Oculopharyngeal muscular dystrophy (OPMD) is a late-onset, dominantly inherited disorder characterized by progressive ptosis, dysphagia and proximal limb weakness. It was first described in 1915 (Taylor, 1915), when it was attributed to a progressive cranial neuropathy. However, in 1962, Victor and colleagues clearly demonstrated that the primary pathology was myopathic and that it was inherited in an autosomal dominant manner (Victor et al., 1962). Muscle histology in affected individuals typically reveals abnormal variability in fibre size, an increase in endomysial fibrosis, and cytoplasmic basophilic rimmed vacuoles similar to those seen in inclusion body myositis (Coquet et al., 1990). Ultrastructural studies on muscle in OPMD have demonstrated filamentous nuclear inclusions in a minority of fibres that are not seen in other inherited or acquired muscle diseases (Tomé and Fardeau, 1980).
https://academic.oup.com/brain/article/124/3/522/334351#89180107