Askanas / Engel Muscle Aging, Inclusion-Body Myositis and Myopathies

1. Auflage 2011
ISBN: 978-1-4443-9829-8
Verlag: John Wiley & Sons
Format: EPUB
Kopierschutz: Adobe DRM (»Systemvoraussetzungen)

E-Book, Englisch, 264 Seiten, E-Book

Muscle weakness with ageing is almost inevitable, generallybeginning to manifest beyond the age of 40, and is usuallyunstoppable. It can lead to reduced mobility, increased risk offalling, injury, and even death. But "you're justgetting old" is not a sufficient diagnosis. Specific causesof neuromuscular symptoms may explain progressive muscle weakness,and should be investigated for potential treatment.
Muscle Ageing, Inclusion-Body Myositis andMyopathies explores the clinical and pathologicalexpression of muscle weakness in aging persons. Case studiesdemonstrate how physicians can more accurately diagnose weakeningelderly patients and make better management decisions.
It also explores sporadic inclusion-body myositis and hereditaryinclusion-body myopathies. The former, the most common progressivemuscle disease in the over 50s, is frequently under-diagnosed and,with the increasing population of aged individuals, is presenting agreater challenge. This disease of muscle has pathologicalsimilarities with the well-known Alzheimer and Parkinson braindiseases.
Edited and written by a leading international cast of authors,Muscle Ageing, Inclusion-Body Myositis and Myopathiesprovides a state-of-the-art guide to ageing-associatedneuromuscular disorders. It should be in the hands of all thoseinvolved in the care of aging and muscle-weakened patients.
Titles of Related Interest
Neuromuscular Disorders
Tawil and Vennance (eds); ISBN 978-0-470-65456-9
European Handbook of Neurological Management, Vol 1,2e
Gilhus, Barnes, Brainin (eds); ISBN 978-1-4051-8533-2
European Handbook of Neurological Management, Vol 2,2e
Gilhus, Barnes, Brainin (eds); ISBN 978-1-4051-8534-9

Autoren/Hrsg.

Askanas, Valerie

Engel, W. King

Inhaltsverzeichnis

List of Contributors, vii
Preface, xi
Part 1 Muscle Aging
1 Aging of the human neuromuscular system: pathological aspects,3
W. King Engel and Valerie Askanas
2 Aging of the human neuromuscular system: clinicalconsiderations, 33
W. King Engel and Valerie Askanas
3 Aging of the human neuromuscular system: patient vignettes,55
W. King Engel, Shalini Mahajan, and Valerie Askanas
4 Mitochondrial changes in aging with particular reference tomuscle, and possible clinical consequences, 81
Salvatore DiMauro, Eric Schon, and Michio Hirano
5 Protein degradation in aging cells and mitochondria: relevanceto the neuromuscular system, 89
Jenny K. Ngo and Kelvin J. A. Davies
6 Human muscle protein metabolism in relation to exercise andaging: potential therapeutic applications, 97
Micah J. Drummond and Blake B. Rasmussen
Part 2 Sporadic Inclusion-Body Myositis
7 Pathogenesis of sporadic inclusion-body myositis: role ofaging and muscle-fiber degeneration, and accumulation of the sameproteins as in Alzheimer and Parkinson brains, 111
Valerie Askanas, W. King Engel, and Anna Nogalska
8 Inflammatory and autoimmune features of inclusion-bodymyositis, 146
Marinos C. Dalakas
9 Sporadic inclusion-body myositis: clinical symptoms, physicalfindings, and diagnostic investigations, 159
Frank L. Mastaglia
10 Pathologic diagnostic criteria of sporadic inclusion-bodymyositis and hereditary inclusion-body myopathy muscle biopsies,168
Valerie Askanas and W. King Engel
Part 3 Hereditary Inclusion-Body Myopathies
11 Function and mutations of the GNE gene leading to distalmyopathy with rimmed vacuoles/hereditary inclusion-body myopathy,animal models, and potential treatment, 177
May Christine V. Malicdan, Satoru Noguchi, and IchizoNishino
12 GNE myopathy (hereditary inclusion-body myopathy/distalmyopathy with rimmed vacuoles): clinical features and epidemiology,191
Zohar Argov, Ichizo Nishino, and Ikuya Nonaka
13 Consequences of the hereditary inclusion-bodymyopathy-characteristic GNE mutations on muscle proteins in vivoand in vitro, 199
Aldobrando Broccolini and Massimiliano Mirabella
14 Function and structure of VCP mutations leading toinclusion-body myopathy associated with Paget disease of bone andfrontotemporal dementia, 206
Cezary Wojcik
15 Clinical spectrum of VCP myopathy, Paget disease, andfrontotemporal dementia: experimental models and potentialtreatments, 219
Virginia E. Kimonis, Eric Dec, Mallikarjun Badadani, AngeleNalbandian, Jouni Vesa, Vincent Caiozzo, Douglas Wallace, BarbaraMartin, Charles Smith, and Giles D. Watts
16 Drosophila and mouse models of hereditary myopathy caused bymutations in VCP/p97, 230
Nisha M. Badders and J. Paul Taylor
Index, 241

Über Autor(innen)

Edited by
Valerie Askanas, MD, PhD, Departments of Neurology andPathology, University of Southern California, Keck School ofMedicine, CA, USA
W. King Engel, MD, Departments of Neurology andPathology, University of Southern California, Keck School ofMedicine, CA, USA