This volume provides a comprehensive and scholarly accounting of motor neuron disorders, a group of diseases that are both clinically and genetically diverse. Clinicians and neuroscientists alike will find invaluable research and information on recent advancements that have occurred in the understanding of these disorders.

From specific discussions of their underlying molecular pathogenesis, to advancements in therapies and diagnosis, users will find a resource that presents a comprehensive understanding of the general clinical features and management of these devastating disorders. In addition, new tactics that can be used to enhance patient care are also presented and discussed.

Specific chapters within the volume include discussions pertaining to the historical aspects of motor neuron diseases, comparative anatomy, physiology, and development of the corticospinal system, and discussions of spinal muscular atrophies, amongst others. In addition, symptomatic therapy and the palliative aspects of clinical patient care are also presented.

Key Features

* A scholarly accounting of new research in motor neuron disorders and their associated diseases
* The intricate relationships that exist between different motor neuron related disorders
* A comprehensive resource that provides a guide to the diagnosis and treatment of motor neuron disorders, along with new approaches to patient care


Neuroscience research workers

Table of Contents

1. Historical aspects of motor neuron diseases, Andrew A. Eisen (Vancouver, Canada)

SECTION 1 Basic aspects

2. Comparative anatomy and physiology of the corticospinal system, Marc H. Schieber (Rochester, NY, USA)

3. Development of the corticospinal system and spinal motor circuits, John H. Martin (New York, NY, USA)

4. Molecular mechanisms of motor neuron degeneration in amyotrophic lateral sclerosis, Siân C. Barber and Pamela J. Shaw (Sheffield, UK)

5. Cytopathology of the motor neuron, Paul G. Ince and Stephen B. Wharton (Sheffield, UK)

6. Animal models of motor neuron death, Jean-Pierre Julien and Jasna Kriz (Quebec, Canada)

SECTION 2 Anterior horn cell disorders and motor neuropathies

7. Spinal muscular atrophies and hereditary motor neuropathies, Kevin Talbot and Kay E. Davies (Oxford, UK)

8. Spinobulbar muscular atrophy (Kennedy’s disease), Jean-Marc Gallo and P. Nigel Leigh (London, UK)

9. GM2 gangliosidoses, Amos D. Korczyn (Ramat-Aviv, Israel)

10. Viral infections of lower motor neurons, Tom Solomon, Mong How Ooi and Macpherson Mallewa (Liverpool, UK and Sarawak, Malaysia)

11. Monomelic amyotrophy of upper or lower limbs, M. Gourie-Devi (New Delhi, India)

12. Multifocal and other motor neuropathies, Leonard H. van den Berg, Hessel Franssen, Jan-Thies H. van Asseldonk, Renske M. van den Berg-Vos and John H. J. Wokke (Utrecht, The Netherlands)

SECTION 3 Amyotrophic lateral sclerosis

13. Amyotrophic lateral sclerosis, P. Nigel Leigh (London, UK)

14. Familial amyotrophic lateral sclerosis, Christopher E. Shaw, Virginia Arechavala-Gomeza and Ammar Al-Chalabi (London, UK)

15. Juvenile amyotrophic lateral sclerosis, Paul Orban, Rebecca S. Devon, Michael R. Hayden and Blair R. Leavitt (Vancouver, Canada and Edinburgh, UK)

SECTION 4 Corticospinal disorders

16. Primary lateral sclerosis, Andrew A. Eisen (Vancouver, Canada)

17. He


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© 2007
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About the editors

Andrew Eisen

Affiliations and Expertise

Department of Neurology, University of British Columbia, Vancouver General Hospital, Vancouver, Canada

Pamela Shaw

Affiliations and Expertise

Professor of Neurology, Academic Neurology Unit, Division of Genomic Medicine, University of Sheffield, Sheffield, UK