OCULAR MYOPATHY (PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA) WITH NEUROPATHIC COMPLICATIONS

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Abstract
The condition known as ocular myopathy or progressive external ophthalmoplegia was reviewed. Case reports (335) were analyzed and 13 personal cases were described. Histological and EM studies on 1 case were reported. It was clear from these cases that, although in its simplest form the condition may consist merely of myopathic changes confined to the upper eyelids and external ocular muscules, myopathic changes may also occur in the muscles of the face, neck and proximal parts of the limbs. A variety of other neurological disorders may also develop and degenerative lesions were found in the peripheral nerves, spinal cord, brain stem and basal ganglia. Other associated conditions, e.g., perceptive deafness, pigmentary retinal degeneration, cardiomyopathy and red cell abnormalities may also occur. There is thus a wide spectrum of syndromes, all based on a condition which is possibly a general metabolic disorder. Recent work has directed attention to mitochondrial abnormalities in the affected muscle fibers and other cells. A positive family history may be present and an autosomal dominant mode of inheritance seems likely.

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