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)
Also Known As:
Progressive External Ophthalmoplegia; CPEO; Ocular Muscular Dystrophy; Ophthalmoplegia, Chronic Progressive External; Graefe's Disease; Ocular Myopathy of Von Graefe-Fuchs; Ophthalmoplegia, Progressive External; Disease, Graefe; Dystrophy, Ocular Muscular; External Ophthalmoplegia, Progressive; Muscular Dystrophies, Ocular; Muscular Dystrophy, Ocular; Myopathy, Mitochondrial Ocular; Ocular Muscular Dystrophies; Ocular Myopathy of Von Graefe Fuchs; Ocular Myopathy, Mitochondrial; Graefe Disease; Mitochondrial Ocular Myopathy