upon Tyne, UK

  3. 8

    Department of Clinical Medicine and Prevention, and Center for Clinical Neurosciences, Donau-Universität Krems, Department of Neurology, Landesklinikum Donauregion Tulln Tulln, Austria

Author Information

  1. 1

    Institute of Human Newcastle upon Tyne, UK

  2. 2

    King's College Hospital, London, UK

  3. 3

    University of Amsterdam, The Netherlands

  4. 4

    Hôpital de la Pitié, Salpétriere, Paris, France

  5. 5

    Genzentrum, Ludwig-Maximilians-Universität, Munich, Germany

Publication History

  1. Published Online: 6 SEP 2010
  2. Published Print: 15 OCT 2010

ISBN Information

Print ISBN: 9781405185332

Online ISBN: 9781444328394

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Keywords:

  • limb girdle muscular dystrophies (LGMD);
  • LGMD, first described - as clinical entity by Walton and Natrass;
  • LGMDs grouped into two - autosomal dominant or recessive and subtypes;
  • Good Practice Points - for LGMD management;
  • thorough clinical assessment - for directing further investigation;
  • LGMDs, predilection for involvement - of proximal musculature in shoulder and pelvic girdles;
  • contractures, most common in LGMD1B - in childhood, or developing over course of condition;
  • characteristic muscle biopsy findings in more common LGMDs;
  • respiratory muscle weakness, and symptomatic hypoventilation and respiratory failure in LGMDs;
  • issue of cardiac complications in LGMD - and muscle conditions, at the 107th ENMC workshop

Summary

This chapter contains sections titled:

  • Objectives

  • Background

  • Search strategy

  • Method for reaching consensus

  • Results

  • Good Practice Points for management of LGMD

  • References