Inflammatory myopathy in oculopharyngeal dystrophy

A 75-year-old French-Canadian woman with familial oculopharyngeal dystrophy demonstrated histopathologic alterations similar to those of idiopathic polymyositis. A second biopsy obtained 15 months later was more consistent with previously reported cases. It is suggested that certain patients with oculopharyngeal dystrophy may pass through an initial phase of secondary muscle inflammation similar to that seen in some other heritable myopathies.