Inclusion-body myositis: muscle-fiber molecular pathology and possible pathogenic significance of its similarity to Alzheimer’s and Parkinson’s disease brains

Sporadic inclusion-body myositis (s-IBM), the most common muscle disease of older persons, is of unknown cause and lacks successful treatment. Here we summarize diagnostic criteria and discuss our current understanding of the steps in the pathogenic cascade. While it is agreed that both degeneration...

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Bibliographic Details
Published in:Acta neuropathologica 2008-12, Vol.116 (6), p.583-595
Main Authors: Askanas, Valerie, Engel, W. King
Format: Article
Language:English
Subjects:
Aging
Alzheimer Disease - pathology
Alzheimer Disease - physiopathology
Alzheimer's disease
Antibodies
Biopsy
Brain - pathology
Brain - physiopathology
Denervation
Disease
Humans
Medicine
Medicine & Public Health
Microscopy
Muscle Fibers, Skeletal - pathology
Muscle Weakness - physiopathology
Myositis, Inclusion Body - diagnosis
Myositis, Inclusion Body - physiopathology
Nerve Degeneration - physiopathology
Neurosciences
Parkinson Disease - pathology
Parkinson Disease - physiopathology
Parkinson's disease
Pathogenesis
Pathology
Review
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Summary:Sporadic inclusion-body myositis (s-IBM), the most common muscle disease of older persons, is of unknown cause and lacks successful treatment. Here we summarize diagnostic criteria and discuss our current understanding of the steps in the pathogenic cascade. While it is agreed that both degeneration and mononuclear-cell inflammation are components of the s-IBM pathology, how each relates to the pathogenesis remains unsettled. We suggest that the intra-muscle-fiber degenerative component plays the primary role, leading to muscle-fiber destruction and clinical weakness, since anti-inflammatory treatments are not of sustained benefit. We discuss possible treatment strategies aimed toward ameliorating a degenerative component, for example, lithium and resveratrol. Also discussed are the intriguing phenotypic similarities between s-IBM muscle fibers and the brains of Alzheimer and Parkinson’s diseases, the most common neurodegenerative diseases associated with aging. Similarities include, in the respective tissues, cellular aging, mitochondrial abnormalities, oxidative and endoplasmic-reticulum stresses, proteasome inhibition and multiprotein aggregates.
ISSN:0001-6322
1432-0533
DOI:10.1007/s00401-008-0449-0