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Published online before print December 16, 2005, doi:10.1212/01.wnl.0000192260.33106.bb)
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NEUROLOGY 2006;66:S20-S29
© 2006 American Academy of Neurology

Inclusion-body myositis

Clinical, diagnostic, and pathologic aspects

W. King Engel, MD and Valerie Askanas, MD, PhD

From the Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, CA.

Address correspondence and reprint requests to Dr. W. King Engel, USC Neuromuscular Center, Good Samaritan Hospital, 637 S. Lucas Ave, Los Angeles, CA 90017-1912; email: askanas{at}hsc.usc.edu

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.


This article was previously published in electronic format as an Expedited E-Pub at www.neurology.org.

Supported by grants from the National Institutes of Health (NS31836, NS34103 and AG16768 Merit Award), the Muscular Dystrophy Association, The Myositis Association (V.A.), and the Helen Lewis Research Fund.

Disclosure: The authors report no conflicts of interest.

Received June 22, 2005. Accepted in final form October 14, 2005.




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