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January 24, 2006; 66 (1 suppl 1) Articles

Inclusion-body myositis

Clinical, diagnostic, and pathologic aspects

W. King Engel, Valerie Askanas
First published December 16, 2005, DOI: https://doi.org/10.1212/01.wnl.0000192260.33106.bb
W. King Engel
MD
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Valerie Askanas
MD, PhD
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Inclusion-body myositis
Clinical, diagnostic, and pathologic aspects
W. King Engel, Valerie Askanas
Neurology Jan 2006, 66 (1 suppl 1) S20-S29; DOI: 10.1212/01.wnl.0000192260.33106.bb

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Abstract

The diagnostic aspects of sporadic inclusion-body myositis (s-IBM), and a few comments on our own approach to its treatment, are presented to foster the goals of this symposium, which was organized to provoke new ideas concerning the cause and treatment of this currently unsolvable disease. s-IBM is the most common, progressive, debilitating muscle disease beginning in persons over age 50 years, and it is more common in men. Diagnostic parameters reviewed are clinical, muscle-biopsy histochemistry, electrophysiologic and CSF evaluations. Overall, the degenerative phenomena in s-IBM muscle fibers seem to be the major cause of the progressive, unstoppable weakness, rather than the lymphocytic inflammation. Available treatments are of only slight, temporary benefit for only some s-IBM patients, indicating a desperate need for definitive therapies.

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  • Article
    • Abstract
    • s-IBM clinical features.
    • Associations with other systemic disorders.
    • Light and electron microscopic pathology.
    • Diagnostic criteria of s-IBM muscle biopsies.
    • Clinical electrophysiology of s-IBM.
    • Hereditary inclusion-body myopathies.
    • Treatment: A personal view.
    • Other treatment possibilities.
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    • References
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