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 InterestNeuromuscular DisordersTawil and Vennance (eds); ISBN 978-0-470-65456-9European Handbook of Neurological Management, Vol 1,2eGilhus, Barnes, Brainin (eds); ISBN 978-1-4051-8533-2European Handbook of Neurological Management, Vol 2,2eGilhus, Barnes, Brainin (eds); ISBN 978-1-4051-8534-9

List of Contributors, viiPreface, xiPart 1 Muscle Aging1 Aging of the human neuromuscular system: pathological aspects,3W. King Engel and Valerie Askanas2 Aging of the human neuromuscular system: clinicalconsiderations, 33W. King Engel and Valerie Askanas3 Aging of the human neuromuscular system: patient vignettes,55W. King Engel, Shalini Mahajan, and Valerie Askanas4 Mitochondrial changes in aging with particular reference tomuscle, and possible clinical consequences, 81Salvatore DiMauro, Eric Schon, and Michio Hirano5 Protein degradation in aging cells and mitochondria: relevanceto the neuromuscular system, 89Jenny K. Ngo and Kelvin J. A. Davies6 Human muscle protein metabolism in relation to exercise andaging: potential therapeutic applications, 97Micah J. Drummond and Blake B. RasmussenPart 2 Sporadic Inclusion-Body Myositis7 Pathogenesis of sporadic inclusion-body myositis: role ofaging and muscle-fiber degeneration, and accumulation of the sameproteins as in Alzheimer and Parkinson brains, 111Valerie Askanas, W. King Engel, and Anna Nogalska8 Inflammatory and autoimmune features of inclusion-bodymyositis, 146Marinos C. Dalakas9 Sporadic inclusion-body myositis: clinical symptoms, physicalfindings, and diagnostic investigations, 159Frank L. Mastaglia10 Pathologic diagnostic criteria of sporadic inclusion-bodymyositis and hereditary inclusion-body myopathy muscle biopsies,168Valerie Askanas and W. King EngelPart 3 Hereditary Inclusion-Body Myopathies11 Function and mutations of the GNE gene leading to distalmyopathy with rimmed vacuoles/hereditary inclusion-body myopathy,animal models, and potential treatment, 177May Christine V. Malicdan, Satoru Noguchi, and IchizoNishino12 GNE myopathy (hereditary inclusion-body myopathy/distalmyopathy with rimmed vacuoles): clinical features and epidemiology,191Zohar Argov, Ichizo Nishino, and Ikuya Nonaka13 Consequences of the hereditary inclusion-bodymyopathy-characteristic GNE mutations on muscle proteins in vivoand in vitro, 199Aldobrando Broccolini and Massimiliano Mirabella14 Function and structure of VCP mutations leading toinclusion-body myopathy associated with Paget disease of bone andfrontotemporal dementia, 206Cezary Wojcik15 Clinical spectrum of VCP myopathy, Paget disease, andfrontotemporal dementia: experimental models and potentialtreatments, 219Virginia E. Kimonis, Eric Dec, Mallikarjun Badadani, AngeleNalbandian, Jouni Vesa, Vincent Caiozzo, Douglas Wallace, BarbaraMartin, Charles Smith, and Giles D. Watts16 Drosophila and mouse models of hereditary myopathy caused bymutations in VCP/p97, 230Nisha M. Badders and J. Paul TaylorIndex, 241