We report a patient with progressive proximal and distal weakness, dysphagia, respiratory weakness, subcutaneous calcifications, ptosis and ophthalmoparesis with inflammation, rimmed vacuoles and positive amyloid and ubiquitin on muscle biopsy. The histopathological features fit best with inclusion body myositis but ophthalmoparesis and ptosis have not been described in that disorder. The clinical phenotype fits best with hereditary inclusion body myopathy or distal-oculopharyngeal muscular dystrophy but the degree of inflammation seen is unusual. None of these are associated with calcinosis. We present this as an unusual phenotype for any vacuolar myopathy and as a challenge to the current diagnostic criteria for sporadic inclusion body myositis.
- Inclusion body myositis