Inclusion-body myositis: clinical, diagnostic, and pathologic aspects.
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.
Engel
W King
WK
The Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, CA, USA. askanas@hsc.usc.edu
Askanas
Valerie
V
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D000682:Amyloid / Q000032:analysis; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D004272:DNA, Mitochondrial / Q000235:genetics; D018450:Disease Progression; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000473:pathology; D009132:Muscles / Q000737:chemistry / Q000473:pathology; D018979:Myositis, Inclusion Body / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D033581:Stem Cell Transplantation; D016875:tau Proteins / Q000032:analysis
D000305:Adrenal Cortex Hormones; D000682:Amyloid; D000893:Anti-Inflammatory Agents; D004272:DNA, Mitochondrial; D016756:Immunoglobulins, Intravenous; D016875:tau Proteins