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CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA

This rather rare disease is characterized by a slowly progressive inability to move the eyes and severe early ptosis yet normal pupillary reactions and accommodation. It may begin at any age and progresses over a period of 5-15 years to complete external ophthalmoplegia. It is a form of mitochondrial myopathy and may be associated with other manifestations of mitochondrial disease such as pigmentary degeneration of the retina, deafness, cerebellar-vestibular abnormalities, seizures, cardiac conduction defects, and peripheral sensorimotor neuropathy, in which case the term "ophthalmoplegia-plus" may be applied. Onset before 15 years of age of chronic progressive external ophthalmoplegia, heart block, and pigmentary reti-nopathy constitutes the Kearns-Sayre syndrome. Chronic progressive external ophthalmoplegia is associated with deletions of mitochondrial DNA, which are more frequent and more extensive in the cases with nonocular manifestations.

10.1036/1535-8860.ch14

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