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Ophthalmoplegia, Chronic Progressive External

A mitochondrial myopathy characterized by slowly progressive paralysis of the levator palpebrae, orbicularis oculi, and extraocular muscles. Ragged-red fibers and atrophy are found on muscle biopsy. Familial and sporadic forms may occur. Disease onset is usually in the first or second decade of life, and the illness slowly progresses until usually all ocular motility is lost. (From Adams et al., Principles of Neurology, 6th ed, p1422)

Year introduced: 1993

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Tree Number(s): C05.651.460.700, C10.292.562.750.250, C10.597.622.447.511, C10.668.491.500.700, C11.590.472.250, C18.452.660.560.700, C23.550.291.500.688, C23.888.592.636.447.511

MeSH Unique ID: D017246

Entry Terms:

  • Graefe Disease
  • Disease, Graefe
  • Mitochondrial Ocular Myopathy
  • Myopathy, Mitochondrial Ocular
  • Ocular Myopathy, Mitochondrial
  • Chronic Progressive External Ophthalmoplegia
  • Graefe's Disease
  • Ocular Myopathy of Von Graefe-Fuchs
  • Ocular Myopathy of Von Graefe Fuchs
  • Ophthalmoplegia, Progressive External
  • External Ophthalmoplegia, Progressive
  • Progressive External Ophthalmoplegia
  • CPEO
  • Ocular Muscular Dystrophy
  • Dystrophy, Ocular Muscular
  • Muscular Dystrophies, Ocular
  • Muscular Dystrophy, Ocular
  • Ocular Muscular Dystrophies

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