Considering that intranuclear PABPN1 accumulation is seen only in OPMD and that the size of tubulofilamentous inclusion is reported to be different between OPMD and OPDM [7, 10, 15], the two diseases may have a difference in the mechanism of their etiopathogenesis. The gene discussed is PABPN1; the disease is oculopharyngodistal myopathy.