Oculopharyngodistal myopathy in three unrelated Korean patients: Early distal limb weakness is a characteristic fi nding in oculopharyngodistal myopathy

Objectives: To determine the clinical characteristics of Korean patients with sporadic oculopharyngodistal\nmyopathy. Methods: Three consecutive unrelated Korean patients with sporadic oculopharyngodistal\nmyopathy participated in this study. Stepwise approaches were performed including careful clinical\nexamination, electrophysiological study, pathology evaluation and genetic study. The literature on\noculopharyngodistal myopathy was reviewed. Results: Clinically, our patients showed consistent\nfeatures of initial distal limb weakness with variable ophthalmoplegia, bulbar weakness or proximal\nweakness. All the patients showed rimmed vacuoles and myopathic changes.\nConclusion: Through the literature review and this study, we believe that initial distal limb weakness\nis a characteristic fi nding in patients with oculopharyngodistal myopathy in some ethnic groups.