inclusion body myopathy
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Epidemiology
- mean age of onset 42 years
Pathology
- benign myopathy
- cytoplasmic inclusions (rimmed vacuoles) by light microscopy in type 1 muscle fibers
- myofibrillary origin of the inclusions
Clinical manifestations
- clinical features variable
- mild weakness of pelvic & shoulder girdles
- weakness & atrophy of muscles in the distal extremities, neck, thorax, & shoulder girdle
- disabilty after 5 to 10 years due to chronic respiratory failure
Laboratory
- serum creatine kinase normal or slightly elevated
Notes
- OMIM reference[1] has been moved to[2] which raises question of how inclusion body myopathy differs from myofibrillar myopathy