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October 1968

Progressive OphthalmoplegiaReport of Cases

Arch Neurol. 1968;19(4):362-376. doi:10.1001/archneur.1968.00480040028002

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 cases1,2 in which there were neuronal abnormalities in postmortem study of the oculomotor nuclei. In 1951, however, Kiloh and Nevin3 concluded 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 cases4-9 also 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 reports10-31 of similar cases associated with neural disease. These combined disorders raise

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