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Abstract

Chronic progressive external ophthalmoplegia is an inherited or sporadic form of dystrophy that preferentially affects the extraocular muscles of the eye. While this dystrophy can manifest systemically, the extraocular muscles are commonly involved due to their constant metabolic demands and vulnerability to oxidative stress.1 This preferential involvement of the eyelid levator muscle and extraocular muscles makes CPEO an important condition to consider in a differential diagnosis for eyelid asymmetry and double vision among other conditions such as myasthenia gravis, thyroid-associated ophthalmoplegia, and other mitochondrial myopathies.2 This dystrophy tends to occur in the third or fourth decade of life. Nearly 60% of cases are de novo and occur due to mitochondrial DNA deletions; less commonly, this dystrophy can be inherited in an autosomal dominant or recessive fashion.2-4

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