[Progressive external ophthalmoplegia and distal myopathy]

A patient, 58 years old, presents progressive blepharoptosis, in both eyes and external ophthalmoplegia. The general somatic examination, shows, at the level of the higher limbs, distal myopathy with muscular hypotony and articular deformities fibrosis and tendinous retraction. On examining the eye bottom we found colloid degeneration in the muscle region. Within the same family a ten-year-old nephew presents congenital ptosis. The muscular biopsy from the levator palpebrae shows muscular degenerative lesions with the reduction of nuclei and the proliferation of conjunctive tissue. It is shown that progressive external ophthalmoplegia must be interpreted as an ocular myopathy. The association with the distal myopathy at the level of the higher limbs, in the presented observation, upholds this pathogeny.