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January 1954

CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIAA Clinical and Neuropathologic Report

Author Affiliations

PHILADELPHIA

From the Departments of Neurology and Anatomy, University of Pennsylvania School of Medicine.

AMA Arch NeurPsych. 1954;71(1):31-53. doi:10.1001/archneurpsyc.1954.02320370033003
Abstract

THERE is a disease of man which is most typically manifested as a gradually progressive weakness of the muscles of the eyelids and of the external muscles of the eyeballs. The disease begins insidiously in early life (before the age of 30) and gradually involves more and more of these muscles until finally, after many years (30 to 40), severe ptosis and external ophthalmoplegia are present. The process may be bilateral or unilateral; it may be asymmetric in degree of involvement; progress of the disease may stop temporarily or permanently at any point, but remissions do not occur; the pupils remain unaffected; family incidence has been noted, and other muscles of the face, limbs, or trunk may show a similar kind of progressive weakness and wasting. The literature on this disease has recently been summarized by Kiloh and Nevin,1 and it is they who have furnished a full and

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