Ophthalmoplegia, Chronic Progressive External

**Definition:** 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)

**Tree numbers:** - 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

**Synonyms:** - CPEO - Graefe Disease - Mitochondrial Ocular Myopathy - Ocular Muscular Dystrophy - Graefe's Disease - Ocular Myopathy of Von Graefe-Fuchs - Ophthalmoplegia, Progressive External - Progressive External Ophthalmoplegia - Chronic Progressive External Ophthalmoplegia