Progressive Ophthalmoplegia

UNTIL 1950 the syndrome of progressive ptosis and ophthalmoplegia was generally considered to be neurogenic in origin as indicated by the designation "progressive nuclear ophthalmoplegia." This concept was based upon two cases¹,²in which there were neuronal abnormalities in postmortem study of the oculomotor nuclei. In 1951, however, Kiloh and Nevin³concluded that the ocular signs were due to myopathy because of histological abnormalities in the medial rectus muscle in one case; they considered the neural changes of earlier cases to be insignificant, and they introduced the terms "ocular myopathy" or "ocular dystrophy." Their view dominated the subsequent literature, and six later autopsy cases⁴⁻⁹also supported the concept of myopathy. In our own experience, however, the same syndrome has sometimes been associated with a variety of neurological abnormalities. There have also been several reports¹⁰⁻³¹of similar cases associated with neural disease. These combined disorders raise