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Published online before print December 16, 2005, doi:10.1212/01.wnl.0000192260.33106.bb)
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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.


* To whom correspondence should be addressed. E-mail: askanas{at}hsc.usc.edu.

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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